Drug-induced lupus syndrome. Drug-induced systemic lupus erythematosus

LUPUS ERYTHEMATOSUS (lupus erythematodes, lupus erythematosus; syn.: erythema centrifugum, erythematosis) - a group concept that includes a number of nosological units, Ch. arr. systemic lupus erythematosus and discoid lupus erythematosus, as well as drug-induced lupus syndrome. Systemic and discoid K. v. have a number of common features. So, both systemic and discoid K. v. affects predominantly women; Both forms are characterized by erythematous rashes on the skin of the face, limbs, trunk and mucous membranes (enanthema), increased sensitivity to solar radiation (photosensitization); transition to discoid K. is possible. systemic (in 3-5% of patients); in some families there may be patients with discoid, systemic K. v. and other collagen diseases. At the same time, differences in the nature of erythematous rashes and especially systemic manifestations in systemic and discoid K. v., features of pathogenesis, in particular deep disturbances of immunogenesis in systemic K. v., allow most authors to consider them as separate nosole forms. This was reflected in the “Statistical Classification of Diseases and Causes of Death” (1969): discoid K. v. belongs to class XII “Diseases of the skin and subcutaneous tissue", and systemic K. v. - to class XIII "Diseases of the musculoskeletal system and connective tissue."

Systemic lupus erythematosus

Systemic K.v. ( lupus erythematosus systemicus; syn.: acute lupus erythematosus, erythematous chroniosepsis, Liebmann-Sachs disease) - chronic systemic inflammatory disease connective tissue and blood vessels with pronounced autoimmune pathogenesis and, apparently, viral etiology; refers to diffuse connective tissue diseases - collagenosis (see Collagen diseases). Systemic K.v. is a disease of women of childbearing age (20-30 years), teenage girls often get sick. The ratio of women to men suffering from this disease, according to most statistics, is 8: 1 - 10: 1.

Story

Systemic K.v. described in 1872 by the Viennese dermatologist M. Kaposi as discoid K. v., characterized by fever, pleuropneumonia, rapid development coma or stupor and death. In 1923, Libman and Sacks (E. Libman and V. Sacks) described atypical verrucous endocarditis (Libman-Sacks endocarditis), polyserositis, pneumonia and erythematous rashes in the dorsum of the nose and zygomatic arches - the so-called. butterfly Modern doctrine of systemic K. v. associated with the names of Klemperer, Pollack and Baer (P. Klemperer, A.D. Pollack and G. Baehr), who in 1941 drew attention to diffuse collagen disease, describing systemic damage to connective tissue in this disease and scleroderma. With the discovery of LE cells (Lupus erythematosus cells) by Hargraves, Richmond and Morton (M. M. Hargraves, H. Richmond, R. Morton) in 1948, and in 1949 by Y. R. Ha-serick, the lupus factor was attention is paid to autoimmune disorders.

In the domestic literature, the first wedge, description of “acute lupus erythematosus” belongs to G. I. Meshchersky (1911), and pathomorphology - I. V. Davydovsky (1929) and others. Systematic study of systemic K. v. in our country, started by E. M. Tareev, O. M. Vinogradova and others. In 1965, E. M. Tareev et al., in the monograph “Collagenoses,” after analyzing 150 observations, described systemic K. v. in all its diversity, raised the question of the curability of the disease and outlined ways for further study. Unconditional progress in the development of the doctrine of systemic medicine. due to highly effective treatment with corticosteroids and immunosuppressants.

Statistics

Population studies by Siegel et al. (1962-1965) showed that the incidence in the Manhattan region (New York) increased from 25 to 1 million people. in 1955 to 83 per 1 million in 1964. Dubois (E. L. Dubois, 1974) suggests that in the USA systemic K. century. 5,200 people fall ill every year, therefore, every 5 years a minimum of 25,000 patients accumulate. Leonhardt (T. Leonhardt) in 1955 showed that the prevalence of systemic K. v. in Malmo (Sweden) from 1955 to 1960 there was 29 per 1 million. Mortality in the USA, according to Cobb (Cobb, 1970), is 5.8 per 1 million population, higher among women aged 25-44 of the year. Mortality, according to materials from the Institute of Rheumatism of the USSR Academy of Medical Sciences, decreased from 90% in 1959-1960. up to 10% by 1975

Etiology

The etiology is not clear, however, the hypothesis about the role of hron, a persistent viral infection, was developed in connection with the discovery by electron microscopy in the affected organs (skin, kidneys, synovium) of tubuloreticular structures located in the cytoplasm of endothelial cells, as well as in lymphocytes and platelets of peripheral blood , which resembled the nucleoprotein of paramyxoviruses. With systemic K. v. Circulating antibodies to measles, rubella, parainfluenza and other RNA viruses from the group of paramyxoviruses were also detected in high titers. Lymphocytotoxic antibodies, which are markers of persistent viral infection, were detected in patients and their relatives, and, in addition, in the same groups and among medical staff working with patients, antibodies to double-stranded (viral) RNA were detected. In connection with the viral etiology of systemic K. v. phenomena such as hybridization of the measles virus genome with the DNA of cells of affected organs (spleen, kidneys), detection of oncornavirus type C antigens in fractions of the spleen, placenta and kidney are discussed. Hypothesis about the significance of hron, viral infection in systemic K. century. is also based on the study of the disease in New Zealand mice, in which the role of oncornavirus type C has been proven.

Intolerance to drugs, vaccines, photosensitivity, the formation of the menstrual cycle, pregnancy, childbirth, abortion, etc. are considered as factors provoking the disease or its exacerbation; they are important for prevention and timely diagnosis, since the connection between the onset or exacerbations of the disease with these factors is more typical for systemic K. v. than for other related diseases.

Pathological anatomy

Systemic K. v., being a representative of the group of collagen diseases, is characterized by a generalized spread of patol, a process that covers all organs and systems, which determines the clinical and anatomical polymorphism of the disease. Generalization is caused by the circulation in the blood of immune complexes that damage the vessels of the microvasculature, resulting in systemic progressive disorganization of connective tissue. Immunopathol. reactions are confirmed by increased function of the organs of immunogenesis, precipitation in the walls of blood vessels and in the affected tissues of immune complexes with the appearance of immunocompetent cells (see). Damage to microcirculation vessels is manifested by common vasculitis of a destructive or proliferative nature (see Vasculitis). In the endothelium of the capillaries, electron microscopy reveals peculiar tubular formations (Fig. 1), similar to the ribonucleoprotein of the paramyxovirus and, possibly, playing an etiol role.

The specificity of tissue reactions during systemic K. v. cause signs of pathology of cell nuclei: fibrinoid basophilia, karyorrhexis, hematoxylin bodies, LE cells, central chromatolysis. Fibrinoid basophilia is caused by the admixture of acidic nuclear decay products. Hematoxylin bodies, described in 1932 by L. Gross, are swollen nuclei of dead cells with lysed chromatin. LE cells, or lupus erythematosus cells, are mature neutrophils, the cytoplasm of which is almost entirely filled with the phagocytosed nucleus of a dead leukocyte. At the same time, the own core is pushed to the periphery. They can be found in the sinuses of lymph nodes, in imprint smears from inflammatory exudate, for example, from pneumonic foci (Fig. 2). Central chromatolysis is manifested by the washing out of chromatin from the center of cell nuclei with clearing of the latter.

Rice. 6. Microscopic specimen of a kidney for lupus glomerulonephritis with characteristic signs of systemic lupus erythematosus: 1 - focal fibrinoid: 2 - “wire loops”; 3 - hyaline thrombi; 4 - karyorrhexis.

The most characteristic changes in systemic K. century. noted in the kidneys, heart, spleen. Kidney damage is characterized by the development of lupus glomerulonephritis, microscopically manifested in two forms: 1) with characteristic signs of systemic K. v.; 2) without characteristic signs of systemic K. v. (V.V. Serov et al., 1974). Characteristic signs include fibrinoid in the glomerular capillaries, the phenomenon of “wire loops”, hyaline thrombi, karyorrhexis (tsvetn. fig. 6). “Wire loops” are thickened, impregnated with plasma proteins and exposed due to desquamation of the endothelium, the basement membranes of glomerular capillaries, which are considered as a prestage of fibrinoid changes. They were described in 1935 by G. Baehr et al. Hyaline thrombi are located in the lumen of the glomerular capillaries and, based on their tinctorial properties, are regarded as intravascular fibrinoid. The second form is characterized by the development of membranous, membranous-proliferative or fibroplastic changes inherent in banal glomerulonephritis. Both forms are often found in combination.

The development of lupus glomerulonephritis is based on damage to the renal glomeruli by immune complexes. Immunofluorescence microscopy reveals luminescence of immunoglobulins (Fig. 3), complement, and fibrin in the glomeruli. Electron microscopy reveals equivalents of immune complexes in the form of deposits (Fig. 4). When the latter are localized on the subepithelial surface of the basement membrane, damage to the processes of podocytes and the formation of spiny outgrowths of the membrane are observed, which is referred to as membranous transformation. In the clinic, nephrotic syndrome is often noted. The proliferative reaction, according to V.V. Serov et al. (1974), is associated with the proliferation of mesangial cells. As a result of lupus nephritis, secondary shrinkage of the kidneys develops.

Heart damage is characterized by the development of Libman-Sachs endocarditis (Fig. 5). Endocarditis affects the leaflets and chords of the valves, the parietal endocardium, usually does not lead to heart disease, but the development of mitral valve insufficiency is possible. In the myocardium, fatty degeneration of muscle cells ("tiger" heart) is found, and less commonly, diffuse proliferative interstitial myocarditis - lupus carditis. The pericardium is most often affected.

The spleen is enlarged, microscopically it is found characteristic feature- “bulbous” sclerosis - layered ring-shaped growth of collagen fibers in the form of a coupling around sclerotic arteries and arterioles (Fig. 6). The follicles are atrophied, plasmatization and macrophage reaction are expressed in the red pulp. Plasmatization is also noted in enlarged lymph nodes, bone marrow, and thymus.

Possible development of lupus pneumonitis, proceeding according to the type interstitial pneumonia with vasculitis and cellular infiltration of interstitial tissue. Lung damage may be associated with a secondary infection.

Lupus can affect the liver. In this case, lymphoplasmacytic infiltration and degeneration of hepatocytes are observed in the portal tracts.

Vasculitis is associated with damage to the nervous system.

Visceral lesions are often combined with lesions of the musculoskeletal system and skin. With high disease activity in the skeletal muscles, the picture of acute focal myositis is determined. In the joints, a picture of acute synovitis may develop with a predominance of exudative reactions and usually without subsequent deforming processes.

Microscopic examination of the skin of affected and externally unaffected areas reveals vasculitis, often proliferative, in 70-80% of patients (tsvetn. Fig. 7). Immunofluorescence study reveals the glow of immunoglobulins on the basement membrane in the area of ​​the dermal-epidermal junction (Fig. 7).

Complications and manifestations of the disease leading to the death of patients (renal failure, focal confluent pneumonia, sepsis, anemia, vasculitis leading to cerebral and heart infarctions) have clear morphological signs. For morphol. the picture is left imprinted by corticosteroid therapy, the consequence of which is inhibition of the reaction of immunogenesis organs, adrenal atrophy, osteoporosis, areactive ulcers of the gastrointestinal tract. tract, signs of Itsenko-Cushing syndrome, sometimes an outbreak of tuberculosis, sepsis. Active treatment caused a medicinal pathomorphosis of the disease, characterized by a predominance of chronic forms of the disease over acute ones, an increase specific gravity proliferative processes, sclerotic changes, decreased frequency of karyorrhexis, hematoxylin bodies, Libman-Sachs endocarditis.

Morphol, diagnosis of systemic K. v. is based on taking into account nuclear pathology, lupus glomerulonephritis, “bulbous” sclerosis in the spleen, positive immunofluorescence results, vasculitis, connective tissue disorganization, Liebman-Sachs endocarditis. For intravital morphology, diagnosis, biopsy material of the kidneys, skin, and skeletal muscles is examined with the mandatory use of immunofluorescent methods.

Pathogenesis

With systemic K. v. the role of violations of the humoral immunity with the development of organ-nonspecific autoimmune reactions is obvious, which is manifested by hyperfunction of B-lymphocytes and a wide range of circulating autoantibodies (see) - to whole cell nuclei and individual components of the nucleus (DNA, nucleoprotein), as well as lysosomes, mitochondria, cardiolipids ( false-positive Wasserman reaction), blood coagulation factors, leukocytes, platelets, erythrocytes, aggregated gamma globulin (see Rheumatoid factor), etc. These antibodies, being antibodies that witness the damage that has occurred, are capable of forming circulating immune complexes that are deposited on the basal membranes of the kidneys , skin, etc., cause their damage with the development inflammatory reaction. This is the immune-complex mechanism for the development of lupus nephritis, vasculitis, etc. The presence of a DNA complex - an antibody to this DNA and complement is proven by the isolation of antibodies to DNA from kidney tissue, and the immune complexes themselves are detected by immunofluorescence (see). High activity of systemic K. v. characterized by hypocomplementemia - a decrease in the content of whole complement (CH50) and its components, especially C3, which takes part in the antigen-antibody reaction, C4, CD1, C9, etc. (see Complement). Many facts have accumulated indicating that there is an imbalance in the humoral and cellular components of immunity; the latter is manifested by various delayed-type hypersensitivity reactions and a decrease in the content of T-lymphocytes. The presence in certain families of systemic and discoid K. v., various autoimmune diseases, photosensitivity and drug intolerance, the detection of a wide range of circulating autoantibodies in members of these families allows us to think about the role of genetic predisposition in the development of the disease, but the specific mechanisms of this predisposition are not yet known.

Experimental models of systemic K. v. - a disease of New Zealand mice (NZB, NZW and their hybrids NZB/NZW F1) and dogs of special genetic lines (canine lupus) - confirm the above statements, since these models are certainly characterized by a genetic predisposition, an imbalance in humoral and cellular immunity and vertical transmission of oncornavirus C in New Zealand mice.

Clinical picture

The complaints of patients are varied, but most often they complain of pain in the joints, fever, loss of appetite, and sleep. As a rule, systemic K. v. begins subacutely with recurrent polyarthritis, reminiscent of rheumatic, fever, various skin rashes, malaise, weakness, weight loss. Less common is an acute onset with high fever, sharp pain and swelling of the joints, the “butterfly” symptom, polyserositis, nephritis, etc. In 1/3 of patients 5-10 years or more, one of the monosyndromes is observed - recurrent arthritis, polyserositis, Raynaud’s syndrome, Werlhoff, epileptiform, but later the disease acquires relapsing course with the development of characteristic polysyndromy.

Lupus arthritis observed in almost all patients; it is manifested by migrating arthralgia (see), arthritis (see), transient painful flexion contractures. Mostly small joints of the hands, wrists, ankles, and less often large joints are affected. In 10-15% of patients, fusiform deformation of the fingers and muscle atrophy on the back of the hands may develop. Articular syndrome is usually accompanied by myalgia, myositis, ossalgia and tendovaginitis. When rentgenol, the study reveals epiphyseal osteoporosis, mainly in the joints of the hands and wrists.

Rice. 1. "Butterfly" type of centrifugal erythema.

Rice. 2. “Butterfly” in the form of spots with sharp dense swelling.

Skin damage. The most typical syndrome is “butterfly” - erythematous rashes on the face in the area of ​​the dorsum of the nose (“butterfly body”) and zygomatic arches (“butterfly wings”). According to O. L. Ivanov, V. A. Nasonova (1970), the following variants of erythema are observed: 1) vascular (vasculitic) “butterfly” - unstable, pulsating, diffuse redness with a cyanotic tint in the middle zone of the face, intensifying when exposed to external factors (insolation, wind, cold, etc.) or excitement; 2) “butterfly” type of centrifugal erythema - persistent erythematous-edematous spots, sometimes with mild follicular hyperkeratosis (erythema centrifugum Biett; color Fig. 1); 3) “butterfly” in the form of bright pink spots with sharp dense swelling against the background of general swelling and redness of the face (erysipelas faciei perstans Kaposi; color Fig. 2); 4) “butterfly”, consisting of discoid-type elements with clear cicatricial atrophy. Erythematous changes are also localized on the earlobes, neck, forehead, scalp, red border of the lips, trunk (usually in upper section chest in the form of a neckline), limbs, over the affected joints. Some patients experience polymorphic erythema, urticaria, purpura, nodules and other elements.

A peculiar analogue of the “butterfly” of the first and second types are vasculitis (capillaritis) - small erythematous spots with slight swelling, telangiectasia and mild atrophy on the terminal phalanges of the fingers and toes, less often on the palms and soles (color Fig. 3). Various trophic disorders - hair loss, deformation and brittleness of nails, ulcerative skin defects, bedsores, etc. create the characteristic appearance of a patient with systemic K. v.

Damage to mucous membranes manifested by enanthema on the hard palate, aphthous stomatitis, thrush, hemorrhages, lupus cheilitis.

Polyserositis- migrating bilateral pleurisy and pericarditis, less commonly peritonitis - is considered an integral component of the diagnostic triad, along with dermatitis and arthritis. The effusion is usually small and its composition resembles rheumatic effusion, but contains LE cells and antinuclear factors. Recurring, polyserositis (see) leads to the development of adhesions up to obliteration of the pericardial cavity, pleura, perisplenitis and perihepatitis. Wedge, manifestations of serositis are usual (pain, friction noise of the pericardium, pleura, peritoneum, etc.), but due to the small amount of exudates and the tendency to quickly disappear, clinicians easily view them, however, with X-ray examination, pleuropericardial adhesions or thickening of the costal bone are often revealed , interlobar, mediastinal pleura.

Lupus carditis very typical for systemic K. century; it is characterized by the simultaneous or sequential development of pericarditis (see), myocarditis (see) or atypical Libman-Sachs warty endocarditis on the mitral and other heart valves, as well as the parietal endocardium and large vessels. Endocarditis ends with marginal sclerosis of the valve, less often with mitral valve insufficiency with characteristic auscultatory symptoms.

Vascular damage with systemic K. century. characteristic of pathol. processes in organs. Nevertheless, it should be noted the possibility of developing Raynaud's syndrome (long before the typical picture of the disease), damage to both small and large arterial and venous trunks (endarteritis, phlebitis).

Lupus pneumonitis- a vascular-connective tissue process in the lungs, in an acute course it proceeds as vasculitis (“vascular pneumonia”), and in other variants of the course - in the form of basal pneumonitis (see) with a normal wedge, a picture of a parenchymal process, but characteristic rentgenol, symptoms ( the reticular structure of the enhanced pulmonary pattern, the high position of the diaphragm and basal discoid atelectasis) gives the syndrome a large diagnostic value.

Lupus glomerulonephritis(lupus nephritis) - classic immune complex glomerulonephritis (see), observed in half of the patients during the generalization of the process according to the type of urinary syndrome, nephritic and nephrotic. Kidney biopsy followed by gistol and immunomorphol is of great diagnostic importance. research.

Damage to the neuropsychic sphere(neurolupus) - manifests itself at the onset of the disease asthenovegetative syndrome, and at the height of the disease one can observe a variety of symptoms and syndromes from the central and peripheral nervous systems, usually combined - meningoencephalitis, encephalopolyneuritis, encephalomyelitis or meningoencephalomyelitis with polyradiculoneuritis (the latter has diagnostic value).

In the acute form of the disease, affective disorders, delirious-oneiric and delirious types of stupefaction, and patterns of stupor varying in depth can be observed.

Affective disorders manifested by states of anxious depression, as well as manic-euphoric syndromes. Anxiety depression are accompanied by pictures of verbal hallucinosis of condemning content, fragmentary ideas of attitude and nihilistic delirium (the latter is characterized by instability and lack of a tendency to systematize). In manic-euphoric states, there is an elevated mood with a feeling of carelessness, self-satisfaction, and a complete lack of awareness of the disease. At times, some psychomotor agitation is observed, and persistent insomnia is characteristic; during short periods of sleep - vivid dreams, the content of which is often mixed in the patient’s mind with real events.

Delirious-oneiric states excessively volatile; either dream disorders with fantastic or ordinary themes, or abundant colorful, scene-like visual hallucinations come to the fore. Patients feel like observers of ongoing events or victims of violence. Excitement in these cases is of a confused and fussy nature, limited to the boundaries of the bed, and is often replaced by a state of immobility with muscle tension and a loud, monotonously prolonged cry.

Delirious states begin with the appearance of vivid nightmares during the period of falling asleep, followed by multiple, colorful, threatening visual hallucinations, accompanied by verbal hallucinations and a constant feeling of fear.

The intensity of mental disorders correlates with the severity of somatic manifestations, with high degree activity of the lupus process.

The described correlations of somatopsychic disorders make it possible to attribute psychoses to systemic K. v. to the group of exogenous organic brain lesions.

It must be borne in mind that with systemic K. v. disturbances in the emotional sphere can also develop in connection with hormonal therapy (steroid psychoses).

Damage to the reticuloendothelial system is expressed in polyadenia (enlargement of all groups of lymph nodes) - a very common and, apparently, early sign of generalization of the lupus process, as well as enlargement of the liver and spleen.

Flow

There are acute, subacute and chronic course of the disease. With an acute onset, patients can indicate the day of development of fever, acute polyarthritis, serositis, “butterfly”, and in the next 3-6 months. One can note pronounced polysyndromic behavior and lupus nephritis or meningoencephalomyelitis with polyradiculoneuritis. Untreated acute systemic K. v. previously led to death 1 - 2 years from the onset of the disease.

With a subacute onset, general asthenic syndromes or recurrent arthralgia, arthritis, and nonspecific skin lesions gradually develop. With each exacerbation in patol, the process involves more and more new organs and systems. A polysyndromic pattern develops, similar to that observed in the acute course of the disease, with a significant incidence of diffuse lupus nephritis and neurolupus.

When chronic, the course of the disease manifests itself for a long time as individual relapses of certain syndromes, and in the 5-10th year of the disease other organ manifestations (pneumonitis, nephritis, etc.) may develop with the development of characteristic polysyndromicity.

Variants of the onset and course of systemic K. century. have age-related patterns. The acute course is usually observed in children and adolescents, menopausal women and the elderly, subacute - mainly in women of childbearing age.

Complications

Among the complications of systemic K. century. the most common is a secondary infection (coccal, tuberculous, fungal, viral), associated with a violation of natural immunity, or with illness, or with inadequate treatment with corticosteroids, the use of immunosuppressants. With the progressive course of systemic K. v. and long-term treatment with corticosteroid drugs, especially in young people, miliary tuberculosis develops, therefore attention to tuberculosis infection with systemic K. v. must be constant for timely recognition and appropriate correction. Shingles (herpes zoster) develops in 10-15% of patients treated for a long time with large doses of corticosteroids and cytotoxic drugs.

Diagnosis

The diagnosis does not present great difficulties in patients with a typical “butterfly” of any type. However, this sign occurs in less than half of patients, and as an early sign - only in 15-20% of patients. Therefore, other symptoms, such as arthritis, nephritis, and their combinations, become of great diagnostic importance. The possibility of intravital biopsy of the joint and kidney makes it possible to more often recognize the lupus nature of arthritis or nephritis. Polysyndromy, detection of LE cells, high titer of antinuclear factors (ANF) or antibodies to native DNA (nDNA) are of diagnostic value. LE cells are found in 70% of patients with systemic K. v. and more. Single LE cells can also be observed in other diseases.

ANF ​​is an IgG directed against the patient's cell nuclei. Usually, to determine ANF, the Immunofluorescence method is used (Fig. 8), in which sections of rat liver, rich in nuclei, are taken as antigenic material, on which the patient’s serum and fluorescein-labeled antiglobulins are layered. For systemic K. century. the most characteristic is peripheral, edge luminescence (Fig. 8.2), due to the presence of antibodies to DNA, and a high titer of this reaction.

Antibodies to DNA are determined various methods in RIGA (see Hemagglutination), in which sheep red blood cells are loaded with DNA, in the reaction of flocculation of bentonite particles (see Flocculation), also loaded with DNA; In addition, they use the method of radioimmune binding of iodine-labeled nDNA and immunofluorescence, where the culture of Crithidia luciliae is taken as an nDNA substrate.

With hron, polyarthritis and severe liver damage, positive reactions to rheumatoid factor can be detected in the Wohler-Rose reaction (see Rheumatoid arthritis) or latex agglutination (see Agglutination). It is also useful to study complement CH50 and its components, the decrease of which usually correlates with the activity of lupus nephritis. In almost all patients, ROE is significantly accelerated - up to 60-70 mm per hour. More than half of the patients have leukopenia (below 4000 in 1 μl) with a shift in the blood count to promyelocytes, myelocytes and young ones in combination with lymphopenia (5-10% of lymphocytes). Moderate hypochromic anemia is very often observed. In rare cases, hemolytic anemia develops with features of acquired hemolysis (see) and a positive Coombs reaction (see Coombs reaction). Thrombocytopenia (below 100,000 in 1 μl) is often observed, in rare cases - Werlhoff syndrome.

Thus, when establishing a diagnosis of systemic K. v. the whole wedge, the picture, the lab data should be taken into account. research methods and biopsy material of kidneys, synovium and skin.

For a more complete assessment of the patient’s condition, it is advisable to determine the degree of activity of the patol process. Klin, and lab. characterization of the degrees of activity of systemic To. v. is given in Table 1.

Treatment

Treatment started at the onset of the disease gives best effect. In the acute period, treatment is carried out in a hospital, where patients should be provided with adequate nutrition with sufficient amounts of vitamins B and C.

For individualization of treatment, a differentiated determination of the degrees of activity of the patol process is crucial (Table 1).

In case of patol, a process of III degree of activity, all patients, regardless of the course, are indicated for treatment with glucocorticosteroids in large doses (per day 40-60 mg of prednisolone or another drug in equivalent doses), in case of II degree - correspondingly smaller doses (30-40 mg per day). day), and for stage I - 15-20 mg per day. It is extremely important that the initial dose of glucocorticosteroids is sufficient to reliably suppress the activity of the patol process. Particularly large doses (50-60-80 mg per day of prednisolone) should be prescribed for nephrotic syndrome, meningoencephalitis and other diffuse processes in the nervous system - the so-called. lupus crisis. Treatment with glucocorticosteroids in the maximum dose is carried out until a pronounced effect occurs (according to a decrease in clinical and laboratory indicators of activity), and in case of nephrotic syndrome - for at least 2-3 months, then the dose of the hormone is slowly reduced, focusing on the proposed scheme (Table 2), but respecting the principle of individualization in order to prevent withdrawal syndrome or dose reduction syndrome.

Glucocorticosteroids should be prescribed in combination with potassium preparations, vitamins, anabolic hormones and symptomatic agents (diuretics, antihypertensives, ATP, cocarboxylase, etc.). When reducing their dose, salicylates, aminoquinolines and other drugs should be added. Treatment with hormones, as a rule, cannot be completely stopped due to rapidly developing deterioration of the condition (withdrawal syndrome), so it is important that the maintenance dose is minimal. The maintenance dose is usually 5-10 mg of the drug, but may be higher in case of unstable remission.

Such side symptoms that occur during treatment, such as Cushingoid, hirsutism, ecchymosis, stretch marks, acne, develop in many patients and do not require additional therapy. On the contrary, it is noted that persistent improvement in the condition usually occurs with the development of signs of hormone overdose. For persistent edema, diuretics, plasma and albumin transfusions may be recommended. Hypertension is relatively easily controlled with antihypertensive drugs.

Much more serious are complications such as steroid ulcers, exacerbations of focal infections, disorders of mineral metabolism with osteoporosis, etc., but they can also be prevented with systematic monitoring. An undoubted contraindication to continued treatment is steroid psychosis or increased seizures(epilepsy). Correction with psychotropic drugs is necessary.

If glucocorticosteroids are ineffective in patients with systemic K. v. treatment is prescribed with cytostatic immunosuppressants of the alkylating series (cyclophosphamide) or metabolites (azathioprine). Indications for the use of these drugs for systemic K. v. are: high (III) degree of disease activity involving many organs and systems, especially in adolescents; developed lupus nephritis (nephrotic and nephritic syndromes); the need to reduce the suppressive dose of glucocorticosteroid due to developed side effects this therapy.

Azathioprine (imuran) and cyclophosphamide are prescribed in doses of 1-3 mg per 1 kg of patient weight per day in combination with 10-40 mg of prednisolone per day to control extrarenal symptoms. Treatment with immunosuppressants should also be long-term, subject to regular medical supervision. Serious complications may develop during treatment with immunosuppressants, so monitoring of blood (including platelets) and urine is necessary, especially in the first 3 weeks. treatment. With inf. complications are treated with active antibiotic therapy. Other complications, including total alopecia, resolve when the dose of the immunosuppressant is reduced and symptomatic therapy is prescribed.

When hron, the course of systemic K. century. with predominant skin lesions of the discoid type. Recommend chloroquine, delagil or other quinoline drugs.

When signs of damage to internal organs subside and clinical and laboratory signs of activity decrease to degree I, treatment can be used. physical education and massage under the control of the general condition and condition of the internal organs. Physiotherapeutic and spa treatment for systemic K. v. not recommended due to the possibility of provoking the disease by ultraviolet irradiation, balneotherapy, and insolation.

Forecast

Prognosis for life with early recognition of systemic K. v. and adequate patol activity, the process of long-term treatment is satisfactory; 70-75% of patients return to active work at work and in the family. However, with the development of lupus nephritis, cerebrovasculitis and the addition of a secondary infection, the prognosis worsens.

Prevention

Prevention is aimed at preventing exacerbations and progression of the disease and the occurrence of the disease.

Prevention of disease progression (secondary) is carried out by timely, adequate, rational complex therapy, therefore patients should undergo regular medical examinations, take hormonal medications in a strictly prescribed dose, do not sunbathe or overcool, avoid surgical interventions, vaccinations, vaccines and serums (except for vital ones). In case of exacerbation of focal or intercurrent infection, bed rest, antibiotics, and desensitizing therapy are required. Treatment of focal infection should be persistent, predominantly conservative.

Measures primary prevention are especially important for family members of patients with systemic K. v., who have signs of photosensitivity, drug intolerance, disorders humoral immunity. To prevent the disease or generalization of the process, these people should avoid ultraviolet irradiation, treatment with radioisotope gold, spa treatment, etc.

Features of the course of systemic lupus erythematosus in children

Predominantly girls of prepubertal and pubertal age are affected. The rise in incidence begins from the 9th year of life, its peak occurs at 12-14 years. Sometimes systemic K. v. occurs in children 5-7 years old; Cases of illness in children in the first months of life are described as casuistic. There are no cases of congenital disease.

In the vast majority of cases in children and adolescents, systemic K. v. begins and proceeds more acutely and severely, giving a higher mortality rate than in adults. This is due to the peculiarities of the reactivity of the growing organism, the uniqueness of connective tissue structures, organs of immunogenesis, the complement system, etc. Generalization of pathol, the process in children develops much faster, and damage to various organs is characterized by a predominance of the exudative component of inflammation in combination with signs of an intensively developing syndrome of intravascular coagulation disorder in in the form of hemorrhages and bleeding, collaptoid, soporous and shock states, thrombocytopenia.

At the onset of the disease, children most often complain of joint pain, weakness, and malaise. Along with this, fever is noted, dystrophy increases quite quickly, often reaching cachexia, significant changes in the blood appear, signs of damage to many vital signs are revealed. important organs and systems.

Skin changes in manifestations typical of lupus are not always found. A combination of acute exudative and discoid changes is characteristic, as well as a tendency to merge individual lesions with the total spread of dermatitis, involving the entire skin and scalp. Hair falls out rapidly, which leads to alopecia areata or complete baldness, and breaks off, forming a kind of brush above the forehead line. The mucous membranes of the mouth, upper respiratory tract, and genitals may be affected. Nonspecific allergic manifestations in the form of urticaria and morbilliform rash or mesh-vascular pattern of the skin, as well as petechial-hemorrhagic elements are much more common and can be found in almost every patient in the active period of systemic K. v.

Articular syndrome, which is the most common and almost always one of the first signs of the disease, can be represented by arthralgia of a volatile nature, acute or subacute arthritis and periarthritis with mild ephemeral exudative manifestations. Articular syndrome is usually combined with damage to the tendon-muscular system, although myalgia and myositis are sometimes an independent sign of systemic K. v.

Involvement in patol, the process of serous membranes is observed in almost all cases; In the clinic, pleurisy and pericarditis are most often recognized, usually in combination with perihepatitis, perisplenitis, and peritonitis. Massive effusion in the pleura and pericardium, requiring repeated punctures, are characteristic manifestations of systemic K. v.

One of the most common visceral signs of systemic K. v. is carditis; its combination with arthritis in the early stages of the disease is almost always mistakenly interpreted as rheumatism. All three membranes of the heart can be affected, but in children and adolescents, symptoms of myocarditis predominate.

Lung lesions are diagnosed less frequently in the clinic than pleural lesions. Typical lupus pneumonitis is accompanied by an alveolar-capillary block, and percussion-auscultatory data are scanty, however, increasing hypoxia, phenomena of respiratory failure attract attention, confirm the presence of pneumonitis and rentgenol data.

Lupus nephritis occurs in children and adolescents more often than in adults (about 2/3 of cases) and in the vast majority of patients it is severe kidney damage with nephrotic syndrome, hematuria, a tendency to arterial hypertension, and is often accompanied by eclampsia. The nature of the course of lupus nephritis in children is close to mixed form hron, banal glomerulonephritis, often it is a variant of rapidly progressing glomerulonephritis and only in some patients it occurs in the form of minimal urinary syndrome.

Damage to the central and peripheral nervous system, generally similar to that in adults, includes a chorea-like syndrome with all the wedge, features inherent in minor chorea (see).

Quite often there are signs of damage to the colon. tract. Abdominal pain can be caused by intestinal damage, the development of peritonitis, perisplenitis, perihepatitis, as well as hepatitis and pancreatitis. Before establishing the diagnosis of systemic K. v. abdominal crises can be taken for granted acute appendicitis, cholecystitis, ulcerative colitis, dysentery, etc. Sometimes a picture of an acute abdomen develops (see). A symptom complex of malignant ongoing Crohn's disease is possible. The active period of the disease is accompanied by an increase in peripheral lymph nodes, sometimes so significant that in order to differential diagnosis their puncture or biopsy is required.

In 2/3 of sick children and adolescents, systemic K. v. develops acutely or subacutely; There may also be cases of the most acute course of the disease, which is characterized by rapid development of hyperergic reactions, high fever of the wrong type and other signs (damage to the skin, joints, lymph nodes), hemorrhagic diathesis, damage to the nervous system. Rapidly progressing vasculitis in a short time leads to severe inflammatory-destructive and dystrophic changes internal organs (heart, kidneys, lungs), with disruption of their functions and possible death in the first 3-9 months. from the onset of the disease. Death in such cases most often occurs due to symptoms of cardiopulmonary and (or) renal failure due to intoxication, deep violations homeostasis, coagulopathic disorders, water and electrolyte imbalance, as well as the addition of a secondary infection.

With subacute systemic K. v., moderate in severity and duration, generalization of the process occurs in the first 3-6 months. from the onset of the disease, the course is persistent or wavy with constantly remaining signs of activity and relatively quickly joining function. inferiority of one or another organ.

Approximately 1/3 of children have a variant of primary chronic course disease, close to the picture of classical systemic K. v. adults, with a pre-systemic period lasting from one to 3 years, and with subsequent generalization of the process. Pre-systemic lupus manifestations in children most often include hemopathy, hemorrhagic and nephritic syndromes, arthropathy, and chorea. Other rarer monosyndromes are also possible.

Complications and diagnostic methods are the same as in adults.

Each child with pronounced clinical and laboratory signs of systemic To. v. activity. should be treated in a hospital setting. Corticosteroids and cytostatics are used to suppress immune hyperactivity. The size of their daily dose is determined not only by the age of the child, but also by the degree of activity of the patol process. For grade III activity with symptoms of nephritis, carditis, serositis, neurolupus, large doses of corticosteroids are prescribed (prednisolone at the rate of 1.25-2 mg or more per 1 kg of patient weight per day). If the indicated dose of prednisolone or an equivalent amount of a similar drug cannot be given to the patient, azathioprine or cyclophosphamide must be introduced into therapy at a rate of at least 1 - 3 mg per 1 kg per day. In case of nephrotic syndrome, autoimmune hemolytic anemia, hemorrhagic syndrome and crisis conditions, in all cases, combination immunosuppressive therapy in combination with heparin (250-600 units per 1 kg of body weight per day) is carried out from the very beginning. Upon achieving a clear clinical and laboratory improvement in the patient’s condition, the maximum immunosuppressive dose of prednisolone should be reduced (Table 2), heparin should be replaced with antiplatelet agents (chimes) and (or) indirect anticoagulants.

With a moderate degree of systemic activity. The immunosuppressive dose of corticosteroids should be lower (prednisolone - 0.5-1.2 mg per 1 kg of body weight per day), instead of heparin, chimes are prescribed at 6-8 mg per 1 kg of body weight per day, salicylates, quinoline drugs, and methindole are used more widely . With hron, current and low degree of activity of systemic K. v. in the absence of clear symptoms of damage to the kidneys, blood, nervous system, heart, lungs, corticosteroids are prescribed in small doses (prednisolone - less than 0.5 mg per 1 kg of body weight per day) or not used at all.

After discharge from the hospital, children are under the supervision of a rheumatologist and continue to receive supportive immunosuppressive and symptomatic therapy. During the first year after acute period systemic K. v. It is not recommended to attend school, but home education can be arranged. It is necessary to cancel all scheduled preventive vaccinations.

With adequate treatment of patients, it is increasingly possible to achieve relative or complete remission. At the same time, the general physical Children's development is progressing more or less satisfactorily, secondary sexual characteristics appear in a timely manner, and girls begin menstruation on time. Mortality is most often associated with renal failure.

Discoid lupus erythematosus

Discoid K. v. (syn.: lupus erythematodes discoides s. chronicus, erythematodes, seborrhea congestiva, erythema atrophicans etc.) - the most common chronic form K. v., when the cut is dominant in the picture of the disease is damage to the skin and mucous membranes. The name “lupus erythematodes” was proposed by P. Cazenave in 1851, believing that the disease was a type of tuberculous lupus. It was first described by R. F. Rayer in 1827 as a rare form of sebaceous discharge (fluxus sebaceus). Discoid K. v. accounts for 0.25-1% of all dermatoses (M.A. Agronik et al.), more often found in countries with cold, humid climates, mainly in middle-aged people [Gertler (W. Gertler)]. Women get sick more often than men.

Etiology

The etiology has not been definitively established. The origin of the disease is assumed to be viral. Electron microscopy reveals tubuloreticular cytoplasmic inclusions in skin lesions.

Pathogenesis

In the pathogenesis of individual cases of the disease, genetic and immunol factors are important. In provoking discoid K. v. and its exacerbations important role Excessive insolation, medications, various types of injuries (mechanical, thermal, chemical) play a role.

Pathological anatomy

Discoid K. v. and its disseminated form is limited to skin changes. With discoid K. v. the lesion is most often localized on the face. Microscopically (Fig. 9) hyperkeratosis is found (see), keratosis pilaris, vacuolar dystrophy of the epidermis (see Vacuolar dystrophy), acanthosis (see). Focal lymphoid-macrophage infiltrates with an admixture of neutrophils and plasma cells are visible in the dermis. The walls of blood vessels are impregnated with plasma proteins. The collagen fibers of the dermis are swollen, picrinophilic, and merge into fibrinoid masses. In the infiltration zone, elastic and collagen fibers are destroyed. During treatment, scarring occurs with atrophy and depigmentation of the skin.

For disseminated cutaneous form K.v. characterized by multiple rashes throughout the body, in which microscopic changes resemble those of discoid K. v., but are less pronounced, exudative reactions predominate over proliferative ones, cellular infiltration is less significant. The result is no scars or areas of skin atrophy.

Clinical picture

Discoid K. v. begins with the appearance of one or two pink, slightly swollen spots, which gradually increase in size, infiltrate, and become covered in the central zone with tightly packed whitish scales. Scraping the lesions causes pain (Besnier-Meshchersky symptom), because on the underside of the scale there is a horny spine (ladies heel symptom), which is fixed in the expanded mouth of the hair follicle. Subsequently, cicatricial atrophy develops in the central part of the lesion. In a long-existing lesion, three zones are clearly distinguished: a central atrophic zone, then a hyperkeratotic zone and an erythematous zone bordering it (tsvetn. Fig. 4). Within the latter there are often telangiectasia (see). Brown hyperpigmentation may be expressed to varying degrees along the periphery of the lesion. Erythema (see), hyperkeratosis and skin atrophy (see) are the cardinal symptoms of K. v. Infiltration, telangiectasia and pigmentation are common but not obligatory signs.

Rice. 5. Lupus “butterfly” on the face of a patient with discoid skin lesions.

The most typical localization of discoid K. v. is in areas of the skin exposed to insolation: face, ch. arr. its middle part is the noe, cheeks, zygomatic, preauricular areas. As well as for systemic K. v., the so-called butterfly (color fig. 5) - the lesion is on the back of the nose and cheeks. According to I.I. Lelis, who observed 518 patients, the primary foci of K. v. were located on the nose in 48%, on the cheeks in 33%, on the ears or adjacent skin - in 22.5%, on the forehead - in 16.5%, on the scalp - in 10%, on the red border lips, usually lower - in 12.5%, on the mucous membrane of the mouth - in 7%. Damage to the mucous membrane of the eyelids L. I. Mashkilleyson et al. observed in 3.4% of patients. More rare, including isolated localizations are known - on the chest, back, shoulders, etc. Lesions of the mucous membrane of the genitals, bladder, cornea, and lesions of the nails are described. Along with typical discoid K. v. There are its varieties: hyperkeratotic K. v., with a cut hyperkeratosis is pronounced; papillomatous discoid K. v. - increased proliferation of dermal papillae, leading to the formation of a villous surface of the lesions; warty K. v. - papillomatosis is accompanied by severe keratinization; pigmented K. v. - excessive deposition of pigment that colors the lesions in dark brown color; seborrheic K. v. - hair follicles are greatly expanded and filled with fatty, loose scales; tumor-like K. v. - bluish-red, highly elevated foci with edematous, clearly defined edges, mild hyperkeratosis and atrophy.

Rare varieties are telangiectatic discoid K. v. with multiple telangiectasias, hemorrhagic discoid K. v. with hemorrhages in the foci, mutilating. A special form of hron. K.v. is centrifugal erythema (erythema centrifugum Biett). It accounts for 5.2-11% in relation to all forms of K. v., characterized by clearly demarcated foci of erythema on the face, less often on other areas of the skin. They may have telangiectasia and slight swelling. There is no hyperkeratosis. Atrophy is absent or mild. Centrifugal erythema responds fairly quickly to treatment, but recurs easily. Some authors classify it, along with disseminated K. v., as forms intermediate between discoid and systemic.

In the foci of discoid K. v. On the oral mucosa, dark red erythema, telangiectasias, stripe-like, coarse network-like areas of epithelial opacification, erosion, and superficial ulcerations are observed. On the red border of the lips K. v. has the appearance of irregularly oval ribbon-like foci of erythema and hyperkeratosis, sometimes with cracks and erosions. Foci of discoid K. v. more often single, less often multiple. Without treatment, they exist for years and, as a rule, do not cause discomfort. Erosive and ulcerative rashes in the mouth cause pain. They occur especially persistently in smokers. Disseminated discoid K. v. characterized by scattered erythematous-edematous, papular elements or discoid-type lesions. Predominant localization: face, open part of the chest and back, hands, feet, skin over the elbows and knee joints. The general condition of patients with discoid and disseminated K. v., as a rule, does not noticeably suffer. However, during wedge examination, 20-50% of patients reveal arthralgia, functional disorders, disorders of the internal organs (heart, stomach, kidneys), nervous system, accelerated ROE, leukopenia, hypochromic anemia, changes in the composition of immunoglobulins, antinuclear antibodies, immune complexes in the area of ​​the dermoepidermal junction, etc.

Deep K. v. (L. e. profundus Kaposi - Irgang) is characterized by the simultaneous presence of typical skin lesions characteristic of discoid K. v., and nodes in the subcutaneous tissue, the skin over which is mostly unchanged. A number of authors, eg. Pautrier (L. M. Pautrier), consider this form as a combination of deep Darrieus-Russi sarcoids and discoid K. v.

Complications

Occasionally, skin cancer develops, mainly in lesions on the red border of the lower lip, very rarely - sarcoma, erysipelas; a serious complication, more often observed with disseminated discoid K. v., is its transition to systemic K. v. under the influence of unfavorable factors.

Diagnosis

The diagnosis in typical cases is established without difficulty. Foci of discoid K. v. may be similar to seborrheic eczema, rosacea, psoriasis, eosinophilic granuloma of the face, tuberculous lupus. Clear boundaries of lesions, horn plugs in dilated hair funnels, tightly fitting scales, positive symptom Besnier-Meshchersky, the development of atrophy indicates the presence of K. v. Foci of seborrheic eczema (see) do not have such sharp boundaries, their surface is covered with loose, fatty scales, they respond well to antiseborrheic therapy. Psoriatic lesions are usually numerous, covered with easily scraped off silvery scales (see Psoriasis). Both of them, in contrast to K. v. usually decrease under the influence of sunlight. With rosacea (see) there is diffuse erythema, telangiectasia is pronounced, nodules and pustules often appear. Eosinophilic granuloma of the face (see) is characterized by particular resistance to therapeutic effects. Its foci are often single, uniform brown-red color, without hyperkeratosis, with isolated telangiectasias. Tuberculous lupus (see Tuberculosis of the skin) usually begins in childhood, it is characterized by the presence of lupoma with the characteristic apple jelly and probe phenomena. In cases of erythematous tuberculous lupus erythematosus of Leloir, the wedge, the diagnosis is extremely difficult, a histol examination is necessary. Discoid K. v. should also be differentiated with Essner-Kanoff lymphocytic infiltration; manifestations of the cut are less persistent, tend to resolve in the center, lack of peeling, hyperkeratosis, and atrophy. K.v. on the scalp differentiated from pseudopelade (see). The latter is characterized by the absence of inflammation, horny spines, finger-like arrangement, and more superficial atrophy. Discoid K. v. on the oral mucosa should be distinguished from lichen planus, the rashes of which have a more delicate pattern and are not accompanied by atrophy.

Patients with discoid K. v., including limited forms, should be examined to exclude systemic damage to internal organs and the nervous system, as well as to identify concomitant diseases.

Treatment

Leading role in the treatment of discoid and disseminated K. v. belongs to aminoquinoline drugs - chloroquine, resokhin, delagil y, plaquenil y, etc. They are prescribed continuously or in cycles, usually 0.25 g 2 times, Plaquenil - 0.2 g 3 times a day after meals. The duration of cycles (5-10 days) and the intervals between them (2-5 days) depends on the tolerability of treatment. Repeated courses of treatment are recommended, especially in spring. Adding small doses of corticosteroids (2-3 tablets of prednisolone per day) to chloroquine improves treatment results and tolerability. This technique is recommended for particularly persistent cases of K. v., extensive skin lesions.

It is useful to include vitamins B6, B12, calcium pantothenate, and nicotinic acid in the therapeutic complex. Treatment the effect occurs faster with the simultaneous administration of ointments with fluoride-containing corticosteroids (sinalar, flucinar, etc.), which, in case of limited lesions, can be the main method of therapy. It is also recommended to administer intradermally into the affected areas 5% chloroquine solution once every 5-7 days (4-6 injections per course). Limited lesions with a strong infiltrate and hyperkeratosis without signs of peripheral growth can be subjected to cryotherapy.

Forecast

The prognosis for life is favorable. With adequate treatment and patients following the recommended regimen, their ability to work remains for many years.

Prevention

Patients K. v. subject to medical examination. They must comply with the gig. mode of work, rest, nutrition, avoid physical activity. and nervous overload, exposure to the sun, wind, frost, use photoprotective creams and films with para-aminobenzoic acid, tannin, etc. It is necessary to sanitize foci of focal infection. For the treatment of concomitant diseases of patients with K. v. should not be directed to the south. resorts in the spring and summer, they should be prescribed physiotherapeutic procedures with caution, and vaccinated only for serious indications.

Drug-induced lupus erythematosus

Medicinal K. v. develops in connection with long-term use of apressin (hydralazine), procainamide (procainamide), diphenine (hydantoin), trimethine (trimethadione), carbazepine, isoniazid and chlorpromazine. Medicinal K. v. can develop in elderly people suffering from hypertension and arrhythmia, in patients with tuberculosis and epilepsy. The listed drugs are capable of causing the formation of antinuclear antibodies (ANF, antibodies to DNA), the appearance of which precedes the clinical manifestations of medicinal K. v., reminiscent of systemic K. v. When taking certain medications, a certain wedge or syndrome occurs. So, with apressin K. v. glomerulonephritis develops; with long-term use of nicotinamide, pleurisy and pneumonitis are very common, which are the beginning of the syndrome.

Among the mechanisms of development of medicinal To. v. The role of predisposition is discussed, since such a reaction occurs in approximately 10% of patients taking apressin and other drugs, as well as metabolic disorders, in particular the rate of acetylation of these drugs.

The diagnosis is made based on taking the listed medications.

Timely recognition of the disease and discontinuation of the drug that caused medicinal K. v. leads to recovery, however, it may be necessary to prescribe corticosteroids in medium doses (20-30 mg of prednisolone per day), especially with isoniazid medicinal K. v. With the development of the clinic of systemic K. century. appropriate therapeutic tactics are necessary.

Tables

Table 1. Clinical and laboratory indicators of the degree of activity of systemic lupus erythematosus

Table 2. Approximate scheme for reducing the dose of prednisolone depending on the initial (maximum) dose

Bibliography: Vinogradova O. M. Systemic lupus erythematosus in the clinic of internal diseases, Sov. med., No. 4, p. 15, 1958; Guseva L. L. and Luninskaya I. R. Psychopathological manifestations in systemic lupus erythematosus, Zhurn, neuropath, and psychiat., t. 75, century. 4, p. 562, 1975, bibliogr.; Davydovsky I.V. On the issue of lupus erythematodes disseminatus acutus, Rus. Vestn. derm., vol. 7, no. 5, p. 450, 1929, bibliogr.; And Smailov T. I. and F r u m k i-n and S. L. On the psychopathology and pathogenesis of symptomatic psychosis in systemic lupus erythematosus, Zhurn, neuropath, and psychiat., t. 72, no. 12, p. 1860, 1972; L e l and with I. I. Lupus erythematosus, L., 1970, bibliogr.; Meshchersky G.I. and Grinchar F.N. About the case of erythema faciei perstans (Kaposi - Kreibich’a) of tuberculous origin, Kharkov. pathologist Sat., dedicated. prof. M. N. Nikiforov, on the 25th anniversary of his scientist, activity, p. 406, M., 1911; Nasonova V. A. Systemic lupus erythematosus, M., 1972, bibliogr.; S e r about in V.V. and others. Immunomorphological characteristics of skin changes in lupus erythematosus, Soz. med., No. 9, p. 15, 1972; S e r about in V.V. and others. Electron microscopic characteristics of lupus nephritis, Arch. pathol., t. 36, no. 6, p. 21, 1974, bibliogr.; S to r i p k i n Yu. K., Somov B. A. and B u t o v Yu. S. Allergic dermatoses, p. 130, M., 1975, bibliogr.; With t r u-k about in A. I. and B e g l a r I n A. G. Pathological anatomy and pathogenesis of collagen diseases, p. 248, M., 1963; Tare-e in E. M. Collagenoses, M., 1965, bibliogr.; Tareeva I.E. Lupus nephritis, M., 1976, bibliogr.; Tareeva I. E., Serov V. V. and Kupriyanova L. A. Intraendothelial inclusions in systemic lupus erythematosus, Bull. Experiment, biol, and med., v. 77, no. 5, p. 119, 1974; O' C o n n o r J. F. a. Musher D. M. Central nervous system involvement in systemic lupus erythematosus, Arch. Neurol. (Chic.), v. 14, p. 157, 1966; Hargraves M. M., Richmond H. a. M o r t o n R. Presentation of two bone marrow elements, the “tart” cell and the “L. E." cell, Proc. Mayo Clin., v. 23, p. 25, 1948; Klemperer P., Pollack A. D. a. Baehr G. Pathology of disseminated lupus erythematosus, Arch. Path., v. 32, p. 569, 1941; Lupus erythematosus, ed. by E. L. Dubois, Los Angeles, 1974; Recent advances in rheumatology, ed. by W. W. Buchanan a. W. C. Dick, pt 1, Edinburgh -L., 1976; Ropes M. W. Systemic lupus erythematosus, Cambridge - L., 1976, bibliogr.

V. A. Nasonova; L. A. Isaeva (ped.), A. I. Strukov, L. V. Kaktursky (pat. an.), A. S. Tiganov (psychiat.), L. Ya. Trofimova (derm.).

This is an autoimmune pathology that affects the kidneys, blood vessels, connective tissues and other organs and systems. If, under normal conditions, the human body produces antibodies that can attack foreign organisms entering from the outside, then in the presence of a disease, the body produces a large number of antibodies to the body’s cells and their components. As a result, an immune complex inflammatory process is formed, the development of which leads to dysfunction of various elements of the body. Systemic lupus affects internal and external organs, including:

• lungs;
• kidneys;
• skin;
• heart;
• joints;
• nervous system.

Causes

The etiology of systemic lupus still remains unclear. Doctors suggest that the cause of the disease is viruses (RNA, etc.). In addition, a risk factor for the development of pathology is a hereditary predisposition to it. Women suffer from lupus erythematosus about 10 times more often than men, which is explained by the characteristics of their hormonal system (there is a high concentration of estrogen in the blood). The reason why the disease occurs less frequently in men is the protective effect of androgens (male sex hormones). The following may increase the risk of SLE:

• bacterial infection;
• taking medications;
• viral infection.

Development mechanism

A normally functioning immune system produces substances to fight antigens of any infections. In systemic lupus, antibodies deliberately destroy the body's own cells, and they cause absolute disorganization of connective tissue. Typically, patients exhibit fibroid changes, but other cells are susceptible to mucoid swelling. In the affected structural units of the skin, the core is destroyed.

In addition to damage to skin cells, plasma and lymphoid particles, histiocytes, and neutrophils begin to accumulate in the walls of blood vessels. Immune cells settle around the destroyed nucleus, which is called the “rosette” phenomenon. Under the influence of aggressive complexes of antigens and antibodies, lysosome enzymes are released, which stimulate inflammation and lead to damage to connective tissue. New antigens with antibodies (autoantibodies) are formed from destruction products. As a result of chronic inflammation, tissue sclerosis occurs.

Forms of the disease

Depending on the severity of the symptoms of the pathology, systemic disease has a certain classification. Clinical types of systemic lupus erythematosus include:

1. Acute form. At this stage, the disease progresses sharply, and the patient’s general condition worsens, while he complains of constant fatigue, high temperature (up to 40 degrees), pain, fever and muscle aches. The symptoms of the disease develop quickly, and within a month it affects all human tissues and organs. The prognosis for the acute form of SLE is not comforting: often the life expectancy of a patient with such a diagnosis does not exceed 2 years.
2. Subacute form. It may take more than a year from the onset of the disease to the onset of symptoms. Characteristic for this type of disease frequent change periods of exacerbation and remission. The prognosis is favorable, and the patient’s condition depends on the treatment chosen by the doctor.
3. Chronic. The disease is sluggish, the symptoms are mild, the internal organs are practically undamaged, so the body functions normally. Despite the mild course of the pathology, it is virtually impossible to cure it at this stage. The only thing that can be done is to alleviate a person’s condition with the help of medications during an exacerbation of SLE.

It should be distinguished skin diseases, related to lupus erythematosus, but not systemic and not having generalized lesions. Such pathologies include:

• discoid lupus (a red rash on the face, head, or other parts of the body that is slightly raised above the skin);
• drug-induced lupus (inflammation of the joints, rash, high fever, pain in the sternum associated with taking medications; symptoms go away after they are discontinued);
• neonatal lupus (rarely expressed, affects newborns when mothers have diseases of the immune system; the disease is accompanied by liver abnormalities, skin rashes, and heart pathologies).

How does lupus manifest?

The main symptoms of SLE include severe fatigue, skin rash, and joint pain. As the pathology progresses, problems with the functioning of the heart, nervous system, kidneys, lungs, and blood vessels become relevant. The clinical picture of the disease in each specific case is individual, since it depends on which organs are affected and what degree of damage they have.

On the skin

Tissue damage appears at the onset of the disease in approximately a quarter of patients; in 60-70% of patients with SLE, the skin syndrome is noticeable later, and in the rest it does not occur at all. As a rule, the localization of the lesion is characterized by areas of the body exposed to the sun - the face (butterfly-shaped area: nose, cheeks), shoulders, neck. The lesions are similar to erythematosus in that they appear as red, scaly plaques. At the edges of the rash there are dilated capillaries and areas with excess/lack of pigment.

In addition to the face and other areas of the body exposed to sunlight, systemic lupus affects the scalp. As a rule, this manifestation is localized in the temporal region, with hair falling out in a limited area of ​​the head (local alopecia). In 30-60% of SLE patients, increased sensitivity to sunlight (photosensitivity) is noticeable.

In the kidneys

Very often, lupus erythematosus affects the kidneys: in about half of patients, damage to the renal apparatus is determined. A common symptom This is due to the presence of protein in the urine; casts and red blood cells are usually not detected at the onset of the disease. The main signs that SLE has affected the kidneys are:

• membranous nephritis;
• proliferative glomerulonephritis.

In the joints

Rheumatoid arthritis is often diagnosed with lupus: in 9 out of 10 cases it is non-deforming and non-erosive. More often the disease affects the knee joints, fingers, and wrists. In addition, patients with SLE sometimes develop osteoporosis (low bone density). Patients often complain of muscle pain and muscle weakness. Immune inflammation is treated with hormonal medications (corticosteroids).

On mucous membranes

The disease manifests itself on the mucous membrane of the oral cavity and nasopharynx in the form of ulcers that do not cause pain. Damage to the mucous membranes is recorded in 1 out of 4 cases. This is typical for:

• decreased pigmentation, red border of the lips (cheilitis);
• ulcerations of the oral cavity/nasal cavity, pinpoint hemorrhages.

On vessels

Lupus erythematosus can affect all structures of the heart, including the endocardium, pericardium and myocardium, coronary vessels, and valves. However, damage to the outer lining of the organ occurs more often. Diseases that can result from SLE:

• pericarditis (inflammation of the serous membranes of the heart muscle, manifested dull pain in the chest area);
• myocarditis (inflammation of the heart muscle, accompanied by disturbances in rhythm, nerve impulse conduction, acute/chronic organ failure);
• heart valve dysfunction;
• damage to the coronary vessels (can develop at an early age in patients with SLE);
• damage to the inside of blood vessels (this increases the risk of developing atherosclerosis);
• damage to the lymphatic vessels (manifested by thrombosis of the extremities and internal organs, panniculitis - painful subcutaneous nodes, livedo reticularis - blue spots forming a mesh pattern).

On the nervous system

Doctors suggest that the failure of the central nervous system is caused by damage to the blood vessels of the brain and the formation of antibodies to neurons - cells that are responsible for nourishing and protecting the organ, as well as to immune cells (lymphocytes. The key signs that the disease has affected the nervous structures of the brain are :

• psychosis, paranoia, hallucinations;
• migraine, headaches;
• Parkinson's disease, chorea;
• depression, irritability;
• brain stroke;
• polyneuritis, mononeuritis, aseptic meningitis;
• encephalopathy;
• neuropathy, myelopathy, etc.

Symptoms

The systemic disease has an extensive list of symptoms, and is characterized by periods of remission and complications. The onset of pathology can be immediate or gradual. Signs of lupus depend on the form of the disease, and since it belongs to the multiorgan category of pathologies, clinical symptoms can be varied. Mild forms of SLE are limited only to damage to the skin or joints; more severe types of the disease are accompanied by other manifestations. Typical symptoms of the disease include:

• swollen eyes, joints of the lower extremities;
• muscle/joint pain;
• enlarged lymph nodes;
• hyperemia;
• increased fatigue, weakness;
• red, allergic-like rashes on the face;
• causeless fever;
• blueness of fingers, hands, feet after stress, contact with cold;
• alopecia;
• pain when inhaling (indicates damage to the lining of the lungs);
• sensitivity to sunlight.

First signs

Early symptoms include temperature, which fluctuates within degrees and can last for several months. After this, the patient develops other signs of SLE, including:

• arthrosis of small/large joints (may go away on its own, and then reappear with greater intensity);
• a butterfly-shaped rash on the face, rashes also appear on the shoulders and chest;
• inflammation of the cervical and axillary lymph nodes;
• In case of severe damage to the body, internal organs - kidneys, liver, heart - suffer, which results in disruption of their functioning.

In children

At an early age, lupus erythematosus manifests itself with numerous symptoms, progressively affecting different organs of the child. At the same time, doctors cannot predict which system will fail next. Primary signs pathologies may resemble common allergies or dermatitis; This pathogenesis of the disease causes difficulties in diagnosis. Symptoms of SLE children may have:

• dystrophy;
• thinning of the skin, photosensitivity;
• fever accompanied by profuse sweating and chills;
• allergic rashes;
• dermatitis, as a rule, is first localized on the cheeks, bridge of the nose (looks like warty rashes, blisters, swelling, etc.);
• joint pain;
• brittle nails;
• necrosis on the fingertips, palms;
• alopecia, up to complete baldness;
• convulsions;
• mental disorders (nervousness, moodiness, etc.);
• stomatitis that cannot be treated.

Diagnostics

To make a diagnosis, doctors use a system developed by American rheumatologists. To confirm that a patient has lupus erythematosus, the patient must have at least 4 of the 11 listed symptoms:

• erythema on the face in the shape of butterfly wings;
• photosensitivity (pigmentation on the face that worsens when exposed to sunlight or UV radiation);
• discoid rash on the skin (asymmetrical red plaques that peel and crack, with areas of hyperkeratosis having jagged edges);
• symptoms of arthritis;
• formation of ulcers on the mucous membranes of the mouth and nose;
• disturbances in the functioning of the central nervous system - psychosis, irritability, tantrums for no reason, neurological pathologies, etc.;
• serous inflammation;
• frequent pyelonephritis, the appearance of protein in the urine, the development of renal failure;
• false positive reaction of the Wasserman test, detection of titers of antigens and antibodies in the blood;
• reduction of platelets and lymphocytes in the blood, changes in its composition;
• causeless increase in antinuclear antibody levels.

The specialist makes a final diagnosis only if four or more signs from the list are present. When the verdict is in doubt, the patient is referred for a highly focused, detailed examination. When diagnosing SLE, the doctor plays a major role in collecting anamnesis and studying genetic factors. The doctor must find out what diseases the patient had during the last year of life and how they were treated.

Treatment

SLE is a chronic disease in which complete cure of the patient is impossible. The goals of therapy are to reduce the activity of the pathological process, restore and maintain the functionality of the affected system/organs, prevent exacerbations to achieve longer duration life of patients and improving their quality of life. Treatment of lupus involves the mandatory use of medications, which are prescribed by the doctor to each patient individually, depending on the characteristics of the body and the stage of the disease.

Patients are hospitalized in cases where they have one or more of the following clinical manifestations of the disease:

• suspicion of stroke, heart attack, severe damage to the central nervous system, pneumonia;
• an increase in temperature above 38 degrees for a long time (the fever cannot be eliminated with the help of antipyretics);
• depression of consciousness;
• a sharp reduction in leukocytes in the blood;
• rapid progression of disease symptoms.

If the need arises, the patient is referred to specialists such as a cardiologist, nephrologist or pulmonologist. Standard treatment for SLE includes:

• hormonal therapy (glucocorticoid drugs are prescribed, for example, Prednisolone, Cyclophosphamide, etc.);
• anti-inflammatory medications (usually Diclofenac in ampoules);
• antipyretics (based on Paracetamol or Ibuprofen).

To relieve burning and peeling of the skin, the doctor prescribes creams and ointments based on hormonal agents to the patient. During the treatment of lupus erythematosus, special attention is paid to maintaining the patient’s immunity. During remission the patient is prescribed complex vitamins, immunostimulants, physiotherapeutic manipulations. Drugs that stimulate the immune system, such as Azathioprine, are taken only during the lull of the disease, otherwise the patient’s condition may worsen sharply.

Acute lupus

Treatment should begin in the hospital as early as possible. The therapeutic course should be long and constant (without breaks). During the active phase of the pathology, the patient is given glucocorticoids in large doses, starting with 60 mg of Prednisolone and increasing by another 35 mg over 3 months. Reduce the volume of the drug slowly, switching to tablets. Afterwards, a maintenance dose of medication (5-10 mg) is prescribed individually.

To prevent disturbances in mineral metabolism, potassium preparations (Panangin, potassium acetate solution, etc.) are prescribed simultaneously with hormonal therapy. After finishing acute phase The disease is treated with complex corticosteroids in reduced or maintenance doses. In addition, the patient takes aminoquinoline medications (1 tablet of Delagin or Plaquenil).

Chronic

The earlier treatment is started, the greater the patient’s chances of avoiding irreversible consequences in the body. Therapy for chronic pathology necessarily includes taking anti-inflammatory drugs, drugs that suppress the activity of the immune system (immunosuppressants) and corticosteroid hormonal drugs. However, only half of patients achieve success in treatment. In the absence of positive dynamics, stem cell therapy is performed. As a rule, there is no autoimmune aggression after this.

Why is lupus erythematosus dangerous?

Some patients with this diagnosis develop severe complications - the functioning of the heart, kidneys, lungs, and other organs and systems is disrupted. The most dangerous form of the disease is systemic, which even damages the placenta during pregnancy, resulting in fetal growth retardation or death. Autoantibodies can cross the placenta and cause neonatal (congenital) disease in the newborn. At the same time, the baby develops a skin syndrome that goes away after 2-3 months.

How long do people live with lupus erythematosus?

Thanks to modern medications, patients can live more than 20 years after diagnosis of the disease. The process of development of pathology occurs at different speeds: in some people, symptoms increase in intensity gradually, in others they increase quickly. Most patients continue to lead their usual lifestyle, but severe course illness, ability to work is lost due to severe joint pain, high fatigue, central nervous system disorders. The duration and quality of life in SLE depends on the severity of symptoms of multiple organ failure.

Video

The information presented on the site is for informational purposes only. The site materials do not call for self-treatment. Only a qualified doctor can make a diagnosis and make recommendations for treatment based on the individual characteristics of a particular patient.

Lupus erythematosus - symptoms

Lupus erythematosus is an inflammatory disease of an autoimmune nature. It occurs against the background of a malfunction of the immune system, in which, for reasons unknown to medicine, it begins to kill the maples of its own body, perceiving them as foreign. Wherein immune system special antibodies are produced, under the influence of which the internal organs of the patient are seriously affected.

There are three forms of lupus erythematosus - cutaneous or discoid, systemic and drug-induced.

Lupus erythematosus manifests symptoms in the form of patches of redness of the skin, which in ancient times people compared to wolf bites, hence the name of the disease. Skin damage is worsened by exposure to sunlight.

Discoid lupus erythematosus - symptoms

The first symptoms of discoid lupus erythematosus appear as small pinkish spots in the lips and oral mucosa. These spots gradually change shape, merge with each other, increasing in size and affecting larger areas of the skin. They are mainly localized on open areas of the skin, including those covered with hair, exposed to sunlight - arms, head, neck, upper back.

Discoid lupus erythematosus does not affect internal organs, but creates an ugly cosmetic effect on the surface of the skin. It can become more serious systemic form lupus erythematosus.

Systemic lupus erythematosus - symptoms

The first symptoms of systemic lupus erythematosus are very vague, common to many other diseases. This:

• malaise;
• slight increase in temperature;
• headache;
• decreased appetite;
• sleep disturbance.

Red spots may also appear in the area of ​​the nail plate, pain in the joints and muscles.

More serious symptoms of systemic lupus erythematosus are pathological changes in muscles, joints, internal organs, in particular in the liver and heart. Also, lupus erythematosus manifests symptoms by affecting the nervous system. In this case, the patient may experience epileptic convulsions, inflammation of the meninges, neuroses, depression, and other mental illnesses.

The composition of the blood changes, namely, the amount of hemoglobin and leukocytes may decrease. In almost half of patients with lupus erythematosus, the presence of special antibodies is detected in the blood - antiphospholipids, which react with cell membranes (containing phospholipids) and affect blood clotting. Patients whose blood contains antiphospholipids very often suffer from thrombosis of veins and arteries, which provoke cardiac or cerebral strokes.

External manifestations of systemic lupus erythematosus appear in the form of rashes on the face, so-called exudative erythema in the shape of a butterfly, rashes can also appear on the cheekbones. But very often the skin remains untouched, only the internal organs and systems of the body are affected.

- symptoms

Drug-induced lupus erythematosus occurs due to long-term use of certain medications prescribed for the treatment of cardiac arrhythmia. It manifests itself in the form of redness of the skin, arthritis, and damage to lung tissue.

As lupus disease worsens, symptoms may expand. Thus, the patient may begin to rapidly lose weight, lose hair in clumps, and his lymph nodes may swell.

As you can see, lupus erythematosus disease has symptoms that affect almost all organs and systems of the body. As the disease progresses, the symptoms worsen, and other serious pathologies and diseases develop. Therefore, having diagnosed lupus erythematosus, you need to begin treatment as quickly as possible.

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/ Lupus

Lupus (systemic lupus erythematosus, SLE) is an autoimmune disease in which the human immune system attacks the host's connective tissue cells as foreign. Connective tissue is found almost everywhere, and most importantly, in the ubiquitous vessels. Inflammation caused by lupus can affect a variety of organs and systems, including the skin, kidneys, blood, brain, heart, and lungs. Lupus is not spread from person to person. Science does not know the exact cause of lupus, like many other autoimmune diseases. These diseases are most likely caused by genetic disorders in the immune system that make it possible for it to produce antibodies against its own host. Lupus is difficult to diagnose because its symptoms are varied and it can masquerade as other diseases. The most distinctive sign of lupus is erythema on the face that resembles butterfly wings spread across both cheeks of the patient (butterfly erythema). But this symptom does not occur in all cases of lupus. There is no definitive treatment for lupus, but its symptoms can be controlled with medications.

Causes and Risk Factors of Lupus

A combination of external factors can push the autoimmune process forward. Moreover, some factors affect one person, but do not affect another. Why this happens remains a mystery. There are many possible causes of lupus: Exposure to ultraviolet rays (sunlight) may cause lupus to develop or worsen lupus symptoms. Female sex hormones do not cause lupus, but they do influence its course. Among them may be high-dose preparations of female sex hormones for the treatment of gynecological diseases. But this does not apply to taking low-dose oral contraceptives (OCs). Smoking is considered a risk factor for lupus, which can cause the disease and worsen its course (especially vascular damage). Some medications can aggravate the course of lupus (in each case, you should read the instructions for the drug). Infections such as cytomegalovirus (CMV), parvovirus ( erythema infectiosum) and hepatitis C can also cause lupus. Epstein-Barr virus associated with the occurrence of lupus in children. Chemicals can cause lupus. Among these substances, trichlorethylene ( narcotic substance, used in the chemical industry). Hair dyes and fixatives, previously thought to be a cause of lupus, are now completely justified. The following groups of people are more likely to develop lupus: Women get lupus more often than men. People of African descent get lupus more often than whites. People between the ages of 15 and 45 get sick most often. Heavy smokers (according to some studies). People with a family history. People regularly taking medications associated with a risk of lupus (sulfonamides, some antibiotics, hydralazine).

Medicines that cause lupus

One of the common causes of lupus is the use of drugs and other chemicals. In the United States, one of the main drugs associated with drug-induced SLE is hydralazine (about 20% of cases), as well as procainamide (up to 20%), quinidine, minocycline, and isoniazid. Drugs most commonly associated with lupus include calcium channel blockers, ACE inhibitors, TNF-alpha antagonists, thiazide diuretics, and terbinafine (an antifungal drug). The following groups of drugs are commonly associated with drug-induced SLE: Antibiotics: minocycline and isoniazid. Antipsychotic drugs: chloropromazine. Biological agents: interleukins, interferons. Antihypertensive drugs: methyldopa, hydralazine, captopril. Hormonal drugs: leuprolide. Inhaled drugs for COPD: tiotropium bromide. Antiarrhythmic drugs: procainamide and quinidine. Anti-inflammatory: sulfasalazine and penicillamine. Antifungals: terbinafine, griseofulvin and voriconazole. Hypocholesterolemic: lovastatin, simvastatin, atorvastatin, gemfibrozil. Anticonvulsants: valproic acid, ethosuximide, carbamazepine, hydantoin. Other drugs: eye drops with timolol, TNF-alpha inhibitors, sulfonamide drugs, high-dose drugs of female sex hormones. Additional list of drugs that cause lupus: Amiodarone. Atenolol. Acebutolol. Bupropion. Hydroxychloroquine. Hydrochlorothiazide. Glyburide. Diltiazem. Doxycycline. Doxorubicin. Docetaxel. Gold and its salts. Imiquimod. Lamotrigine. Lansoprazole. Lithium and its salts. Mephenytoin. Nitrofurantoin. Olanzapine. Omeprazole. Practolol. Propylthiouracil. Reserpine. Rifampicin. Sertalin. Tetracycline. Ticlopidine. Trimethadione. Phenylbutazone. Phenytoin. Fluorouracil. Cefepime. Cimetidine. Esomeprazole. Sometimes systemic lupus erythematosus is caused by chemicals that enter the body from the environment. This only happens to some people, for a reason that is not yet clear. These chemicals include: Some insecticides. Some metal compounds. Eosin (fluorescent liquid in lipsticks). Para-aminobenzoic acid (PABA).

Symptoms of lupus vary widely because the disease can affect different organs. Whole volumes of medical manuals have been written about the symptoms of this complex disease. We can look at them briefly. No two cases of lupus are exactly alike. Symptoms of lupus can appear suddenly or develop gradually; they can be temporary or bother the patient for life. In most patients, lupus is relatively mild, with periodic exacerbations when the symptoms of the disease become worse and then subside or disappear altogether. Symptoms of lupus may include: Fatigue and weakness. Temperature increase. Pain, swelling and stiffness of the joints. Erythema on the face in the form of a butterfly. Skin lesions worsened by sun. Raynaud's phenomenon (decreased blood flow in the fingers). Breathing problems. Chest pain. Dry eyes. Memory loss. Impaired consciousness. Headache. It is almost impossible to suspect that you have lupus before visiting a doctor. Seek advice if you have an unusual rash, fever, joint pain, or fatigue.

Diagnosing lupus can be very difficult due to the variety of manifestations of the disease. Symptoms of lupus can change over time and resemble other diseases. To diagnose lupus, a whole range of tests may be required: 1. Complete blood count. This analysis determines the content of red blood cells, white blood cells, platelets, and hemoglobin. Anemia may be present in lupus. Low content white blood cells and platelets may also indicate lupus. 2. Determination of ESR indicator. The erythrocyte sedimentation rate is determined by how quickly the red blood cells from your blood settle in a prepared blood sample to the bottom of the tube. ESR is measured in millimeters per hour (mm/h). A rapid erythrocyte sedimentation rate may indicate inflammation, including autoimmune inflammation, as with lupus. But ESR also increases with cancer, other inflammatory diseases, even with a common cold. 3. Assessment of liver and kidney functions. Blood tests can show how well your kidneys and liver are working. This is determined by the level of liver enzymes in the blood and the level of toxic substances that the kidneys must cope with. Lupus can affect both the liver and kidneys. 4. Urine tests. Your urine sample may reveal increased content protein or red blood cells. This indicates kidney damage, which can occur with lupus. 5. Analysis for ANA. Antinuclear antibodies (ANAs) are special proteins that are produced by the immune system. A positive ANA test may indicate lupus, although it can also occur in other diseases. If your ANA test is positive, your doctor may order other tests. 6. Chest X-ray. Taking an image of the chest can help detect inflammation or fluid in the lungs. This may be a sign of lupus or other diseases that affect the lungs. 7. Echocardiography. Echocardiography (EchoCG) is a technique that uses sound waves to produce a real-time image of the beating heart. An echocardiogram can reveal problems with the heart valves and more. 8. Biopsy. A biopsy, the removal of a sample of an organ for testing, is widely used in the diagnosis of various diseases. Lupus often affects the kidneys, so your doctor may order a biopsy of your kidneys. This procedure is carried out using a long needle after preliminary anesthesia, so there is nothing to worry about. The resulting piece of tissue will help identify the cause of your illness.

Treatment for lupus is very complex and lengthy. Treatment depends on the severity of the disease's symptoms and requires a serious discussion with your doctor about the risks and benefits of any given therapy. Your doctor should monitor your treatment regularly. If the symptoms of the disease subside, he may change the drug or reduce the dose. If an exacerbation occurs, it’s the other way around. Modern drugs for the treatment of lupus: 1. Nonsteroidal anti-inflammatory drugs (NSAIDs). Over-the-counter NSAIDs such as naproxen (Anaprox, Nalgesin, Floginas) and ibuprofen (Nurofen, Ibuprom) can be used to treat inflammation, swelling, and pain caused by lupus. Stronger NSAIDs, such as diclofenac (Olfen), are available as prescribed by your doctor. Side effects of NSAIDs include abdominal pain, stomach bleeding, kidney problems and an increased risk of cardiovascular complications. The latter is especially true for celecoxib and rofecoxib, which are not recommended for older people. 2. Antimalarial drugs. Medicines commonly prescribed to treat malaria, such as hydroxychloroquine (Plaquenil), help control lupus symptoms. Side effects: stomach discomfort and retinal damage (very rare). 3. Corticosteroid hormones. Corticosteroid hormones are powerful drugs that fight inflammation in lupus. Among them are methylprednisolone, prednisolone, dexamethasone. These drugs are prescribed only by a doctor. They have long-term side effects: weight gain, osteoporosis, high blood pressure, risk of diabetes and susceptibility to infections. The higher the dose you use and the longer the course of treatment, the higher the risk of side effects. 4. Immunosuppressants. Drugs that suppress the immune system can be very helpful for lupus and other autoimmune diseases. Among them are cyclophosphamide (Cytoxan), azathioprine (Imuran), mycophenolate, leflunomide, methotrexate and others. Possible side effects: susceptibility to infections, liver damage, decreased fertility, risk of many types of cancer. A newer drug, belimumab (Benlysta), also reduces inflammation in lupus. Its side effects include fever, nausea and diarrhea. Tips for lupus patients. If you have lupus, there are several steps you can take to help yourself. Simple measures can make flare-ups less frequent and improve your quality of life. Try the following: 1. Adequate rest. People with lupus experience constant fatigue, which is different from that of healthy people and does not go away with rest. For this reason, you may have a hard time judging when to stop and rest. Develop a gentle daily routine for yourself and follow it. 2. Beware of the sun. Ultraviolet rays can trigger lupus flare-ups, so you should wear covered clothing and avoid walking in hot rays. Choose yours Sunglasses darker, and a cream with SPF of at least 55 (for particularly sensitive skin). 3. Eat a healthy diet. A healthy diet should include fruits, vegetables, and whole grains. Sometimes you will have to endure dietary restrictions, especially if you have high blood pressure, kidney problems or gastrointestinal problems. Take this seriously. 4. Exercise regularly. Physical exercise approved by your doctor will help you improve your fitness and recover faster from flare-ups. In the long term, fitness reduces the risk of heart attack, obesity and diabetes. 5. Stop smoking. Among other things, smoking can worsen the damage to the heart and blood vessels caused by lupus.

Alternative medicine and lupus

Sometimes alternative medicine can help people with lupus. But we should not forget that it is unconventional precisely because its effectiveness and safety have not been proven. Be sure to discuss any alternative treatments you want to try with your doctor. Unconventional methods of treating lupus known in the West: 1. Dehydroepiandrosterone (DHEA). Dietary supplements containing this hormone may help reduce the dose of steroids a patient receives. DHEA relieves symptoms of the disease in some patients. 2. Flax seed. Flaxseed contains a fatty acid called alpha-linolenic acid, which may reduce inflammation. Some studies have shown the ability of flax seeds to improve kidney function in lupus patients. Side effects include bloating and abdominal pain. 3. Fish oil. Dietary fish oil supplements contain omega-3 fatty acids, which may be beneficial for lupus. Preliminary studies have shown promising results. Side effects of fish oil include nausea, vomiting, belching and a fishy taste in the mouth. 4. Vitamin D: There is some evidence that this vitamin improves symptoms in people with lupus. True, scientific data on this issue is very limited.

Inflammation caused by lupus can affect different organs. This leads to numerous complications: 1. Kidneys. Kidney failure is one of the leading causes of death in people with lupus. Signs of kidney problems include itching all over the body, pain, nausea, vomiting, and swelling. 2. Brain. If the brain is affected by lupus, the patient may experience headaches, dizziness, behavioral changes, and hallucinations. Sometimes seizures and even strokes occur. Many people with lupus have problems with memory and expression. 3. Blood. Lupus can cause blood disorders such as anemia and thrombocytopenia. The latter is manifested by a tendency to bleeding. 4. Blood vessels. With lupus, the blood vessels of various organs can become inflamed. This is called vasculitis. The risk of vascular inflammation increases if the patient smokes. 5. Lungs. Lupus increases the likelihood of inflammation of the pleura, called pleurisy, which can make breathing painful and difficult. 6. Heart. Antibodies can attack the heart muscle (myocarditis), the sac around the heart (pericarditis), and large arteries. This leads to an increased risk of heart attack and other serious complications. 7. Infections. People with lupus become vulnerable to infection, especially as a result of treatment with steroids and immunosuppressants. Most common infections genitourinary system, respiratory infections. Common pathogens: yeast, salmonella, herpes virus. 8. Avascular necrosis of bones. This condition is also known as aseptic or non-infectious necrosis. Occurs when the blood supply to the bones decreases, leading to fragility and easy destruction of bone tissue. Problems often arise with the hip joint, which experiences heavy loads. 9. Complications of pregnancy. Women with lupus have high risk miscarriage. Lupus increases the likelihood of preeclampsia and premature birth. To reduce your risk, your doctor may advise you not to conceive until at least 6 months have passed since your last outbreak. 10. Cancer. Lupus is associated with an increased risk of many types of cancer. In fact, some lupus drugs (immunosuppressants) themselves increase this risk.

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lupus erythematosus

Lupus erythematodes (lupus erythematodes, lupus erythematosus) is a group of diseases that combine systemic, cutaneous and drug-induced lupus erythematosus. The listed diseases have a number of common features: they mainly affect women, erythematous skin rashes and enanthema on the mucous membranes, and increased sensitivity to solar and UV irradiation are observed. There are cases where individual patients with cutaneous lupus erythematosus develop signs of systemic lupus erythematosus over time.

However, the differences between these two diseases (clinical, including in the nature of skin rashes, immunological and immunogenetic) are still much greater than the similarities. With cutaneous lupus erythematosus, isolated or predominant skin lesions are observed; Drug-induced and systemic lupus erythematosus is characterized by multiple syndromes, and the latter also has a severe progressive course.

In the International Classification of Diseases, Injuries and Causes of Death, cutaneous lupus erythematosus is classified in class XII “Diseases of the skin and subcutaneous tissue,” and systemic and drug-induced lupus erythematosus is classified in class XIII “Diseases of the musculoskeletal system and connective tissue.” Some experts consider lupus erythematosus as a single disease that has two forms: cutaneous and systemic.

Cutaneous lupus erythematosus

Cutaneous lupus erythematosus is most often represented by discoid lupus erythematosus, centrifugal erythema of Biette and the so-called deep lupus erythematosus. Discoid lupus erythematosus is characterized by three cardinal clinical symptoms: erythema, hyperkeratosis and atrophy.

At the beginning of the disease, a small pink or red spot with clear boundaries appears, which is gradually covered in the center with small dense grayish-white dry scales. They hold tightly due to the presence on their lower surface of spike-like protrusions, immersed in the dilated follicular ostia (follicular hyperkeratosis). When removing the scales, pain appears (Besnier-Meshchersky symptom).

Gradually, cicatricial atrophy begins to appear in the center of the lesion, and the lesion takes on a form pathognomonic for discoid lupus erythematosus: in the center - a smooth, delicate white atrophic scar, further to the periphery - a zone of hyperkeratosis and infiltration, outside - a corolla of hyperemia, typical localization of the lesion is in open areas of the skin : face, especially on the nose and cheeks with the formation of a butterfly figure (the so-called lupus butterfly), ears, neck. The scalp and red border of the lips are often affected. The lesions may be located on the oral mucosa, where they can erode.

With centrifugal erythema Bietto (the so-called superficial form of cutaneous lupus erythematosus) of the three main skin symptoms, characteristic of discoid lupus erythematosus, only hyperemia is clearly expressed, while scales and cicatricial atrophy are almost or completely absent. The lesions are usually located on the face and often resemble a butterfly shape.

Multiple foci of discoid lupus erythematosus or centrifugal erythema of Biette scattered over various areas of the skin are defined as disseminated lupus erythematosus.

Among the rare forms of cutaneous lupus erythematosus, deep Kaposi-Irganga lupus erythematosus is distinguished, in which, along with the usual foci, there is one or more sharply demarcated dense mobile nodes covered with normal skin.

Cutaneous lupus erythematosus is characterized by a long-term continuous course with deterioration in the spring and summer due to photosensitivity.

Systemic lupus erythematosus

Systemic lupus erythematosus (lupus erythematosus systemicus) is a chronic progressive polysyndromic disease, which is characterized by genetically determined imperfection of immunoregulatory processes, the development of autoimmune disorders and immune complex chronic inflammation. Mostly women aged 20-30 years are affected (the ratio with the disease in men is 10:1), often teenagers.

The etiology and pathogenesis of systemic lupus erythematosus have not been fully elucidated. The viral genesis of the disease (in particular, the participation of retroviruses) in combination with a family genetic predisposition is assumed. The role of sex hormones (the onset of menstruation, abortion, childbirth), and the general connection of the disease with gender and age are also discussed. Systemic lupus erythematosus is a classic autoimmune disease in which the development of a hyperimmune response is observed against the unchanged components of one's own cells (nuclear and cytoplasmic), especially native DNA. Circulating antinuclear antibodies can form immune complexes, which, deposited in various organs, cause chronic inflammation and local or systemic tissue damage. Systemic connective tissue disorganization and generalized vascular damage are usually observed.

Systemic lupus erythematosus is characterized by the development of glomerulonephritis (lupus nephritis).

The disease most often begins with recurrent arthritis, malaise, fever, skin rashes, rapid weight loss, less often with high fever, acute arthritis and a pronounced characteristic skin syndrome. Subsequently, progressive pathology develops in various organs.

Arthritis is observed in 80-90% of patients. Non-erosive chronic polyarthritis of small joints of the hands, wrist and ankle joints, less often of larger joints, myalgia and myositis are common.

Skin lesions are varied and have important diagnostic value. Only in 10-15% of patients they may be absent (lupus sine lupo), however, such a condition is temporary, transient.

The most common skin lesions are isolated or confluent erythematous spots of various shapes and sizes, more or less edematous, sharply delimited from the surrounding area. healthy skin, which are usually observed on the face, neck, chest, elbow, knee and ankle joints. Typically, the appearance of erythema under the influence of solar and UV irradiation (photosensitization phenomenon). Skin changes in the midface are less common. Sometimes the butterfly has the appearance of persistent erysipelas with severe swelling of the face, especially the eyelids.

Patients with systemic lupus erythematosus often have trophic disorders (general dry skin, diffuse loss hair, deformation and brittleness of nails). The most common and varied skin changes are observed during the acute and subacute course of the disease.

Damage to the serous membranes is observed during the course of the disease in almost all patients in the form of dry or effusion pleurisy and pericarditis, less commonly peritonitis, perisplenitis and perihepatitis. Systemic lupus erythematosus is characterized by polyserositis.

The pathological process often spreads to the heart (lupus carditis), affecting all its membranes. Raynaud's syndrome occurs in 15-20% of patients and is an early sign of systemic lupus erythematosus, one of the manifestations of systemic vasculitis. Often this symptom is combined with Hashimoto's thyroiditis, cytopenias and Sjögren's syndrome.

The duration of acute lupus erythematosus without treatment is no more than 1-2 years.

In the subacute course, the disease begins with arthralgia, recurrent arthritis, and various skin lesions. With subsequent exacerbations, new organs and systems are involved in the pathological process and polysyndromic behavior develops within 2-3 years, lupus nephritis is often observed with a frequent outcome in chronic renal failure and encephalitis.

Diagnostics. Laboratory tests used for systemic lupus erythematosus can determine inflammatory and immunological activity. More than half of the patients have leukopenia, reaching in some cases 1.2 x 109/l, in combination with lymphopenia (5-10% of lymphocytes). Quite often, hypochromic anemia is detected, due to various reasons, including gastric bleeding due to the development of ulcers during treatment with corticosteroids, and renal failure. With the development of hemolytic anemia, a positive Coombs test and moderate thrombocytopenia are noted; thrombocytopenic purpura rarely develops.

Of great diagnostic importance is the detection in the blood of LE cells, which are mature neutrophils, in the cytoplasm of which there are large inclusions - phagocytosed remnants of the nuclei of decayed neutrophils. LE cells are found in 2/3 of patients in an amount of 5 or more per 1000 leukocytes. Single LE cells can also be observed in other diseases. It is important for diagnosis to detect antinuclear antibodies in high titers - antinuclear factor, antibodies to native DNA, etc. In classical cases, the diagnosis of systemic lupus erythematosus is based on the detection of a diagnostic triad (lupus butterfly, recurrent nonerosive polyarthritis, polyserositis), LE cells or antinuclear antibodies ( including antinuclear factor) in diagnostic titers. Circumstances such as age, the connection of the onset of the disease with childbirth, abortion, the onset of menstruation, and excessive insolation have auxiliary diagnostic significance. In cases of monosyndromic onset of systemic lupus erythematosus, a differential diagnosis with other diffuse connective tissue diseases or rheumatic diseases - such as rheumatism, rheumatoid arthritis, juvenile chronic arthritis, etc.

Drug-induced lupus erythematosus

Drug-induced lupus erythematosus develops in some cases with long-term use of procainamide, isoniazid and hydralazine. The clinical picture is characterized by arthritis, erythematous skin rashes, serositis, and lung damage.

Discontinuation of the drug gradually leads to the elimination of the clinical and immunological manifestations of the disease.

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Drug-induced lupus erythematosus

LUPUS ERYTHEMATOSUS ( lupus erythematodes, lupus erythematosus; syn.: erythema centrifugum, erythematosis) - a group concept that includes a number of nosological units, Ch. arr. systemic lupus erythematosus and discoid lupus erythematosus, as well as drug-induced lupus syndrome. Systemic and discoid K. v. have a number of common features. So, both systemic and discoid K. v. affects predominantly women; Both forms are characterized by erythematous rashes on the skin of the face, limbs, trunk and mucous membranes (enanthema), increased sensitivity to solar radiation (photosensitization); transition to discoid K. is possible. systemic (in 3-5% of patients); in some families there may be patients with discoid, systemic K. v. and other collagen diseases. At the same time, differences in the nature of erythematous rashes and especially systemic manifestations in systemic and discoid K. v., features of pathogenesis, in particular deep disturbances of immunogenesis in systemic K. v., allow most authors to consider them as separate nosole forms. This was reflected in the “Statistical Classification of Diseases and Causes of Death” (1969): discoid K. v. belongs to class XII “Diseases of the skin and subcutaneous tissue”, and systemic K. century - to class XIII “Diseases of the musculoskeletal system and connective tissue”.

Systemic lupus erythematosus

Systemic K.v. ( lupus erythematosus systemicus; syn.: acute lupus erythematosus, erythematous chroniosepsis, Liebmann-Sachs disease) - a chronic systemic inflammatory disease of connective tissue and blood vessels with pronounced autoimmune pathogenesis and, apparently, viral etiology; refers to diffuse connective tissue diseases - collagenosis (see Collagen diseases). Systemic K.v. is a disease of women of childbearing age (20-30 years), teenage girls often get sick. The ratio of women to men suffering from this disease, according to most statistics, is 8: 1 - 10: 1.

Story

Systemic K.v. described in 1872 by the Viennese dermatologist M. Kaposi as discoid K. v., characterized by fever, pleuropneumonia, rapid development of coma or stupor, and death. In 1923, Libman and Sacks (E. Libman and V. Sacks) described atypical verrucous endocarditis (Libman-Sacks endocarditis), polyserositis, pneumonia and erythematous rashes in the dorsum of the nose and zygomatic arches - the so-called. butterfly Modern doctrine of systemic K. v. associated with the names of Klemperer, Pollack and Baer (P. Klemperer, A.D. Pollack and G. Baehr), who in 1941 drew attention to diffuse collagen disease, describing systemic damage to connective tissue in this disease and scleroderma. With the discovery of LE cells (Lupus erythematosus cells) by Hargraves, Richmond and Morton (M. M. Hargraves, H. Richmond, R. Morton) in 1948, and in 1949 by Y. R. Ha-serick, the lupus factor was attention is paid to autoimmune disorders.

In the domestic literature, the first wedge, description of “acute lupus erythematosus” belongs to G. I. Meshchersky (1911), and pathomorphology - I. V. Davydovsky (1929) and others. Systematic study of systemic K. v. in our country, started by E. M. Tareev, O. M. Vinogradova and others. In 1965, E. M. Tareev et al., in the monograph “Collagenoses,” after analyzing 150 observations, described systemic K. v. in all its diversity, raised the question of the curability of the disease and outlined ways for further study. Unconditional progress in the development of the doctrine of systemic medicine. due to highly effective treatment with corticosteroids and immunosuppressants.

Statistics

Population studies by Siegel et al. (1962-1965) showed that the incidence in the Manhattan region (New York) increased from 25 to 1 million people. in 1955 to 83 per 1 million in 1964. Dubois (E. L. Dubois, 1974) suggests that in the USA systemic K. century. 5,200 people fall ill every year, therefore, every 5 years minimally ill people accumulate. Leonhardt (T. Leonhardt) in 1955 showed that the prevalence of systemic K. v. in Malmo (Sweden) from 1955 to 1960 there was 29 per 1 million. Mortality in the USA, according to Cobb (Cobb, 1970), is 5.8 per 1 million population, higher among women aged 25-44 of the year. Mortality, according to materials from the Institute of Rheumatism of the USSR Academy of Medical Sciences, decreased from 90% in 1959-1960. up to 10% by 1975

Etiology

The etiology is not clear, however, the hypothesis about the role of hron, a persistent viral infection, was developed in connection with the discovery by electron microscopy in the affected organs (skin, kidneys, synovium) of tubuloreticular structures located in the cytoplasm of endothelial cells, as well as in lymphocytes and platelets of peripheral blood , which resembled the nucleoprotein of paramyxoviruses. With systemic K. v. Circulating antibodies to measles, rubella, parainfluenza and other RNA viruses from the group of paramyxoviruses were also detected in high titers. Lymphocytotoxic antibodies, which are markers of persistent viral infection, were detected in patients and their relatives, and, in addition, in the same groups and among medical staff working with patients, antibodies to double-stranded (viral) RNA were detected. In connection with the viral etiology of systemic K. v. phenomena such as hybridization of the measles virus genome with the DNA of cells of affected organs (spleen, kidneys), detection of oncornavirus type C antigens in fractions of the spleen, placenta and kidney are discussed. Hypothesis about the significance of hron, viral infection in systemic K. century. is also based on the study of the disease in New Zealand mice, in which the role of oncornavirus type C has been proven.

Intolerance to drugs, vaccines, photosensitivity, the formation of the menstrual cycle, pregnancy, childbirth, abortion, etc. are considered as factors provoking the disease or its exacerbation; they are important for prevention and timely diagnosis, since the connection between the onset or exacerbations of the disease with these factors is more typical for systemic K. v. than for other related diseases.

Pathological anatomy

Systemic K. v., being a representative of the group of collagen diseases, is characterized by a generalized spread of patol, a process that covers all organs and systems, which determines the clinical and anatomical polymorphism of the disease. Generalization is caused by the circulation in the blood of immune complexes that damage the vessels of the microvasculature, resulting in systemic progressive disorganization of connective tissue. Immunopathol. reactions are confirmed by increased function of the organs of immunogenesis, precipitation in the walls of blood vessels and in the affected tissues of immune complexes with the appearance of immunocompetent cells (see). Damage to microcirculation vessels is manifested by common vasculitis of a destructive or proliferative nature (see Vasculitis). In the endothelium of the capillaries, electron microscopy reveals peculiar tubular formations (Fig. 1), similar to the ribonucleoprotein of the paramyxovirus and, possibly, playing an etiol role.

The specificity of tissue reactions during systemic K. v. cause signs of pathology of cell nuclei: fibrinoid basophilia, karyorrhexis, hematoxylin bodies, LE cells, central chromatolysis. Fibrinoid basophilia is caused by the admixture of acidic nuclear decay products. Hematoxylin bodies, described in 1932 by L. Gross, are swollen nuclei of dead cells with lysed chromatin. LE cells, or lupus erythematosus cells, are mature neutrophils, the cytoplasm of which is almost entirely filled with the phagocytosed nucleus of a dead leukocyte. At the same time, the own core is pushed to the periphery. They can be found in the sinuses of lymph nodes, in imprint smears from inflammatory exudate, for example, from pneumonic foci (Fig. 2). Central chromatolysis is manifested by the washing out of chromatin from the center of cell nuclei with clearing of the latter.

The most characteristic changes in systemic K. century. noted in the kidneys, heart, spleen. Kidney damage is characterized by the development of lupus glomerulonephritis, microscopically manifested in two forms: 1) with characteristic signs of systemic K. v.; 2) without characteristic signs of systemic K. v. (V.V. Serov et al., 1974). Characteristic signs include fibrinoid in the glomerular capillaries, the phenomenon of “wire loops”, hyaline thrombi, karyorrhexis (tsvetn. fig. 6). “Wire loops” are thickened, impregnated with plasma proteins and exposed due to desquamation of the endothelium, the basement membranes of glomerular capillaries, which are considered as a prestage of fibrinoid changes. They were described in 1935 by G. Baehr et al. Hyaline thrombi are located in the lumen of the glomerular capillaries and, based on their tinctorial properties, are regarded as intravascular fibrinoid. The second form is characterized by the development of membranous, membranous-proliferative or fibroplastic changes inherent in banal glomerulonephritis. Both forms are often found in combination.

The development of lupus glomerulonephritis is based on damage to the renal glomeruli by immune complexes. Immunofluorescence microscopy reveals luminescence of immunoglobulins (Fig. 3), complement, and fibrin in the glomeruli. Electron microscopy reveals equivalents of immune complexes in the form of deposits (Fig. 4). When the latter are localized on the subepithelial surface of the basement membrane, damage to the processes of podocytes and the formation of spiny outgrowths of the membrane are observed, which is referred to as membranous transformation. In the clinic, nephrotic syndrome is often noted. The proliferative reaction, according to V.V. Serov et al. (1974), is associated with the proliferation of mesangial cells. As a result of lupus nephritis, secondary shrinkage of the kidneys develops.

Heart damage is characterized by the development of Libman-Sachs endocarditis (Fig. 5). Endocarditis affects the leaflets and chords of the valves, the parietal endocardium, usually does not lead to heart disease, but the development of mitral valve insufficiency is possible. In the myocardium, fatty degeneration of muscle cells ("tiger" heart) is found, and less commonly, diffuse proliferative interstitial myocarditis - lupus carditis. The pericardium is most often affected.

The spleen is enlarged, microscopically a characteristic feature is found in it - “bulbous” sclerosis - a layered ring-shaped growth of collagen fibers in the form of a muff around sclerotic arteries and arterioles (Fig. 6). The follicles are atrophied, plasmatization and macrophage reaction are expressed in the red pulp. Plasmatization is also noted in enlarged lymph nodes, bone marrow, and thymus.

It is possible to develop lupus pneumonitis, which occurs as interstitial pneumonia with vasculitis and cellular infiltration of interstitial tissue. Lung damage may be associated with a secondary infection.

Lupus can affect the liver. In this case, lymphoplasmacytic infiltration and degeneration of hepatocytes are observed in the portal tracts.

Vasculitis is associated with damage to the nervous system.

Visceral lesions are often combined with lesions of the musculoskeletal system and skin. With high disease activity in the skeletal muscles, the picture of acute focal myositis is determined. In the joints, a picture of acute synovitis may develop with a predominance of exudative reactions and usually without subsequent deforming processes.

Microscopic examination of the skin of affected and externally unaffected areas reveals vasculitis, often proliferative, in 70-80% of patients (tsvetn. Fig. 7). Immunofluorescence study reveals the glow of immunoglobulins on the basement membrane in the area of ​​the dermal-epidermal junction (Fig. 7).

Complications and manifestations of the disease leading to the death of patients (renal failure, focal confluent pneumonia, sepsis, anemia, vasculitis leading to cerebral and heart infarctions) have clear morphological signs. For morphol. the picture is left imprinted by corticosteroid therapy, the consequence of which is inhibition of the reaction of immunogenesis organs, adrenal atrophy, osteoporosis, areactive ulcers of the gastrointestinal tract. tract, signs of Itsenko-Cushing syndrome, sometimes an outbreak of tuberculosis, sepsis. Active treatment caused a medicinal pathomorphosis of the disease, characterized by a predominance of chronic forms of the disease over acute ones, an increase in the proportion of proliferative processes, sclerotic changes, a decrease in the frequency of karyorrhexis, hematoxylin bodies, and Libman-Sachs endocarditis.

Morphol, diagnosis of systemic K. v. is based on taking into account nuclear pathology, lupus glomerulonephritis, “bulbous” sclerosis in the spleen, positive immunofluorescence results, vasculitis, connective tissue disorganization, Liebman-Sachs endocarditis. For intravital morphology, diagnosis, biopsy material of the kidneys, skin, and skeletal muscles is examined with the mandatory use of immunofluorescent methods.

Pathogenesis

With systemic K. v. the role of disorders of the humoral immunity with the development of organ-nonspecific autoimmune reactions is obvious, which is manifested by hyperfunction of B-lymphocytes and a wide range of circulating autoantibodies (see) - to whole cell nuclei and individual components of the nucleus (DNA, nucleoprotein), as well as lysosomes, mitochondria, cardiolipids ( false-positive Wasserman reaction), blood coagulation factors, leukocytes, platelets, erythrocytes, aggregated gamma globulin (see Rheumatoid factor), etc. These antibodies, being antibodies that witness the damage that has occurred, are capable of forming circulating immune complexes that are deposited on the basal membranes of the kidneys , skin, etc., cause their damage with the development of an inflammatory reaction. This is the immune-complex mechanism for the development of lupus nephritis, vasculitis, etc. The presence of a DNA complex - an antibody to this DNA and complement is proven by the isolation of antibodies to DNA from kidney tissue, and the immune complexes themselves are detected by immunofluorescence (see). High activity of systemic K. v. characterized by hypocomplementemia - a decrease in the content of whole complement (CH50) and its components, especially C3, which takes part in the antigen-antibody reaction, C4, CD1, C9, etc. (see Complement). Many facts have accumulated indicating that there is an imbalance in the humoral and cellular components of immunity; the latter is manifested by various delayed-type hypersensitivity reactions and a decrease in the content of T-lymphocytes. The presence in certain families of systemic and discoid K. v., various autoimmune diseases, photosensitivity and drug intolerance, the detection of a wide range of circulating autoantibodies in members of these families allows us to think about the role of genetic predisposition in the development of the disease, but the specific mechanisms of this predisposition are not yet known.

Experimental models of systemic K. v. - a disease of New Zealand mice (NZB, NZW and their hybrids NZB/NZW F1) and dogs of special genetic lines (canine lupus) - confirm the above statements, since these models are certainly characterized by a genetic predisposition, an imbalance in humoral and cellular immunity and vertical transmission of oncornavirus C in New Zealand mice.

Clinical picture

The complaints of patients are varied, but most often they complain of pain in the joints, fever, loss of appetite, and sleep. As a rule, systemic K. v. begins subacutely with recurrent polyarthritis, reminiscent of rheumatic, fever, various skin rashes, malaise, weakness, weight loss. Less commonly observed is an acute onset with high fever, sharp pain and swelling of the joints, the “butterfly” symptom, polyserositis, nephritis, etc. In 1/3 of patients 5-10 years or more, one of the monosyndromes is observed - recurrent arthritis, polyserositis, Raynaud's syndrome , Verlhof, epileptiform, but later the disease acquires a relapsing course with the development of a characteristic polysyndromic pattern.

Lupus arthritis occurs in almost all patients; it is manifested by migrating arthralgia (see), arthritis (see), transient painful flexion contractures. Mostly small joints of the hands, wrists, ankles, and less often large joints are affected. In 10-15% of patients, fusiform deformation of the fingers and muscle atrophy on the back of the hands may develop. Articular syndrome is usually accompanied by myalgia, myositis, ossalgia and tendovaginitis. When rentgenol, the study reveals epiphyseal osteoporosis, mainly in the joints of the hands and wrists.

Skin damage. The most typical syndrome is “butterfly” - erythematous rashes on the face in the area of ​​the dorsum of the nose (“butterfly body”) and zygomatic arches (“butterfly wings”). According to O. L. Ivanov, V. A. Nasonova (1970), the following variants of erythema are observed: 1) vascular (vasculitic) “butterfly” - unstable, pulsating, diffuse redness with a cyanotic tint in the middle zone of the face, intensifying when exposed to external factors (insolation, wind, cold, etc.) or excitement; 2) “butterfly” type of centrifugal erythema - persistent erythematous-edematous spots, sometimes with mild follicular hyperkeratosis (erythema centrifugum Biett; color Fig. 1); 3) “butterfly” in the form of bright pink spots with sharp dense swelling against the background of general swelling and redness of the face (erysipelas faciei perstans Kaposi; color Fig. 2); 4) “butterfly”, consisting of discoid-type elements with clear cicatricial atrophy. Erythematous changes are also localized on the earlobes, neck, forehead, scalp, red border of the lips, torso (usually in the upper chest in the form of a décolleté), limbs, and above the affected joints. Some patients experience polymorphic erythema, urticaria, purpura, nodules and other elements.

A peculiar analogue of the “butterfly” of the first and second types are vasculitis (capillaritis) - small erythematous spots with slight swelling, telangiectasia and mild atrophy on the terminal phalanges of the fingers and toes, less often on the palms and soles (color Fig. 3). Various trophic disorders - hair loss, deformation and brittleness of nails, ulcerative skin defects, bedsores, etc. create the characteristic appearance of a patient with systemic K. v.

Damage to the mucous membranes is manifested by enanthema on the hard palate, aphthous stomatitis, thrush, hemorrhages, and lupus cheilitis.

Polyserositis - migratory bilateral pleurisy and pericarditis, less commonly peritonitis - is considered an integral component of the diagnostic triad, along with dermatitis and arthritis. The effusion is usually small and its composition resembles rheumatic effusion, but contains LE cells and antinuclear factors. Recurring, polyserositis (see) leads to the development of adhesions up to obliteration of the pericardial cavity, pleura, perisplenitis and perihepatitis. Wedge, manifestations of serositis are usual (pain, friction noise of the pericardium, pleura, peritoneum, etc.), but due to the small amount of exudates and the tendency to quickly disappear, clinicians easily view them, however, with X-ray examination, pleuropericardial adhesions or thickening of the costal bone are often revealed , interlobar, mediastinal pleura.

Lupus carditis is very characteristic of systemic K. century; it is characterized by the simultaneous or sequential development of pericarditis (see), myocarditis (see) or atypical Libman-Sachs warty endocarditis on the mitral and other heart valves, as well as the parietal endocardium and large vessels. Endocarditis ends with marginal sclerosis of the valve, less often with mitral valve insufficiency with characteristic auscultatory symptoms.

Vascular damage during systemic K. v. characteristic of pathol. processes in organs. Nevertheless, it should be noted the possibility of developing Raynaud's syndrome (long before the typical picture of the disease), damage to both small and large arterial and venous trunks (endarteritis, phlebitis).

Lupus pneumonitis is a vascular-connective tissue process in the lungs, in an acute course it occurs as a vasculitis (“vascular pneumonia”), and in other variants of the course - in the form of basal pneumonitis (see) with a normal wedge, a picture of a parenchymal process, but characteristic roentgenol, symptoms (mesh structure of an enhanced pulmonary pattern, high position of the diaphragm and basal discoid atelectasis) give the syndrome great diagnostic significance.

Lupus glomerulonephritis (lupus nephritis) is a classic immune complex glomerulonephritis (see), observed in half of the patients during the generalization of the process according to the type of urinary syndrome, nephritic and nephrotic. Kidney biopsy followed by gistol and immunomorphol is of great diagnostic importance. research.

Damage to the neuropsychic sphere (neurolupus) - manifests itself at the onset of the disease asthenovegetative syndrome, and at the height of the disease one can observe a variety of symptoms and syndromes from the central and peripheral nervous system, usually combined - meningoencephalitis, encephalopolyneuritis, encephalomyelitis or meningoencephalomyelitis with polyradiculoneuritis (the latter has a diagnostic meaning).

In the acute form of the disease, affective disorders, delirious-oneiric and delirious types of stupefaction, and patterns of stupor varying in depth can be observed.

Affective disorders are manifested by states of anxious depression, as well as manic-euphoric syndromes. Anxious depression is accompanied by pictures of verbal hallucinosis of condemning content, fragmentary ideas of attitude and nihilistic delusions (the latter is characterized by instability and lack of a tendency to systematize). In manic-euphoric states, there is an elevated mood with a feeling of carelessness, self-satisfaction, and a complete lack of awareness of the disease. At times, some psychomotor agitation is observed, and persistent insomnia is characteristic; during short periods of sleep - vivid dreams, the content of which is often mixed in the patient’s mind with real events.

Delirious-oneiric states are excessively variable; either dream disorders with fantastic or ordinary themes, or abundant colorful, scene-like visual hallucinations come to the fore. Patients feel like observers of ongoing events or victims of violence. Excitement in these cases is of a confused and fussy nature, limited to the boundaries of the bed, and is often replaced by a state of immobility with muscle tension and a loud, monotonously prolonged cry.

Delirious states begin with the appearance of vivid nightmares during the period of falling asleep, followed by multiple, colorful, threatening visual hallucinations, accompanied by verbal hallucinations and a constant feeling of fear.

The intensity of mental disorders correlates with the severity of somatic manifestations, with a high degree of activity of the lupus process.

The described correlations of somatopsychic disorders make it possible to attribute psychoses to systemic K. v. to the group of exogenous organic brain lesions.

It must be borne in mind that with systemic K. v. disturbances in the emotional sphere can also develop in connection with hormonal therapy (steroid psychoses).

Damage to the reticuloendothelial system is expressed in polyadenia (enlargement of all groups of lymph nodes) - a very common and, apparently, early sign of generalization of the lupus process, as well as enlargement of the liver and spleen.

Flow

There are acute, subacute and chronic course of the disease. With an acute onset, patients can indicate the day of development of fever, acute polyarthritis, serositis, “butterfly”, and in the next 3-6 months. One can note pronounced polysyndromic behavior and lupus nephritis or meningoencephalomyelitis with polyradiculoneuritis. Untreated acute systemic K. v. previously led to death 1 - 2 years from the onset of the disease.

With a subacute onset, general asthenic syndromes or recurrent arthralgia, arthritis, and nonspecific skin lesions gradually develop. With each exacerbation in patol, the process involves more and more new organs and systems. A polysyndromic pattern develops, similar to that observed in the acute course of the disease, with a significant incidence of diffuse lupus nephritis and neurolupus.

When chronic, the course of the disease manifests itself for a long time as individual relapses of certain syndromes, and in the 5-10th year of the disease other organ manifestations (pneumonitis, nephritis, etc.) may develop with the development of characteristic polysyndromicity.

Variants of the onset and course of systemic K. century. have age-related patterns. The acute course is usually observed in children and adolescents, menopausal women and the elderly, subacute - mainly in women of childbearing age.

Complications

Among the complications of systemic K. century. the most common is a secondary infection (coccal, tuberculous, fungal, viral), associated with a violation of natural immunity, or with illness, or with inadequate treatment with corticosteroids, the use of immunosuppressants. With the progressive course of systemic K. v. and long-term treatment with corticosteroid drugs, especially in young people, miliary tuberculosis develops, therefore attention to tuberculosis infection with systemic K. v. must be constant for timely recognition and appropriate correction. Shingles (herpes zoster) develops in 10-15% of patients treated for a long time with large doses of corticosteroids and cytotoxic drugs.

Diagnosis

The diagnosis does not present great difficulties in patients with a typical “butterfly” of any type. However, this sign occurs in less than half of patients, and as an early sign - only in 15-20% of patients. Therefore, other symptoms, such as arthritis, nephritis, and their combinations, become of great diagnostic importance. The possibility of intravital biopsy of the joint and kidney makes it possible to more often recognize the lupus nature of arthritis or nephritis. Polysyndromy, detection of LE cells, high titer of antinuclear factors (ANF) or antibodies to native DNA (nDNA) are of diagnostic value. LE cells are found in 70% of patients with systemic K. v. and more. Single LE cells can also be observed in other diseases.

ANF ​​is an IgG directed against the patient's cell nuclei. Usually, to determine ANF, the Immunofluorescence method is used (Fig. 8), in which sections of rat liver, rich in nuclei, are taken as antigenic material, on which the patient’s serum and fluorescein-labeled antiglobulins are layered. For systemic K. century. the most characteristic is peripheral, edge luminescence (Fig. 8.2), due to the presence of antibodies to DNA, and a high titer of this reaction.

Antibodies to DNA are determined by various methods in RIGA (see Hemagglutination), in which sheep red blood cells are loaded with DNA, in the reaction of flocculation of bentonite particles (see Flocculation), also loaded with DNA; In addition, they use the method of radioimmune binding of iodine-labeled nDNA and immunofluorescence, where the culture of Crithidia luciliae is taken as an nDNA substrate.

With hron, polyarthritis and severe liver damage, positive reactions to rheumatoid factor can be detected in the Wohler-Rose reaction (see Rheumatoid arthritis) or latex agglutination (see Agglutination). It is also useful to study complement CH50 and its components, the decrease of which usually correlates with the activity of lupus nephritis. In almost all patients, ROE is significantly accelerated - up to 60-70 mm per hour. More than half of the patients have leukopenia (below 4000 in 1 μl) with a shift in the blood count to promyelocytes, myelocytes and young ones in combination with lymphopenia (5-10% of lymphocytes). Moderate hypochromic anemia is very often observed. In rare cases, hemolytic anemia develops with features of acquired hemolysis (see) and a positive Coombs reaction (see Coombs reaction). Thrombocytopenia (below 1 μl) is often observed, in rare cases - Werlhoff syndrome.

Thus, when establishing a diagnosis of systemic K. v. the whole wedge, the picture, the lab data should be taken into account. research methods and biopsy material of kidneys, synovium and skin.

For a more complete assessment of the patient’s condition, it is advisable to determine the degree of activity of the patol process. Klin, and lab. characterization of the degrees of activity of systemic To. v. is given in Table 1.

Treatment

Treatment started at the onset of the disease has the best effect. In the acute period, treatment is carried out in a hospital, where patients should be provided with adequate nutrition with sufficient amounts of vitamins B and C.

For individualization of treatment, a differentiated determination of the degrees of activity of the patol process is crucial (Table 1).

In case of patol, a process of III degree of activity, all patients, regardless of the course, are indicated for treatment with glucocorticosteroids in large doses (per day 40-60 mg of prednisolone or another drug in equivalent doses), in case of II degree - correspondingly smaller doses (30-40 mg per day). day), and for stage I - 15-20 mg per day. It is extremely important that the initial dose of glucocorticosteroids is sufficient to reliably suppress the activity of the patol process. Particularly large doses (50-60-80 mg per day of prednisolone) should be prescribed for nephrotic syndrome, meningoencephalitis and other diffuse processes in the nervous system - the so-called. lupus crisis. Treatment with glucocorticosteroids in the maximum dose is carried out until a pronounced effect occurs (according to a decrease in clinical and laboratory indicators of activity), and in case of nephrotic syndrome - for at least 2-3 months, then the dose of the hormone is slowly reduced, focusing on the proposed scheme (Table 2), but respecting the principle of individualization in order to prevent withdrawal syndrome or dose reduction syndrome.

Glucocorticosteroids should be prescribed in combination with potassium preparations, vitamins, anabolic hormones and symptomatic agents (diuretics, antihypertensives, ATP, cocarboxylase, etc.). When reducing their dose, salicylates, aminoquinolines and other drugs should be added. Treatment with hormones, as a rule, cannot be completely stopped due to rapidly developing deterioration of the condition (withdrawal syndrome), so it is important that the maintenance dose is minimal. The maintenance dose is usually 5-10 mg of the drug, but may be higher in case of unstable remission.

Such side symptoms that occur during treatment, such as Cushingoid, hirsutism, ecchymosis, stretch marks, acne, develop in many patients and do not require additional therapy. On the contrary, it is noted that persistent improvement in the condition usually occurs with the development of signs of hormone overdose. For persistent edema, diuretics, plasma and albumin transfusions may be recommended. Hypertension is relatively easily controlled with antihypertensive drugs.

Much more serious are complications such as steroid ulcers, exacerbations of focal infections, disorders of mineral metabolism with osteoporosis, etc., but they can also be prevented with systematic monitoring. An undoubted contraindication to continued treatment is steroid psychosis or increased seizures (epilepsy). Correction with psychotropic drugs is necessary.

If glucocorticosteroids are ineffective in patients with systemic K. v. treatment is prescribed with cytostatic immunosuppressants of the alkylating series (cyclophosphamide) or metabolites (azathioprine). Indications for the use of these drugs for systemic K. v. are: high (III) degree of disease activity involving many organs and systems, especially in adolescents; developed lupus nephritis (nephrotic and nephritic syndromes); the need to reduce the suppressive dose of glucocorticosteroid due to the developed side effects of this therapy.

Azathioprine (imuran) and cyclophosphamide are prescribed in doses of 1-3 mg per 1 kg of patient weight per day in combination with 10-40 mg of prednisolone per day to control extrarenal symptoms. Treatment with immunosuppressants should also be long-term, subject to regular medical supervision. Serious complications may develop during treatment with immunosuppressants, so monitoring of blood (including platelets) and urine is necessary, especially in the first 3 weeks. treatment. With inf. complications are treated with active antibiotic therapy. Other complications, including total alopecia, resolve when the dose of the immunosuppressant is reduced and symptomatic therapy is prescribed.

When hron, the course of systemic K. century. with predominant skin lesions of the discoid type. Recommend chloroquine, delagil or other quinoline drugs.

When signs of damage to internal organs subside and clinical and laboratory signs of activity decrease to degree I, treatment can be used. physical education and massage under the control of the general condition and condition of the internal organs. Physiotherapeutic and spa treatment for systemic K. v. not recommended due to the possibility of provoking the disease by ultraviolet irradiation, balneotherapy, and insolation.

Forecast

Prognosis for life with early recognition of systemic K. v. and adequate patol activity, the process of long-term treatment is satisfactory; 70-75% of patients return to active work at work and in the family. However, with the development of lupus nephritis, cerebrovasculitis and the addition of a secondary infection, the prognosis worsens.

Prevention

Prevention is aimed at preventing exacerbations and progression of the disease and the occurrence of the disease.

Prevention of disease progression (secondary) is carried out by timely, adequate, rational complex therapy, therefore patients should undergo regular medical examinations, take hormonal medications in a strictly prescribed dose, do not sunbathe or overcool, avoid surgical interventions, vaccinations, vaccines and serums (except for vital ones). In case of exacerbation of focal or intercurrent infection, bed rest, antibiotics, and desensitizing therapy are required. Treatment of focal infection should be persistent, predominantly conservative.

Primary prevention measures are especially important for family members of patients with systemic K. v., who have signs of photosensitivity, drug intolerance, and impaired humoral immunity. To prevent the disease or generalization of the process, these people should avoid ultraviolet irradiation, treatment with radioisotope gold, spa treatment, etc.

Features of the course of systemic lupus erythematosus in children

Predominantly girls of prepubertal and pubertal age are affected. The rise in incidence begins from the 9th year of life, its peak occurs at 12-14 years. Sometimes systemic K. v. occurs in children 5-7 years old; Cases of illness in children in the first months of life are described as casuistic. There are no cases of congenital disease.

In the vast majority of cases in children and adolescents, systemic K. v. begins and proceeds more acutely and severely, giving a higher mortality rate than in adults. This is due to the peculiarities of the reactivity of the growing organism, the uniqueness of connective tissue structures, organs of immunogenesis, the complement system, etc. Generalization of pathol, the process in children develops much faster, and damage to various organs is characterized by a predominance of the exudative component of inflammation in combination with signs of an intensively developing syndrome of intravascular coagulation disorder in in the form of hemorrhages and bleeding, collaptoid, soporous and shock conditions, thrombocytopenia.

At the onset of the disease, children most often complain of joint pain, weakness, and malaise. Along with this, fever is noted, dystrophy increases quite quickly, often leading to cachexia, significant changes in the blood appear, and signs of damage to many vital organs and systems are revealed.

Skin changes in manifestations typical of lupus are not always found. A combination of acute exudative and discoid changes is characteristic, as well as a tendency to merge individual lesions with the total spread of dermatitis, involving the entire skin and scalp. Hair falls out rapidly, which leads to alopecia areata or complete baldness, and breaks off, forming a kind of brush above the forehead line. The mucous membranes of the mouth, upper respiratory tract, and genitals may be affected. Nonspecific allergic manifestations in the form of urticaria and morbilliform rash or mesh-vascular pattern of the skin, as well as petechial-hemorrhagic elements are much more common and can be found in almost every patient in the active period of systemic K. v.

Articular syndrome, which is the most common and almost always one of the first signs of the disease, can be represented by arthralgia of a volatile nature, acute or subacute arthritis and periarthritis with mild ephemeral exudative manifestations. Articular syndrome is usually combined with damage to the tendon-muscular system, although myalgia and myositis are sometimes an independent sign of systemic K. v.

Involvement in patol, the process of serous membranes is observed in almost all cases; In the clinic, pleurisy and pericarditis are most often recognized, usually in combination with perihepatitis, perisplenitis, and peritonitis. Massive effusion in the pleura and pericardium, requiring repeated punctures, are characteristic manifestations of systemic K. v.

One of the most common visceral signs of systemic K. v. is carditis; its combination with arthritis in the early stages of the disease is almost always mistakenly interpreted as rheumatism. All three membranes of the heart can be affected, but in children and adolescents, symptoms of myocarditis predominate.

Lung lesions are diagnosed less frequently in the clinic than pleural lesions. Typical lupus pneumonitis is accompanied by an alveolar-capillary block, and percussion-auscultatory data are scanty, however, increasing hypoxia, phenomena of respiratory failure attract attention, confirm the presence of pneumonitis and rentgenol data.

Lupus nephritis occurs in children and adolescents more often than in adults (about 2/3 of cases) and in the vast majority of patients it is severe kidney damage with nephrotic syndrome, hematuria, a tendency to arterial hypertension, and is often accompanied by eclampsia. According to the nature of the course, lupus nephritis in children is close to the mixed form of hron, banal glomerulonephritis, often it is a variant of rapidly progressing glomerulonephritis and only in some patients it occurs in the form of minimal urinary syndrome.

Damage to the central and peripheral nervous system, generally similar to that in adults, includes a chorea-like syndrome with all the wedge, features inherent in minor chorea (see).

Quite often there are signs of damage to the colon. tract. Abdominal pain can be caused by intestinal damage, the development of peritonitis, perisplenitis, perihepatitis, as well as hepatitis and pancreatitis. Before establishing the diagnosis of systemic K. v. abdominal crises can be mistaken for banal acute appendicitis, cholecystitis, ulcerative colitis, dysentery, etc. Sometimes a picture develops acute abdomen(cm.). A symptom complex of malignant ongoing Crohn's disease is possible. The active period of the disease is accompanied by an increase in peripheral lymph nodes, sometimes so significant that for the purpose of differential diagnosis their puncture or biopsy is required.

In 2/3 of sick children and adolescents, systemic K. v. develops acutely or subacutely; There may also be cases of the most acute course of the disease, which is characterized by rapid development of hyperergic reactions, high fever of the wrong type and other signs (damage to the skin, joints, lymph nodes), hemorrhagic diathesis, damage to the nervous system. Rapidly progressing vasculitis in a short time leads to severe inflammatory-destructive and dystrophic changes in internal organs (heart, kidneys, lungs), with disruption of their functions and possible death in the first 3-9 months. from the onset of the disease. Death in such cases most often occurs due to symptoms of cardiopulmonary and (or) renal failure due to intoxication, profound disturbances of homeostasis, coagulopathic disorders, water and electrolyte imbalance, as well as the addition of a secondary infection.

With subacute systemic K. v., moderate in severity and duration, generalization of the process occurs in the first 3-6 months. from the onset of the disease, the course is persistent or wavy with constantly remaining signs of activity and relatively quickly joining function. inferiority of one or another organ.

In approximately 1/3 of children, a variant of the primary chronic course of the disease is observed, close to the picture of classical systemic K. v. adults, with a pre-systemic period lasting from one to 3 years, and with subsequent generalization of the process. Pre-systemic lupus manifestations in children most often include hemopathy, hemorrhagic and nephritic syndromes, arthropathy, and chorea. Other rarer monosyndromes are also possible.

Complications and diagnostic methods are the same as in adults.

Each child with pronounced clinical and laboratory signs of systemic To. v. activity. should be treated in a hospital setting. Corticosteroids and cytostatics are used to suppress immune hyperactivity. The size of their daily dose is determined not only by the age of the child, but also by the degree of activity of the patol process. For grade III activity with symptoms of nephritis, carditis, serositis, neurolupus, large doses of corticosteroids are prescribed (prednisolone at the rate of 1.25-2 mg or more per 1 kg of patient weight per day). If the indicated dose of prednisolone or an equivalent amount of a similar drug cannot be given to the patient, azathioprine or cyclophosphamide must be introduced into therapy at a rate of at least 1 - 3 mg per 1 kg per day. In case of nephrotic syndrome, autoimmune hemolytic anemia, hemorrhagic syndrome and crisis conditions, in all cases, combination immunosuppressive therapy in combination with heparin (250-600 units per 1 kg of body weight per day) is carried out from the very beginning. Upon achieving a clear clinical and laboratory improvement in the patient’s condition, the maximum immunosuppressive dose of prednisolone should be reduced (Table 2), heparin should be replaced with antiplatelet agents (chimes) and (or) indirect anticoagulants.

With a moderate degree of systemic activity. The immunosuppressive dose of corticosteroids should be lower (prednisolone - 0.5-1.2 mg per 1 kg of body weight per day), instead of heparin, chimes are prescribed at 6-8 mg per 1 kg of body weight per day, salicylates, quinoline drugs, and methindole are used more widely . With hron, current and low degree of activity of systemic K. v. in the absence of clear symptoms of damage to the kidneys, blood, nervous system, heart, lungs, corticosteroids are prescribed in small doses (prednisolone - less than 0.5 mg per 1 kg of body weight per day) or not used at all.

After discharge from the hospital, children are under the supervision of a rheumatologist and continue to receive supportive immunosuppressive and symptomatic therapy. During the first year after the acute period of systemic K. century. It is not recommended to attend school, but home education can be arranged. It is necessary to cancel all scheduled preventive vaccinations.

With adequate treatment of patients, it is increasingly possible to achieve relative or complete remission. At the same time, the general physical Children's development is progressing more or less satisfactorily, secondary sexual characteristics appear in a timely manner, and girls begin menstruation on time. Mortality is most often associated with renal failure.

Discoid lupus erythematosus

Discoid K. v. (syn.: lupus erythematodes discoides s. chronicus, erythematodes, seborrhea congestiva, erythema atrophicans etc.) is the most common chronic form of K. v., in which the dominant pattern in the disease picture is damage to the skin and mucous membranes. The name “lupus erythematodes” was proposed by P. Cazenave in 1851, believing that the disease was a type of tuberculous lupus. It was first described by R. F. Rayer in 1827 as a rare form of sebaceous discharge (fluxus sebaceus). Discoid K. v. accounts for 0.25-1% of all dermatoses (M.A. Agronik et al.), more often found in countries with cold, humid climates, mainly in middle-aged people [Gertler (W. Gertler)]. Women get sick more often than men.

Etiology

The etiology has not been definitively established. The origin of the disease is assumed to be viral. Electron microscopy reveals tubuloreticular cytoplasmic inclusions in skin lesions.

Pathogenesis

In the pathogenesis of individual cases of the disease, genetic and immunol factors are important. In provoking discoid K. v. and its exacerbations, an important role is played by excessive insolation, medications, various types of injuries (mechanical, thermal, chemical).

Pathological anatomy

Discoid K. v. and its disseminated form is limited to skin changes. With discoid K. v. the lesion is most often localized on the face. Microscopically (Fig. 9) one finds hyperkeratosis (see), follicular keratosis, vacuolar degeneration of the epidermis (see Vacuolar degeneration), acanthosis (see). Focal lymphoid-macrophage infiltrates with an admixture of neutrophils and plasma cells are visible in the dermis. The walls of blood vessels are impregnated with plasma proteins. The collagen fibers of the dermis are swollen, picrinophilic, and merge into fibrinoid masses. In the infiltration zone, elastic and collagen fibers are destroyed. During treatment, scarring occurs with atrophy and depigmentation of the skin.

For disseminated cutaneous form of K. v. characterized by multiple rashes throughout the body, in which microscopic changes resemble those of discoid K. v., but are less pronounced, exudative reactions predominate over proliferative ones, cellular infiltration is less significant. The result is no scars or areas of skin atrophy.

Clinical picture

Discoid K. v. begins with the appearance of one or two pink, slightly swollen spots, which gradually increase in size, infiltrate, and become covered in the central zone with tightly packed whitish scales. Scraping the lesions causes pain (Besnier-Meshchersky symptom), because on the underside of the scale there is a horny spine (ladies heel symptom), which is fixed in the expanded mouth of the hair follicle. Subsequently, cicatricial atrophy develops in the central part of the lesion. In a long-existing lesion, three zones are clearly distinguished: a central atrophic zone, then a hyperkeratotic zone and an erythematous zone bordering it (tsvetn. Fig. 4). Within the latter there are often telangiectasia (see). Brown hyperpigmentation may be expressed to varying degrees along the periphery of the lesion. Erythema (see), hyperkeratosis and skin atrophy (see) are the cardinal symptoms of K. v. Infiltration, telangiectasia and pigmentation are common but not obligatory signs.

The most typical localization of discoid K. v. is in areas of the skin exposed to insolation: face, ch. arr. its middle part is the noe, cheeks, zygomatic, preauricular areas. As well as for systemic K. v., the so-called butterfly (color fig. 5) - the lesion is on the back of the nose and cheeks. According to I.I. Lelis, who observed 518 patients, the primary foci of K. v. were located on the nose in 48%, on the cheeks in 33%, on the ears or adjacent skin - in 22.5%, on the forehead - in 16.5%, on the scalp - in 10%, on the red border lips, usually lower - in 12.5%, on the mucous membrane of the mouth - in 7%. Damage to the mucous membrane of the eyelids L. I. Mashkilleyson et al. observed in 3.4% of patients. More rare, including isolated localizations are known - on the chest, back, shoulders, etc. Lesions of the mucous membrane of the genitals, bladder, cornea, and lesions of the nails are described. Along with typical discoid K. v. There are its varieties: hyperkeratotic K. v., with a cut hyperkeratosis is pronounced; papillomatous discoid K. v. - increased proliferation of dermal papillae, leading to the formation of a villous surface of the lesions; warty K. v. - papillomatosis is accompanied by severe keratinization; pigmented K. v. - excessive deposition of pigment, coloring the lesions dark brown; seborrheic K. v. - hair follicles are greatly expanded and filled with fatty, loose scales; tumor-like K. v. - bluish-red, highly elevated foci with edematous, clearly defined edges, mild hyperkeratosis and atrophy.

Rare varieties are telangiectatic discoid K. v. with multiple telangiectasias, hemorrhagic discoid K. v. with hemorrhages in the foci, mutilating. A special form of hron. K.v. is centrifugal erythema (erythema centrifugum Biett). It accounts for 5.2-11% in relation to all forms of K. v., characterized by clearly demarcated foci of erythema on the face, less often on other areas of the skin. They may have telangiectasia and slight swelling. There is no hyperkeratosis. Atrophy is absent or mild. Centrifugal erythema responds fairly quickly to treatment, but recurs easily. Some authors classify it, along with disseminated K. v., as forms intermediate between discoid and systemic.

In the foci of discoid K. v. On the oral mucosa, dark red erythema, telangiectasias, stripe-like, coarse network-like areas of epithelial opacification, erosion, and superficial ulcerations are observed. On the red border of the lips K. v. has the appearance of irregularly oval ribbon-like foci of erythema and hyperkeratosis, sometimes with cracks and erosions. Foci of discoid K. v. more often single, less often multiple. Without treatment, they exist for years and, as a rule, do not cause discomfort. Erosive and ulcerative rashes in the mouth cause pain. They occur especially persistently in smokers. Disseminated discoid K. v. characterized by scattered erythematous-edematous, papular elements or discoid-type lesions. Predominant localization: face, open part of the chest and back, hands, feet, skin over the elbow and knee joints. The general condition of patients with discoid and disseminated K. v., as a rule, does not noticeably suffer. However, during wedge examination, 20-50% of patients reveal arthralgia, functional disorders, disorders of the internal organs (heart, stomach, kidneys), nervous system, accelerated ROE, leukopenia, hypochromic anemia, changes in the composition of immunoglobulins, antinuclear antibodies, immune complexes in the area of ​​the dermoepidermal junction, etc.

Deep K. v. (L. e. profundus Kaposi - Irgang) is characterized by the simultaneous presence of typical skin lesions characteristic of discoid K. v., and nodes in the subcutaneous tissue, the skin over which is mostly unchanged. A number of authors, eg. Pautrier (L. M. Pautrier), consider this form as a combination of deep Darrieus-Russi sarcoids and discoid K. v.

Complications

Occasionally, skin cancer develops, mainly in lesions on the red border of the lower lip, very rarely - sarcoma, erysipelas; a serious complication, more often observed with disseminated discoid K. v., is its transition to systemic K. v. under the influence of unfavorable factors.

Diagnosis

The diagnosis in typical cases is established without difficulty. Foci of discoid K. v. may be similar to seborrheic eczema, rosacea, psoriasis, eosinophilic granuloma of the face, tuberculous lupus. Clear boundaries of the lesions, horny plugs in dilated hair funnels, tightly fitting scales, a positive Besnier-Meshchersky sign, and the development of atrophy indicate the presence of K. v. Foci of seborrheic eczema (see) do not have such sharp boundaries, their surface is covered with loose, fatty scales, they respond well to antiseborrheic therapy. Psoriatic lesions are usually numerous, covered with easily scraped off silvery scales (see Psoriasis). Both of them, in contrast to K. v. usually decrease under the influence of sunlight. With rosacea (see) there is diffuse erythema, telangiectasia is pronounced, nodules and pustules often appear. Eosinophilic granuloma of the face (see) is characterized by particular resistance to therapeutic effects. Its foci are often single, uniform brown-red color, without hyperkeratosis, with isolated telangiectasias. Tuberculous lupus (see Tuberculosis of the skin) usually begins in childhood, it is characterized by the presence of lupoma with the characteristic apple jelly and probe phenomena. In cases of erythematous tuberculous lupus erythematosus of Leloir, the wedge, the diagnosis is extremely difficult, a histol examination is necessary. Discoid K. v. should also be differentiated with Essner-Kanoff lymphocytic infiltration; manifestations of the cut are less persistent, tend to resolve in the center, lack of peeling, hyperkeratosis, and atrophy. K.v. on the scalp differentiated from pseudopelade (see). The latter is characterized by the absence of inflammation, horny spines, finger-like arrangement, and more superficial atrophy. Discoid K. v. on the oral mucosa should be distinguished from lichen planus, the rashes of which have a more delicate pattern and are not accompanied by atrophy.

Patients with discoid K. v., including limited forms, should be examined to exclude systemic damage to internal organs and the nervous system, as well as to identify concomitant diseases.

Treatment

Leading role in the treatment of discoid and disseminated K. v. belongs to aminoquinoline drugs - chloroquine, resokhin, delagil y, plaquenil y, etc. They are prescribed continuously or in cycles, usually 0.25 g 2 times, Plaquenil - 0.2 g 3 times a day after meals. The duration of cycles (5-10 days) and the intervals between them (2-5 days) depends on the tolerability of treatment. Repeated courses of treatment are recommended, especially in spring. Adding small doses of corticosteroids (2-3 tablets of prednisolone per day) to chloroquine improves treatment results and tolerability. This technique is recommended for particularly persistent cases of K. v., extensive skin lesions.

It is useful to include vitamins B6, B12, calcium pantothenate, and nicotinic acid in the therapeutic complex. Treatment the effect occurs faster with the simultaneous administration of ointments with fluoride-containing corticosteroids (sinalar, flucinar, etc.), which, in case of limited lesions, can be the main method of therapy. It is also recommended to administer intradermally into the affected areas 5% chloroquine solution once every 5-7 days (4-6 injections per course). Limited lesions with a strong infiltrate and hyperkeratosis without signs of peripheral growth can be subjected to cryotherapy.

Forecast

The prognosis for life is favorable. With adequate treatment and patients following the recommended regimen, their ability to work remains for many years.

Prevention

Patients K. v. subject to medical examination. They must comply with the gig. mode of work, rest, nutrition, avoid physical activity. and nervous overload, exposure to the sun, wind, frost, use photoprotective creams and films with para-aminobenzoic acid, tannin, etc. It is necessary to sanitize foci of focal infection. For the treatment of concomitant diseases of patients with K. v. should not be directed to the south. resorts in the spring and summer, they should be prescribed physiotherapeutic procedures with caution, and vaccinated only for serious indications.

Drug-induced lupus erythematosus

Medicinal K. v. develops in connection with long-term use of apressin (hydralazine), procainamide (procainamide), diphenine (hydantoin), trimethine (trimethadione), carbazepine, isoniazid and chlorpromazine. Medicinal K. v. can develop in elderly people suffering from hypertension and arrhythmia, in patients with tuberculosis and epilepsy. The listed drugs are capable of causing the formation of antinuclear antibodies (ANF, antibodies to DNA), the appearance of which precedes the clinical manifestations of medicinal K. v., reminiscent of systemic K. v. When taking certain medications, a certain wedge or syndrome occurs. So, with apressin K. v. glomerulonephritis develops; with long-term use of nicotinamide, pleurisy and pneumonitis are very common, which are the beginning of the syndrome.

Among the mechanisms of development of medicinal To. v. The role of predisposition is discussed, since such a reaction occurs in approximately 10% of patients taking apressin and other drugs, as well as metabolic disorders, in particular the rate of acetylation of these drugs.

The diagnosis is made based on taking the listed medications.

Timely recognition of the disease and discontinuation of the drug that caused medicinal K. v. leads to recovery, however, it may be necessary to prescribe corticosteroids in medium doses (20-30 mg of prednisolone per day), especially with isoniazid medicinal K. v. With the development of the clinic of systemic K. century. appropriate therapeutic tactics are necessary.

Tables

Table 1. Clinical and laboratory indicators of the degree of activity of systemic lupus erythematosus

Level of activity of systemic lupus erythematosus

“Butterfly” and lupus-type erythema

Cardiosclerosis, myocardial dystrophy

Mitral valve insufficiency

Damage to one (usually the mitral) valve

Multiple valve damage

Nephritic or urinary syndrome

LE cells (per 1 thousand leukocytes)

Single or absent

Antinuclear factor (in credits)

Homogeneous and edge

Antibodies to nDNA (in credits)

Table 2. Approximate scheme for reducing the dose of prednisolone depending on the initial (maximum) dose

Initial (maximum) dose of prednisolone, mg per day

Reducing the dose of prednisolone by week, mg per day

Bibliography: Vinogradova O. M. Systemic lupus erythematosus in the clinic of internal diseases, Sov. med., No. 4, p. 15, 1958; G u s e v a L. L. and JI u n i n s k a i I. R. Psychopathological manifestations in systemic lupus erythematosus, Zhurn, neuropath, and psychiat., t. 75, century. 4, p. 562, 1975, bibliogr.; Davydovsky I.V. On the issue of lupus erythematodes disseminatus acutus, Rus. Vestn. derm., vol. 7, no. 5, p. 450, 1929, bibliogr.; And Smailov T. I. and F r u m k i-n and S. L. On the psychopathology and pathogenesis of symptomatic psychosis in systemic lupus erythematosus, Zhurn, neuropath, and psychiat., t. 72, no. 12, p. 1860, 1972; L e l and with I. I. Lupus erythematosus, L., 1970, bibliogr.; Meshchersky G.I. and Grinchar F.N. About the case of erythema faciei perstans (Kaposi - Kreibich’a) of tuberculous origin, Kharkov. pathologist Sat., dedicated. prof. M. N. Nikiforov, on the 25th anniversary of his scientist, activity, p. 406, M., 1911; Nasonova V. A. Systemic lupus erythematosus, M., 1972, bibliogr.; S e r about in V.V. and others. Immunomorphological characteristics of skin changes in lupus erythematosus, Soz. med., No. 9, p. 15, 1972; S e r about in V.V. and others. Electron microscopic characteristics of lupus nephritis, Arch. pathol., t. 36, no. 6, p. 21, 1974, bibliogr.; S to r i p k i n Yu. K., Somov B. A. and B u t o v Yu. S. Allergic dermatoses, p. 130, M., 1975, bibliogr.; With t r u-k about in A. I. and B e g l a r I n A. G. Pathological anatomy and pathogenesis of collagen diseases, p. 248, M., 1963; Tare-e in E. M. Collagenoses, M., 1965, bibliogr.; Tareeva I.E. Lupus nephritis, M., 1976, bibliogr.; Tareeva I. E., Serov V. V. and Kupriyanova L. A. Intraendothelial inclusions in systemic lupus erythematosus, Bull. Experiment, biol, and med., v. 77, no. 5, p. 119, 1974; O' C o n n o r J. F. a. Musher D. M. Central nervous system involvement in systemic lupus erythematosus, Arch. Neurol. (Chic.), v. 14, p. 157, 1966; Hargraves M. M., Richmond H. a. M o r t o n R. Presentation of two bone marrow elements, the “tart” cell and the “L. E." cell, Proc. Mayo Clin., v. 23, p. 25, 1948; Klemperer P., Pollack A. D. a. Baehr G. Pathology of disseminated lupus erythematosus, Arch. Path., v. 32, p. 569, 1941; Lupus erythematosus, ed. by E. L. Dubois, Los Angeles, 1974; Recent advances in rheumatology, ed. by W. W. Buchanan a. W. C. Dick, pt 1, Edinburgh -L., 1976; Ropes M. W. Systemic lupus erythematosus, Cambridge - L., 1976, bibliogr.

V. A. Nasonova; L. A. Isaeva (ped.), A. I. Strukov, L. V. Kaktursky (pat. an.), A. S. Tiganov (psychiat.), L. Ya. Trofimova (derm.).

Lupus erythematosus (lupus erythematodes, lupus erythematosus) is a group of diseases that combine systemic, cutaneous and drug-induced lupus erythematosus. The listed diseases have a number of common features: they mainly affect women, erythematous skin rashes and enanthema on the mucous membranes, and increased sensitivity to solar and UV irradiation are observed. There are cases where individual patients with cutaneous lupus erythematosus develop signs of systemic lupus erythematosus over time.

However, the differences between these two diseases (clinical, including in the nature of skin rashes, immunological and immunogenetic) are still much greater than the similarities. With cutaneous lupus erythematosus, isolated or predominant skin lesions are observed; Drug-induced and systemic lupus erythematosus is characterized by multiple syndromes, and the latter also has a severe progressive course.

In the International Classification of Diseases, Injuries and Causes of Death, cutaneous lupus erythematosus is classified in class XII “Diseases of the skin and subcutaneous tissue,” and systemic and drug-induced lupus erythematosus is classified in class XIII “Diseases of the musculoskeletal system and connective tissue.” Some experts consider lupus erythematosus as a single disease that has two forms: cutaneous and systemic.

Cutaneous lupus erythematosus

Cutaneous lupus erythematosus is most often represented by discoid lupus erythematosus, centrifugal erythema of Biette and the so-called deep lupus erythematosus. Discoid lupus erythematosus is characterized by three cardinal clinical symptoms: erythema, hyperkeratosis and atrophy.

At the beginning of the disease, a small pink or red spot with clear boundaries appears, which is gradually covered in the center with small dense grayish-white dry scales. They hold tightly due to the presence on their lower surface of spike-like protrusions, immersed in the dilated follicular ostia (follicular hyperkeratosis). When removing the scales, pain appears (Besnier-Meshchersky symptom).

Gradually, cicatricial atrophy begins to appear in the center of the lesion, and the lesion takes on a form pathognomonic for discoid lupus erythematosus: in the center there is a smooth, delicate white atrophic scar, further to the periphery there is a zone of hyperkeratosis and infiltration, outside there is a corolla of hyperemia, the typical localization of the lesion is in open areas of the skin : face, especially on the nose and cheeks with the formation of a butterfly figure (the so-called lupus butterfly), ears, neck. The scalp and red border of the lips are often affected. The lesions may be located on the oral mucosa, where they can erode.

With centrifugal erythema Bietto (the so-called superficial form of cutaneous lupus erythematosus), of the three main skin symptoms characteristic of discoid lupus erythematosus, only hyperemia is clearly expressed, while scales and cicatricial atrophy are almost or completely absent. The lesions are usually located on the face and often resemble a butterfly shape.

Multiple foci of discoid lupus erythematosus or centrifugal erythema of Biette scattered over various areas of the skin are defined as disseminated lupus erythematosus.

Among the rare forms of cutaneous lupus erythematosus, deep Kaposi-Irganga lupus erythematosus is distinguished, in which, along with the usual foci, there is one or more sharply demarcated dense mobile nodes covered with normal skin.

Cutaneous lupus erythematosus is characterized by a long-term continuous course with deterioration in the spring and summer due to photosensitivity.

Systemic lupus erythematosus

Systemic lupus erythematosus (lupus erythematosus systemicus) is a chronic progressive polysyndromic disease, which is characterized by genetically determined imperfection of immunoregulatory processes, the development of autoimmune disorders and immune complex chronic inflammation. Mostly women aged 20–30 years are affected (the ratio with the disease in men is 10:1), often teenagers.

The etiology and pathogenesis of systemic lupus erythematosus have not been fully elucidated. The viral genesis of the disease (in particular, the participation of retroviruses) in combination with a family genetic predisposition is assumed. The role of sex hormones (the onset of menstruation, abortion, childbirth), and the general connection of the disease with gender and age are also discussed. Systemic lupus erythematosus is a classic autoimmune disease in which the development of a hyperimmune response is observed against the unchanged components of one's own cells (nuclear and cytoplasmic), especially native DNA. Circulating antinuclear antibodies can form immune complexes, which, deposited in various organs, cause chronic inflammation and local or systemic tissue damage. Systemic connective tissue disorganization and generalized vascular damage are usually observed.

Systemic lupus erythematosus is characterized by the development of glomerulonephritis (lupus nephritis).

The disease most often begins with recurrent arthritis, malaise, fever, skin rashes, rapid weight loss, and less often with high fever, acute arthritis and a pronounced characteristic skin syndrome. Subsequently, progressive pathology develops in various organs.

Arthritis is observed in 80-90% of patients. Non-erosive chronic polyarthritis of small joints of the hands, wrist and ankle joints, less often of larger joints, myalgia and myositis are common.

Skin lesions are varied and have important diagnostic value. Only in 10-15% of patients they may be absent (lupus sine lupo), however, such a condition is temporary, transient.

The most common skin lesions are isolated or confluent erythematous spots of various shapes and sizes, more or less edematous, sharply demarcated from the surrounding healthy skin, which are usually observed on the face, neck, chest, elbow, knee and ankle joints. Typically, the appearance of erythema under the influence of solar and UV irradiation (photosensitization phenomenon). Skin changes in the midface are less common. Sometimes the butterfly has the appearance of persistent erysipelas with severe swelling of the face, especially the eyelids.

Patients with systemic lupus erythematosus often have trophic disorders (general dry skin, diffuse hair loss, deformation and brittleness of nails). The most common and varied skin changes are observed during the acute and subacute course of the disease.

Damage to the serous membranes is observed during the course of the disease in almost all patients in the form of dry or effusion pleurisy and pericarditis, less commonly peritonitis, perisplenitis and perihepatitis. Systemic lupus erythematosus is characterized by polyserositis.

The pathological process often spreads to the heart (lupus carditis), affecting all its membranes. Raynaud's syndrome occurs in 15-20% of patients and is an early sign of systemic lupus erythematosus, one of the manifestations of systemic vasculitis. Often this symptom is combined with Hashimoto's thyroiditis, cytopenias and Sjögren's syndrome.

The duration of acute lupus erythematosus without treatment is no more than 1-2 years.

In the subacute course, the disease begins with arthralgia, recurrent arthritis, and various skin lesions. With subsequent exacerbations, new organs and systems are involved in the pathological process and polysyndromic behavior develops within 2-3 years; lupus nephritis is often observed with a frequent outcome in chronic renal failure and encephalitis.

Diagnostics. Laboratory tests used for systemic lupus erythematosus can determine inflammatory and immunological activity. More than half of the patients have leukopenia, reaching in some cases 1.2 x 109/l, in combination with lymphopenia (5-10% of lymphocytes). Quite often, hypochromic anemia is detected, due to various reasons, including gastric bleeding due to the development of ulcers during treatment with corticosteroids, and renal failure. With the development of hemolytic anemia, a positive Coombs test and moderate thrombocytopenia are noted; thrombocytopenic purpura rarely develops.

Of great diagnostic importance is the detection in the blood of LE cells, which are mature neutrophils, in the cytoplasm of which there are large inclusions - phagocytosed remnants of the nuclei of decayed neutrophils. LE cells are found in 2/3 of patients in an amount of 5 or more per 1000 leukocytes. Single LE cells can also be observed in other diseases. It is important for diagnosis to detect antinuclear antibodies in high titers - antinuclear factor, antibodies to native DNA, etc. In classical cases, the diagnosis of systemic lupus erythematosus is based on the detection of a diagnostic triad (lupus butterfly, recurrent non-erosive polyarthritis, polyserositis), LE cells or antinuclear antibodies ( including antinuclear factor) in diagnostic titers. Circumstances such as age, the connection of the onset of the disease with childbirth, abortion, the onset of menstruation, and excessive insolation have auxiliary diagnostic significance. In cases of monosyndromic onset of systemic lupus erythematosus, a differential diagnosis is necessary with other diffuse connective tissue diseases or rheumatic diseases - such as rheumatism, rheumatoid arthritis, juvenile chronic arthritis, etc.

Drug-induced lupus erythematosus

Drug-induced lupus erythematosus develops in some cases with long-term use of procainamide, isoniazid and hydralazine. The clinical picture is characterized by arthritis, erythematous skin rashes, serositis, and lung damage.

Discontinuation of the drug gradually leads to the elimination of the clinical and immunological manifestations of the disease.

Drug-induced lupus occurs approximately 10 times less frequently than systemic lupus erythematosus (SCR). Recently, the list of drugs that can cause lupus syndrome has expanded significantly. These include primarily antihypertensives (hydralazine, methyldopa); antiarrhythmic (procainamide); anticonvulsants (diphenin, hydantoin) and other drugs: isoniazid, aminazine, methylthiouracil, oxodoline (chlorthalidone), diuretin, D-penicillamine, sulfonamides, penicillin, tetracycline, oral contraceptives.

We observed severe nephrotic syndrome with the development of multisystem SLE, which required many years of treatment with corticosteroids, after administration of bilitrast to the patient. Therefore, you should carefully collect anamnesis before prescribing treatment.

The mechanism of development of drug-induced lupus may be due to changes in immune status or allergic reaction. Positive antinuclear factor is detected in drug-induced lupus caused by drugs of the first three groups listed above. The detection rate of antinuclear factor in drug-induced lupus is higher than in true SLE. Hydralazine and procainamide are especially capable of inducing the appearance of antinuclear, antilymphocyte, and antierythrocyte antibodies in the blood. These antibodies themselves are harmless and disappear when you stop taking the drug.

Sometimes they persist in the blood for several months without causing any clinical symptoms. During development. autoimmune process in a small percentage of patients with a genetic predisposition develop lupus syndrome. The clinical picture is dominated by polyserositis and pulmonary symptoms. Skin syndrome, lymphadenopathy, hepatomegaly, and polyarthritis are observed. In the blood - hypergammaglobulinemia, leukopenia, antinuclear factor, LE cells; the test for antibodies to native DNA is usually negative, the complement level is normal.

Antibodies to single-stranded DNA and antibodies to nuclear histone can be detected. The absence of complement-fixing antibodies partly explains the rarity of renal involvement. Although damage to the kidneys and central nervous system is rare, it can develop with prolonged and persistent use of the drugs listed above. Sometimes all disorders disappear soon after discontinuation of the drug that caused the disease, but in some cases it is necessary to prescribe corticosteroids, sometimes for quite a long time. Severe cases of lupus with cardiac tamponade due to pericarditis, requiring treatment for many years, have been described with the use of hydralazine.

Treatment

At the onset of the disease, it is sometimes difficult to predict its outcome, and only extensive clinical experience and observation of a significant number of patients make it possible to determine some prognostic signs and choose the right treatment method in order not only to help the patient, but also not to harm him with the so-called aggressive therapy. Unfortunately, All drugs used for SLE have one side effect or another, and the stronger the drug, the greater the danger of such an effect. This further emphasizes the importance of determining the activity of the disease, the severity of the patient’s condition, and damage to vital organs and systems.

The main drugs for the treatment of patients with SLE Corticosteroids, cytostatic immunosuppressants (azathioprine, cyclophosphamide, chlorambucil), as well as 4-aminoquinoline derivatives (plaquenil, delagil) remain. Recently, methods of so-called mechanical blood purification have gained recognition: plasma exchange, lymphpheresis, immunosorption. In our country, hemosorption is more often used - blood filtration through activated carbon. Used as an additional means non-steroidal anti-inflammatory drugs (NSAIDs).

When treating patients with systemic lupus erythematosus, an individual approach to the choice of therapy is necessary (since there are so many variants of the disease that we can talk about the unique course of SLE in each patient and individual response to treatment) and establishing contact with patients, since they need to be treated throughout their lives , determining, after suppressing the acute phase in the hospital, a set of rehabilitation measures, and then a set of measures to prevent exacerbation and progression of the disease.

It is necessary to train (educate) the patient, convince him of the need for long-term treatment, compliance with the recommended rules of treatment and behavior, teach him to recognize how earlier signs side effects of medications or exacerbation of the disease. With good contact with the patient, complete trust and mutual understanding, many issues of mental hygiene that often arise in patients with SLE, as in all long-term ill people, are resolved.

Corticosteroids

In case of severe organ pathology, the daily dose of corticosteroids should be at least 1 mg/kg body weight with a very gradual transition to a maintenance dose. Analysis of our data obtained from the treatment of more than 600 patients with SLE with a reliably established diagnosis, observed at the Institute of Rheumatology of the Russian Academy of Medical Sciences from 3 to 20 years, showed that 35% of patients received a daily dose of prednisolone of at least 1 mg/kg. If the dose was less than indicated, combination therapy with cytostatic immunosuppressants was carried out.

Most patients received corticosteroids in maintenance doses continuously for more than 10 years. Patients with lupus nephritis or lupus of the central nervous system received 50-80 mg of prednisolone (or equivalent other corticosteroid drug) daily for 1-2 months with a gradual reduction over the year of this dose to a maintenance dose (10-7.5 mg), which is the majority patients were admitted for 5-20 years.

Our observations showed that in a number of patients with cutaneous-articular syndrome without severe visceral manifestations, it was necessary to add corticosteroids at a dose of 0.5 mg/(kg day) to quinoline drugs and NSAIDs and carry out long-term maintenance treatment (5-10 mg per day) due to persistent spread of the skin process, frequent exacerbation of arthritis, exudative polyserositis, myocarditis, which occurred when trying to cancel even such a maintenance dose as 5 mg of the drug per day.

Although assessment of the effectiveness of corticosteroids for SLE has never been conducted in controlled trials in comparison with placebo, however, all rheumatologists recognize their high effectiveness in severe organ pathology. Thus, L. Wagner and J. Fries in 1978 published data from 200 US rheumatologists and nephrologists who observed 1900 patients with systemic lupus erythematosus. With active nephritis in 90% of patients, the daily dose of corticosteroids was at least 1 mg/kg. For CNS lesions, all patients received corticosteroids at a dose of at least 1 mg/kg per day.

The authors emphasize the need for long-term treatment of seriously ill patients with SLE, a gradual dose reduction, which is consistent with the data of our long-term observation. Thus, the generally accepted tactic is to switch from 60 mg of prednisolone per day to a daily dose of 35 mg for 3 months, and to 15 mg only after another 6 months. Essentially, over the years, the dosage of the drug (both initial and maintenance) was adjusted empirically.

Of course, certain dosage provisions have been established in accordance with the degree of disease activity and certain visceral pathology. Most patients experience improvement when using adequate therapy. It is clear that in some cases improvement is observed only with a daily dose of prednisolone 120 mg for several weeks, in other cases - more than 200 mg per day.

In recent years, there have been reports of the effective intravenous use of ultra-high doses methylprednisolone(1000 mg/day) for short period(3-5 days). Such loading doses of methylprednisolone (pulse therapy) were initially used only for resuscitation and kidney transplant rejection. In 1975, we had to use intravenous loading doses of prednisolone (1500-800 mg per day) for 14 days in a patient with chronic SLE due to an exacerbation of the disease that developed after a cesarean section. The exacerbation was accompanied by adrenal insufficiency and a drop in blood pressure, which was stabilized only with the help of pulse therapy followed by oral administration of the drug 40 mg per day for 1 month.

E. Cathcart et al were among the first to report pulse therapy in patients with lupus nephritis. in 1976, who used 1000 mg of methylprednisolone intravenously for 3 days in 7 patients and noted an improvement in renal function, a decrease in serum creatinine levels, and a decrease in proteinuria.

Subsequently, reports from a number of authors appeared, mainly concerning the use of pulse therapy for lupus nephritis. According to all authors, ultra-high doses of methylprednisolone administered intravenously for short periods of time rapidly improve renal function in cases of lupus nephritis in cases with recently developed renal failure. Pulse therapy began to be used in other patients with systemic lupus erythematosus without kidney damage, but during periods of crisis, when all previous therapy was ineffective.

To date, the Institute of Rheumatology of the Russian Academy of Medical Sciences has experience with the intravenous use of 6-methylprednisolone in 120 patients with SLE, most of them with active lupus nephritis. Immediate good results were observed in 87% of patients. Analysis of long-term results after 18-60 months showed that remission remained in 70% of patients, of which 28% had complete disappearance of signs of nephritis.

The mechanism of action of loading doses of methylprednisolone during intravenous administration has not yet been fully disclosed, but available data indicate a significant immunosuppressive effect already in the first day. A short course of intravenous administration of methylprednisolone causes a significant and long-term decrease in serum IgG levels due to increased catabolism and decreased synthesis.

It is believed that loading doses of methylprednisolone suspend the formation of immune complexes and cause a change in their mass by interfering with the synthesis of antibodies to DNA, which in turn leads to a redistribution of the deposition of immune complexes and their release from the subendothelial layers of the basement membrane. It is also possible to block the damaging effects of lymphotoxins.

Taking into account the ability of pulse therapy to quickly suspend the autoimmune process for a certain period of time, the provision on the use of this method only during the period when other therapy no longer helps should be reconsidered. Currently, a certain category of patients has been identified (young age, rapidly progressing lupus nephritis, high immunological activity), in whom this type of therapy should be used at the onset of the disease, since with early suppression of disease activity, long-term therapy with large amounts may not be necessary in the future. doses of corticosteroids, fraught with serious complications.

A large number of complications of corticosteroid therapy with long-term use, especially such as spondylopathy and avascular necrosis, forced the search for additional treatment methods, ways to reduce doses and course of treatment with corticosteroids.

Cytostatic immunosuppressants

Nevertheless, long-term observation has made it possible to develop specific indications for the use of these drugs. Indications for their inclusion in the complex treatment of patients with systemic lupus erythematosus are: 1) active lupus nephritis; 2) high overall disease activity and resistance to corticosteroids or the appearance of adverse reactions of these drugs already in the first stages of treatment (especially the phenomena of hypercortisolism in adolescents, developing already with small doses of prednisolone); 3) the need to reduce the maintenance dose of prednisolone if it exceeds 15-20 mg/day.

There are various combination treatment regimens: Azathioprine and cyclophosphamide orally at an average dose of 2-2.5 mg/(kg day), chlorobutine 0.2-0.4 mg/(kg day) in combination with low (25 mg) and medium (40 mg) doses prednisone. In recent years, several cytostatics have been used simultaneously: azathioprine + cyclophosphamide (1 mg/kg per day orally) in combination with low doses of prednisolone; a combination of oral azathioprine with intravenous cyclophosphamide (1000 mg per 1 m 3 of body surface every 3 months). With this combination treatment, a slowdown in the progression of lupus nephritis was noted.

In recent years, methods of only intravenous administration of cyclophosphamide have been proposed (1000 mg per 1 m 3 of body surface once a month in the first six months, then 1000 mg per 1 m 3 of body surface every 3 months for 1.5 years) against a background of low doses of prednisolone.

Comparison of the effectiveness of azathioprine and cyclophosphamide in double-blind controlled trials showed that cyclophosphamide was more effective in reducing proteinuria, reducing changes in urinary sediment, and the synthesis of antibodies to DNA. In our comparative study (double-blind method) of three drugs - azathioprine, cyclophosphamide and chlorambucil - it was noted that chlorambucil is similar in its effect on “renal” parameters to cyclophosphamide. A clear effect of chlorambucil on articular syndrome was also revealed, while azathioprine turned out to be most effective for diffuse skin lesions.

The effectiveness of cytostatics in SLE is confirmed by the fact of suppression of pronounced immunological activity. J. Hayslett et al. (1979) noted a significant decrease in inflammatory phenomena in a kidney biopsy in 7 patients with severe diffuse proliferative nephritis. When combining treatment with corticosteroids and azathioprine, S. K. Soloviev et al. (1981) discovered a change in the composition of deposits in the dermoepidermal junction during a dynamic immunofluorescent study of a skin biopsy: under the influence of cytostatics in patients with active lupus nephritis, the IgG glow disappeared.

The introduction of cytostatics into the treatment complex makes it possible to suppress disease activity with lower doses of corticosteroids in patients with highly active SLE. The survival rate of patients with lupus nephritis has also increased. According to I. E. Tareeva and T. N. Yanushkevich (1985), 10-year survival is observed in 76% of patients with combined treatment and in 58% of patients treated with prednisolone alone.

With individual selection of doses and regular monitoring, the number of adverse reactions and complications can be significantly reduced. Such serious complications as malignant tumors such as reticulosarcomas, lymphomas, leukemia, hemorrhagic cystitis and bladder carcinoma are extremely rare. Of the 200 patients who received cytostatics at the Institute of Rheumatology of the Russian Academy of Medical Sciences and were observed from 5 to 15 years, one patient developed gastric reticulosarcoma, which does not exceed the incidence of tumors in patients with autoimmune diseases not treated with cytostatics.

The Standing Committee of the European League Against Rheumatism, which studied the results of the use of cytostatic immunosuppressants in 1375 patients with various autoimmune diseases, has not yet recorded a higher incidence of malignant neoplasms in them compared to the group in which these drugs were not used. We observed agranulocytosis in two patients. It was controlled by increasing the dose of corticosteroids. Attachment of a secondary infection, including viral ( herpes zoster), was no more common than in the group treated with prednisolone only.

Nevertheless, taking into account the possibility of complications of cytostatic therapy, strict justification for the use of these potent drugs, careful monitoring of patients, and examination of them every week from the moment treatment is prescribed are necessary. Evaluation of long-term results shows that if the treatment method is followed, the number of complications is small, and there are no harmful effects therapy for the next generation. According to our data, 15 children born to patients with systemic lupus erythematosus treated with cytostatics are healthy (their follow-up period was more than 12 years).

Plasmapheresis, hemosorption

The use of plasmapheresis and hemosorption is based on the possibility of removing biologically active substances from the blood: inflammatory mediators, circulating immune complexes, cryoprecipitins, various antibodies, etc. It is believed that mechanical cleansing helps to unload the mononuclear cell system for a while, thus stimulating the endogenous phagocytosis of new complexes, which ultimately reduces the degree of organ damage.

It is possible that during hemosorption there is not just binding of serum immunoglobulins, but also a change in their composition, which leads to a decrease in the mass of immune complexes and facilitates the process of their removal from the bloodstream. It is possible that when blood passes through the sorbent, immune complexes change their charge, which explains the pronounced improvement observed in patients with kidney damage even with a constant level of immune complexes in the blood. It is known that only positively charged immune complexes are capable of depositing on the basement membrane of the glomeruli of the kidney.

A generalization of experience in the use of plasmapheresis and hemosorption shows the feasibility of including these methods in the complex treatment of patients with SLE with a torpid course of the disease and resistance to previous therapy. Under the influence of the procedures (3-8 per course of treatment), there is a significant improvement in the general well-being of patients (often not correlating with a decrease in the level of circulating immune complexes and antibodies to DNA), a decrease in signs of disease activity, including nephritis with preservation of kidney function, the disappearance of pronounced skin changes and a distinct acceleration of healing of trophic ulcers of the extremities. Both plasmapheresis and hemosorption are carried out while taking corticosteroids and cytostatics.

Although there is not yet sufficient data obtained from control studies and in determining the survival of patients treated with plasmapheresis or hemosorption, the use of these methods opens up new opportunities for reducing high disease activity and preventing its progression as a result of influencing the immunopathological process.

Among other methods of so-called aggressive therapy used for severe forms of systemic lupus erythematosus, mention should be made of local X-ray irradiation of the supra- and subdiaphragmatic lymph nodes (up to 4000 rad per course). This makes it possible to reduce extremely high disease activity, which is not achievable with other treatment methods. This method is under development.

Immunomodulatory drugs- levamisole, frentisole - have not received widespread use in SLE, although there are individual reports of the effect obtained when these drugs are included in therapy with corticosteroids and cytostatics in forms of the disease refractory to conventional treatment methods or when a secondary infection is attached. Most authors report a large number of serious complications in almost 50% of patients treated with levamisole. During more than 20 years of observation of patients with SLE, we used levamisole in isolated cases and always noted serious complications. A controlled trial of levamisole in systemic lupus erythematosus showed no efficacy. Apparently, it is advisable to add levamisole in cases of severe bacterial infection.

Aminoquinoline derivatives and non-steroidal anti-inflammatory drugs serve as the main drugs in the treatment of patients with SLE without severe visceral manifestations and during the period of reducing doses of corticosteroids and cytostatics to maintain remission. Our long-term observation has shown that the risk of developing ophthalmological complications is significantly exaggerated. This is also emphasized by J. Famaey (1982), who notes that complications develop only at a dose significantly higher than the optimal daily dose. At the same time, long-term use of these drugs in the complex treatment of patients with SLE is very effective.

Of the aminoquinoline drugs, delagil (0.25-0.5 g/day) and plaquenil (0.2-0.4 g/day) are usually used. Of the non-steroidal anti-inflammatory drugs, indomethacin is most often used as additional drug for persistent arthritis, bursitis, polymyalgia, as well as Voltaren, Ortofen.

Treatment of patients with SLE with central nervous system involvement

In cases where neuropsychiatric disorders occur while taking corticosteroids and it is difficult to determine whether they are caused by prednisolone or active systemic lupus erythematosus, increasing the dose of prednisolone is safer than decreasing it. If neuropsychiatric symptoms increase when the dose is increased, the dose can always be reduced. Of the cytostatics, cyclophosphamide is the most effective, especially its intravenous administration in the form of pulse therapy. Often, in acute psychosis, along with prednisolone, it is necessary to use antipsychotics, tranquilizers, and antidepressants to relieve psychosis.

Upon appointment anticonvulsants It is important to remember that anticonvulsants accelerate the metabolism of corticosteroids, which may require increasing the dose of the latter. For chorea, the effectiveness of prednisolone has not been proven; there are cases of its spontaneous relief. Recently, anticoagulants have been used to treat chorea. In the most severe situations associated with damage to the central nervous system, pulse therapy and plasmapheresis are performed.

Massive intravenous therapy with methylprednisolone (500 mt daily for 4 days) is also effective for cerebrovasculitis with initial signs of coma. However, three cases of signs of damage to the nervous system appearing after pulse therapy in patients with a previously intact central nervous system. The cause of this complication may be sudden water-electrolyte disturbance in the central nervous system, disruption of the permeability of the blood-brain barrier, the removal of immune complexes through the reticuloendothelial system.

With the improvement of the prognosis for SLE in general against the background adequate treatment Mortality in cases of central nervous system damage has also decreased. Nevertheless, the development of adequate therapeutic and rehabilitation measures for damage to the central nervous system requires continued research in this area.

Corticosteroids and cytostatics in various regimens and combinations remain the basis for the treatment of lupus nephritis.

Many years of experience of two centers (Institute of Rheumatology of the Russian Academy of Medical Sciences, I.M. Sechenov Moscow Medical Academy) made it possible to develop treatment tactics for patients with lupus nephritis, depending on the activity and clinical form of nephritis.

For rapidly progressing glomerulonephritis, when rapid nephrotic syndrome, high hypertension and renal failure are observed at an early stage of the disease, the following regimens can be selectively used:

1) pulse therapy with methylprednisolone + cyclophosphamide 3-6 times monthly, in between - prednisolone 40 mg per day with a dose reduction by the 6th month to 30-20 mg/day and in the next 6 months - to a maintenance dose of 5-10 mg /day, which should be taken for 2-3 years, and sometimes for life. Maintenance therapy is required when using any of the treatment regimens carried out in a hospital, and usually includes, in addition to corticosteroids and cytostatics, aminoquinoline drugs (1-2 tablets per day of Plaquenil or Delagil), antihypertensives, diuretics, angioprotectors, disaggregants, which should be taken in for 6-12 months (courses are repeated if necessary);

2) prednisolone 50-60 mg/day + cyclophosphamide 100-150 mg/day for 2 months in combination with heparin 5000 units 4 times a day for 3-4 weeks and chimes 600-700 mg per day. Then the daily doses of prednisolone are reduced to 40-30 mg, cyclophosphamide to 100-50 mg and treatment is carried out for another 2-3 months, after which maintenance therapy is prescribed in the doses indicated above (see point 1).

Both treatment regimens should be carried out against the background of plasmapheresis or hemosorption (prescribed once every 2-3 weeks, a total of 6-8 procedures), antihypertensive and diuretic drugs. In case of persistent edema, plasma ultrafiltration can be resorted to; in case of increasing renal failure, 1-2 courses of hemodialysis are advisable.

For nephrotic syndrome, you can choose one of the following three regimens:

1) prednisolone 50-60 mg per day for 6-8 weeks, followed by a dose reduction to 30 mg for 6 months and to 15 mg over the next 6 months;

2) prednisolone 40-50 mg + cyclophosphamide or azathioprine 100-150 mg per day for 8-12 weeks, subsequently the rate of reduction in the dose of prednisolone is the same, and cytostatics continue to be prescribed at 50-100 mg/day for 6-12 months;

3) combined pulse therapy with methylprednisolone and cyclophosphamide or an intermittent regimen: pulse therapy with methylprednisolone - hemosorption or plasmapheresis - pulse therapy with cyclophosphamide followed by treatment with oral prednisolone 40 mg per day for 4-6 weeks and then switching to a maintenance dose for 6 weeks 12 months

Symptomatic therapy remains important.

For active nephritis with severe urinary syndrome (proteinuria 2 g/day, erythrocyturia 20-30 in the field of view, but blood pressure and renal function are not significantly changed), treatment regimens may be as follows:

1) prednisolone 50-60 mg 4-6 weeks + aminoquinoline drugs + symptomatic agents;

2) prednisolone 50 mg + cyclophosphamide 100 mg per day for 8-10 weeks, then the rate of reduction in the doses of these drugs and maintenance therapy are carried out as indicated above;

3) pulse therapy with methylprednisolone, combined with cyclophosphamide, is possible (3-day course of 1000 mg methylprednisolone every day and 1000 mg cyclophosphamide one day), followed by prednisolone 40 mg for 6-8 weeks, then dose reduction for 6 months up to 20 mg/day. Then, for many months, maintenance therapy according to the principles described above.

In general, active therapy for patients with lupus nephritis should be carried out for at least 2-3 months. After the exacerbation subsides, long-term maintenance therapy is prescribed with small doses of prednisolone (at least 2 years after the exacerbation), cytostatics (at least 6 months), aminoquinoline drugs, sometimes methindole, chimes, antihypertensives, and sedatives. All patients with lupus nephritis should undergo regular examinations at least once every 3 months with assessment of clinical and immunological activity, determination of renal function, proteinuria, and urinary sediment.

When treating patients with terminal lupus nephritis and nephrosclerosis, hemodialysis and kidney transplantation are used, which can significantly increase life expectancy. Kidney transplantation is performed in patients with SLE with a developed picture of uremia. The activity of systemic lupus erythematosus usually completely subsides by this time, so fears of an exacerbation of SLE with the development of lupus nephritis in the graft should be considered not entirely justified.

Prospects for treating patients with SLE, undoubtedly, behind biological methods of influence. In this regard, the use of anti-idiotypic monoclonal antibodies offers great opportunities. So far, only the experiment has shown that the repeated use of syngeneic monoclonal IgG antibodies to DNA obtained using the hybridoma technique delayed the development of spontaneous glomerulonephritis in New Zealand mouse hybrids by suppressing the synthesis of particularly damaging IgG antibodies to DNA, which carry a cationic charge and are nephritogenic.

Currently, the question of a dietary regimen for systemic lupus erythematosus has again been raised, since there is evidence of the influence of certain nutrients on the mechanism of inflammation, for example, on the concentration of precursors of inflammatory mediators in cell membranes, an increase or decrease in the response of lymphocytes, the concentration of endorphins and other intimate metabolic factors. mechanisms. The experiment obtained data on an increase in the life expectancy of New Zealand mouse hybrids even with a decrease in the total amount of food in the diet, and even more so with an increase in the content of eicosapentanoic acid, a representative of unsaturated fatty acids, in food to 25%.

A reduced content of linoleic acid in food leads to a decrease in the synthesis of prostaglandins and leukotrienes, which have a pro-inflammatory effect. In turn, with an increase in food content unsaturated acids the intensity of the processes of inflammation and fibrosis formation decreases. Knowing the effect of a diet with a certain content of fatty acids on various manifestations diseases in an experiment, one can approach the study of the effect of dietary regimens on the development of pathology in autoimmune diseases in humans.

Therapeutic programs for the main clinical variants of systemic lupus erythematosus are carried out against the background of corticosteroids and cytostatics prescribed orally, symptomatic remedies, including antihypertensive drugs, angioprotectors, antiplatelet agents, etc. Thus, although the problem of treating SLE cannot be considered completely resolved, modern methods Therapy makes it possible to achieve significant improvement in most patients, maintain their ability to work and return them to a normal lifestyle.

Sigidin Ya.A., Guseva N.G., Ivanova M.M.

lupus erythematosus is an inflammatory disease of an autoimmune nature. It occurs against the background of a malfunction of the immune system, in which, for reasons unknown to medicine, it begins to kill the maples of its own body, perceiving them as foreign. In this case, the immune system produces special antibodies, under the influence of which the internal organs of the patient are seriously affected.

There are three forms of lupus erythematosus - cutaneous or discoid, systemic and drug-induced.

Lupus erythematosus manifests symptoms in the form of patches of redness of the skin, which in ancient times people compared to wolf bites, hence the name of the disease. Skin damage is worsened by exposure to sunlight.

Discoid lupus erythematosus - symptoms

The first symptoms of discoid lupus erythematosus appear as small pinkish spots in the lips and oral mucosa. These spots gradually change shape, merge with each other, increasing in size and affecting larger areas of the skin. They are mainly localized on open areas of the skin, including those covered with hair, exposed to sunlight - arms, head, neck, upper back.

Discoid lupus erythematosus does not affect internal organs, but creates an ugly cosmetic effect on the surface of the skin. It can develop into the more serious systemic form of lupus erythematosus.

Systemic lupus erythematosus - symptoms

The first symptoms of systemic lupus erythematosus are very vague, common to many other diseases. This:

• malaise;
• slight increase in temperature;
• headache;
• decreased appetite;
• sleep disturbance.

Red spots may also appear in the area of ​​the nail plate, pain in the joints and muscles.

More serious symptoms of systemic lupus erythematosus are pathological changes in muscles, joints, and internal organs, in particular in the liver and heart. Also, lupus erythematosus manifests symptoms by affecting the nervous system. In this case, the patient may experience epileptic convulsions, inflammation of the meninges, depression, and other mental illnesses.

The composition of the blood changes, namely, the amount of hemoglobin and leukocytes may decrease. In almost half of patients with lupus erythematosus, the presence of special antibodies is detected in the blood - antiphospholipids, which react with cell membranes (containing phospholipids) and affect blood clotting. Patients whose blood contains antiphospholipids very often suffer from veins and arteries that provoke cardiac or cerebral strokes.

External manifestations of systemic lupus erythematosus appear in the form of rashes on the face, the so-called butterfly-shaped exudative erythema; rashes can also appear on the cheekbones. But very often the skin remains untouched, only the internal organs and systems of the body are affected.

Drug-induced lupus erythematosus - symptoms

Drug-induced lupus erythematosus occurs against the background of long-term use of certain medications prescribed in the treatment of cardiac arrhythmia. It manifests itself in the form of redness of the skin, arthritis, and damage to lung tissue.

As lupus disease worsens, symptoms may expand. Thus, the patient may begin to rapidly lose weight, lose hair in clumps, and his lymph nodes may swell.

As you can see, lupus erythematosus disease has symptoms that affect almost all organs and systems of the body. As the disease progresses, the symptoms worsen, and other serious pathologies and diseases develop. Therefore, having diagnosed lupus erythematosus, you need to begin treatment as quickly as possible.