Juvenile dermatomyositis (juvenile dermatomyositis). Juvenile dermatomyositis (Juvenile dermatomyositis) The pathognomonic manifestation of dermatomyositis is

Juvenile dermatomyositis- a disease from the group of diffuse connective tissue diseases with a predominant lesion of the proximal skeletal muscles, the development of muscle weakness, as well as purple erythema on the skin. Since the etiology of the disease is unclear, juvenile dermatomyositis is included in the heterogeneous group of idiopathic inflammatory myopathies with a leading clinical manifestation - inflammatory skeletal muscle lesions. According to R.L. Woltman (1994), in addition to juvenile dermatomyositis, this group also includes other myopathies.

Symptoms of juvenile dermatomyositis

In children, dermatomyositis often begins acutely or subacutely; at the onset of the disease, fever, weakness, malaise, weight loss, myalgia, arthralgia, and a progressive decrease in muscle strength often occur. The clinical picture of dermatomyositis is usually polysyndromic, but the most characteristic changes in the skin and muscles.

Skin lesion

Skin lesions are a characteristic sign of dermatomyositis. Skin manifestations of dermatomyositis include erythematous rashes with a purple tint on the face in the paraorbital region (a symptom of "dermatomyositis glasses"), in the décolleté area, over the metacarpophalangeal and proximal interphalangeal joints of the hands (Gottron's sign) and over large joints of the extremities, primarily the elbows and knee. In the acute period, patients often have superficial skin necrosis at the sites of injury, and subsequently develop atrophy with areas of depigmentation. Some patients observe redness, peeling and cracking of the skin of the palms ("mechanic's hand").

In children with dermatomyositis, bright livedo usually occurs, especially in the area of ​​the shoulder and pelvic girdle, capillaritis of the palms and feet, and telangiectasia. Generalized vascular lesions are especially characteristic of preschool children.

In acute and subacute course, pronounced trophic disorders are observed in the form of xeroderma, brittle nails, and alopecia.

Subcutaneous tissue injury

Over the affected muscles of the limbs and on the face, a testy or dense edema often appears. Perhaps the development of partial lipodystrophy of the face and limbs, usually combined with muscle atrophy.

Muscle damage

Usually, at the beginning of the disease, patients with dermatomyositis complain of fatigue during physical exertion, muscle pain that occurs spontaneously and is aggravated by palpation and movement. Dermatomyositis is characterized by a symmetrical lesion, primarily of the proximal muscles of the limbs, as a result of which children cannot carry a briefcase in their hands, it is difficult for them to raise their hands up and hold them in this position, they cannot comb their hair (“comb symptom”), get dressed (“symptom shirts”), quickly get tired when walking, often fall, cannot climb stairs, get up from a chair, raise and hold their legs. With severe damage to the muscles of the neck and back, patients cannot tear their heads off the pillow, turn around and get out of bed. In the most severe cases, generalized muscle weakness develops with an emphasis on the proximal group, as a result of which patients can be almost completely immobilized.

When the muscles of the larynx and pharynx are affected, a nasal and hoarse voice appears, as well as a violation of swallowing, which can lead to aspiration of food and saliva. With damage to the facial muscles, a mask-like face is noted, with damage to the oculomotor muscles - diplopia and ptosis of the eyelids. Severe damage to the diaphragm and intercostal muscles leads to respiratory failure. In the outcome of polymyositis, muscle hypotrophy develops.

In children, unlike adults, persistent, sometimes painful tendon-muscle contractures are often formed, which sharply limit the range of motion.

Joint damage

Joint damage is observed in more than 75% of patients. Develop arthralgia or polyarthritis. The most commonly affected joints are the small joints of the hands (mainly proximal interphalangeal), knee and elbow. Articular changes are characterized by moderate defiguration and pain on palpation and movement. In most cases, articular syndrome quickly stops during treatment, only 25% of patients note the formation of contractures, deformities and subluxations in the interphalangeal joints with some limitation of functionality.

Calcinosis

Calcinosis in dermatomyositis in children occurs 3-4 times more often than in adults. It develops in almost 40% of patients, mainly in the period from 1 to 5 years after the onset of the disease. Calcifications can be limited in the form of individual foci or plates and localized subcutaneously or in the connective tissue around the muscle fibers, they can also be located in the areas of greatest trauma - around the knee or elbow joints, along the Achilles tendon, on the hips, buttocks, shoulders. In patients with continuously relapsing dermatomyositis, calcification is usually diffuse.

Damage to internal organs

With dermatomyositis, myocarditis most often develops, manifested mainly by rhythm and conduction disturbances, and a decrease in the contractility of the heart muscle. In 25% of patients, pericarditis develops with mild symptoms that quickly disappear after the start of treatment with glucocorticoids.

Lung damage (pneumonitis) is associated with vascular-interstitial changes and is clinically manifested by an unproductive cough, shortness of breath, intermittent wheezing during auscultation. Prognostically unfavorable is the development of diffuse alveolitis with the formation of an alveolar-capillary block, the rapid development of pulmonary insufficiency and death. Damage to the lungs in dermatomyositis can also be due to the development of aspiration and banal hypostatic pneumonia due to damage to the muscles involved in swallowing and breathing. Often, pleurisy is found in children, with a high degree of activity of the process, sometimes accompanied by the formation of exudate.

Kidney damage is rare. The renal syndrome is represented by a transient urinary syndrome, in some cases accompanied by impaired renal function up to the development of acute renal failure due to massive myoglobinuria.

Often in children with a high activity of the process, esophagitis, gastroduodenitis, enterocolitis occur; the development of an erosive-ulcerative process, complicated by perforation and bleeding, is possible. Occasionally, a pseudo-abdominal syndrome is observed, resulting from damage to the muscles of the anterior abdominal wall, with edema, induration and severe pain during breathing and palpation.

Laboratory research

In a laboratory study, patients in the active period of the disease usually reveal an increase in ESR, moderate anemia, in some patients - moderate leukocytosis, hypergammaglobulinemia.

Among the biochemical parameters, characteristic changes reflecting damage to skeletal muscles include an increase in the activity of creatine phosphokinase, as well as aldolase. In addition, patients often show an increase in the concentration of LDH and aminotransferases in the blood serum. Some patients develop myoglobinuria.

The detection of myositis-specific antibodies is important primarily for classification, i.e. clarification of the clinical and immunological subtype of dermatomyositis and polymyositis. In some patients, antibodies to tRNA aminoacyl synthetases are detected, primarily antibodies to histidyl-tRNA synthetase (Jo-1). In the presence of these antibodies in the blood, an antisynthetase syndrome develops, characterized by an acute onset of myositis, interstitial lung damage, fever, symmetrical arthritis, Raynaud's syndrome, skin lesions of the hands like a "mechanic's hand", an incomplete response to the use of glucocorticoids and frequent development of exacerbations against the background of a decrease in their doses, the debut of the disease mainly in the spring.

Diagnosis of juvenile dermatomyositis

The following criteria for the diagnosis of dermatomyositis have been developed ( Tanimoto et al., 1995).

Skin lesion.

1. Heliotrope rash - red-violet erythematous rash on the eyelids.

   Gottron's sign - red-violet scaly atrophic erythema or spots on the extensor surface of the hands over the metacarpophalangeal and proximal interphalangeal joints.

   Erythema on the extensor surface of the limbs, over the elbow and knee joints.

In this article, we raise a very important question - why do children develop such a rare, but very complex autoimmune disease as dermatomyositis, and how to treat it. With this disease, the child's smooth and skeletal muscles are affected, which leads to the fact that the baby loses the ability to move, the connective tissues are affected, and the skin becomes purple and swollen. The etiology of this disease was first described by Dr. E. Wagner in 1863. That is why dermatomyositis is also called "Wagner's disease". 30% of children, especially girls, suffer from this pathology. In our article, we will tell you everything about dermatomyositis with a photo, so that you know how to save your child from the consequences that the disease entails.

Dermatomyositis is a very complex disease, the causes of which are not fully understood, but doctors all unequivocally agree that a lot of factors can provoke the development of the disease.

All of them are conditionally divided into several groups:

1. The main group of causes is infectious diseases that develop in the child's body for more than 3 months. These include diseases caused by:

• picornaviruses
• parvoviruses
• influenza viruses

1. Pathogenetic group of causes - factors that provoke:

• bacteria (for example, group A streptococcus)
• vaccinations
• hormonal preparations

1. Trigger group of causes - factors predisposing to the onset of the disease. These include:

• hypothermia or overheating
• exposure
• severe psychological and physical trauma
• heredity
• drug allergy

Types of dermatomyositis in children

Dermatomyositis can be of 3 types:

1. Idiopathic dermatomyositis- It is also called primary. With it, except for external manifestations, no other symptoms of the disease are found. In humans, salt deposits appear on the skin, which acquire a red tint and are very itchy. Most often, this symptom occurs in young children and the elderly.
2. Paraneoplastic dermatomyositis - a secondary type of disease in which, in addition to a rash already purple in color, muscles begin to weaken. With this form of the disease, malignant tumors are very often formed not only on the skin, but also on the internal organs.
3. Juvenile dermatomyositis - this is a childhood form of the disease, which we will discuss in detail in our article. It is characterized by all the symptoms of the disease, which are also found in adults (most often the skin and muscles are affected). However, they appear with some features, which we will tell you about in more detail below.

Doctors also distinguish a fourth type of dermatomyositis. It is called polymyositis, because, in addition to the symptoms of this disease, signs of other diffuse pathologies also actively manifest themselves.

Degrees of manifestation of dermatomyositis

If your child has been diagnosed with dermatomyositis, you should know that this disease can develop with varying degrees of activity. In total, experts distinguish 3 degrees:

1. I degree- primary chronic, in which only the skin and muscles are affected. With her, the child:

• body temperature does not rise
• the skin acquires a purple hue in some areas (including on the eyelids)
• poorly flexed joints
• muscles weaken only if the child begins to strain them
• voice changes - it becomes nasal
• myocarditis, vascular problems may develop

1. II degree- subacute, in which all organs are affected within 7 months of the active development of the disease. With this form of dermatomyositis in a child:

• subfebrile body temperature
• damage to muscles and skin is more severe than with I degree
• child loses mobility
• all internal organs begin to become inflamed, especially the heart and blood vessels suffer

1. III degree- acute, in which the disease actively develops in just 1.5 months. With this form of dermatomyositis in a child:

• febrile body temperature (very high)
• the skin and muscles are very strongly deformed (all this is accompanied by severe pain)
• blood and urine tests are always bad
• internal organs are severely inflamed

Whatever degree of disease you are diagnosed with, it must be treated urgently. Properly selected treatment will help your child live a full life in the future.

Dermatomyositis: symptoms

Symptoms of dermatomyositis differ depending on the degree of activity of the development of the disease. However, there are several main signs by which doctors most often diagnose dermatomyositis:

1. The first manifestations of dermatomyositis are skin. As we have already mentioned, the eyelids, the area under the eyes, the places where the joints are unbent, become swollen, purple. At the same time, the skin is very flaky, because the dermis becomes dry.
2. On the elbows, knees, buttocks and shoulders, calcification occurs - the deposition of salts on the subcutaneous tissue.
3. Mucous membranes are affected. Most often the mouth, but in girls, the vaginal mucosa can also be affected.
4. All muscle groups are severely weakened. The child may stop walking, it is difficult for him to breathe and swallow. As a result, respiratory diseases also occur, as a symptom of dermatomyositis.
5. myocarditis develops. If the heart was already weak, then we can even talk about myocardial dystrophy.
6. The child's nervous system is also severely affected. As a result, encephalitis, meningoencephalitis develops, which are accompanied by convulsions and epileptic seizures.
7. The fundus of the eye changes and the optic nerve atrophies.
8. The organs of the digestive tract and kidneys are affected. Ulcers and, possibly, even tumors of a malignant nature form on these organs. All these ailments are accompanied by severe pain.

Dermatomyositis: diagnosis

If you have found any of the symptoms we have listed in your child, this is a reason to contact a rheumatologist who will prescribe you to undergo a series of laboratory and clinical studies, which include:

• general urine and blood tests (in them, the doctor, first of all, pays attention to the acceleration of ESR, and the presence of miglobin);
• blood chemistry;
• X-ray;
• Electromyography;
• Muscle biopsy (performed in the most advanced cases).

If the doctor considers it necessary, he will send you for examination to another specialist (this most often happens if the child has some kind of chronic disease that could cause dermatomyositis). If the diagnosis is confirmed, the doctor will give you recommendations on how to treat dermatomyositis.

Dermatomyositis: treatment

Depending on the severity of the disease, the doctor will explain to you where it will be more effective to treat dermatomyositis. But most often, a child with such a diagnosis is hospitalized, where drug therapy is carried out. It includes the use of several drugs to treat a child:

• Corticosteroids (any other than Triamcinolone and Dexamethasone are used because they weaken the muscles)
• "Prednisolone" with "Nerabol" and "Nerobolil" (especially these hormonal drugs are important if the child has II and III degrees of the disease)
• Immunosuppressants such as methotrexate, azathioprine
• "Delagila"
• Salicylates
• "Pyridoxal phosphate"
• "Cocarboxylases"
• Vitamin E, B and C

As soon as the child's condition improves after this drug therapy, he is prescribed physiotherapy, which includes muscle massage and exercise therapy. They are necessary in order to relieve pain. After the child is discharged from the hospital, it is advisable to send him to a sanatorium, where he will continue to undergo physiotherapy. In addition, teachers and psychologists will deal with the child.

If the child was diagnosed with I degree of dermatomyositis, then, most likely, after such treatment, he will have a stable recovery, if II or III, then the baby will have a period of remission, which must be maintained all the time with glucocorticoid drugs. In any case, you and your baby will have to go to the dispensary to come to see a doctor either once a month or once every 3 months (this factor depends on the stage at which the disease was diagnosed).

Dermatomyositis: prognosis and prevention

The prognosis of life in dermatomyositis is rather doubtful. Fortunately, medicine is constantly evolving, so the infant mortality rate due to this disease is only 1%. In most cases, today it is possible to completely restore the child's muscle strength, but for this it is necessary to take corticosteroids constantly, from which the organs of the digestive tract and nervous system will suffer greatly.

As such, there are no special preventive measures that could 100% prevent the development of dermatomyositis in your child. You just need to follow the general rules of life in order to reduce the risk of developing an illness. What is meant:

• make sure that the child leads a proper healthy lifestyle;
• do not allow his internal organs and muscles to be overloaded (for this, especially carefully monitor the child's diet and the amount of medications that he uses if he suddenly becomes ill with something);
• make sure that the baby's psyche is never injured, depressive disorders are a direct path to dermatomyositis;
• all the time reinforce the immunity of the baby, giving him vitamins, hardening him, playing sports with him.

Treatment of dermatomyositis in a child is a complex process. It requires strength, patience and financial costs from parents. But the main thing is not this, but the belief that your baby will recover and will be able to live a full life. We wish all our readers that your children are healthy, beautiful and happy! Take care of their health, take care and love with all your heart and soul - this is the main cure for any disease.

Video: "Dermatomyositis in children"

Dermatomyositis (DM)- a systemic progressive disease with a predominant lesion of striated and smooth muscles with impaired motor function, as well as skin in the form of erythema and edema. In 25-30% of patients, there is no skin syndrome; in this case, the term "polymyositis" (PM) is used. Some authors use the latter to refer to the disease as a whole. Less commonly used is the term "dermatopolymyositis" or the name of the disease by the names of the authors who described it - Wagner's disease, Wagner-Unferricht-Hepp disease. According to the modern international classification, DM belongs to the group of systemic connective tissue diseases.

For the first time DM (acute PM) was described by E. Wagner in 1863, somewhat later by R. Nerr and N. Unverricht (1887). By the beginning of the XX century. already identified various forms of the disease. Subsequently, numerous observations of clinicians and morphologists showed the possibility of a variety of visceral pathologies in DM, as well as the presence of systemic vasculitis and a peculiar lesion of the connective tissue, which made it possible to attribute DM to the group of collagen diseases. Based on the severity of the course and high (more than 50%) mortality in DM, E. M. Tareev included it in the group of so-called malignant or large collagenoses, later transformed into a group of diffuse connective tissue diseases. A fairly clear idea of ​​the disease, its clinical and morphological characteristics has been formed, although the issues of etiology and pathogenesis remain insufficiently studied. Currently, there are relatively large statistics and long-term personal observations of tens and hundreds of patients with DM, the analysis of which allows us to identify general patterns of development and the main clinical forms of the disease. The possible genetic heterogeneity of variants or subtypes of DM, which is designated by some authors as the DM-PM complex, is discussed. In addition to DM and PM, there are also quite frequent combinations of the disease with malignant tumors (paraneoplastic DM-PM), with other connective tissue diseases, and a special variant of juvenile dermatomyositis, which is reflected in the classifications.

There is no generally accepted classification of DM, although a number of groupings and classifications of the disease have been proposed. Among them, the most famous and relatively widely used is the classification of A. Bohan and Y. Peter.

Classification of dermatomyositis (polymyositis) according to A. Bohan and Y. Peter:

• Primary (idiopathic) polymyositis
• Primary (idiopathic) dermatomyositis
• Dermatomyositis (or polymyositis) in combination with neoplasm
• Children's dermatomyositis (or polymyositis) in combination with vasculitis
• Polymyositis or dermatomyositis in combination with other connective tissue diseases

There is an opinion about the increase in the frequency of DM (PM) in recent decades, which is associated with an increasing number of new allergens, with an increase in the frequency of neoplasms, etc., but there are no clear statistics in this regard. The topic should also take into account the improvement in the diagnosis of this group of diseases in recent years.

DM (PM) affects women more often; the sex ratio among adult patients (women and men), according to most authors, is 2:1 or more.

DM can develop at any age. Separate observations of PM in children under 1 year old are described. In these cases, it is very important, although sometimes it presents great difficulties, differential diagnosis with congenital myopathies.

There are two age peaks of DM, one of which (at the age of 10-14 years) reflects the juvenile form, and the second (at the age of 45-64 years) corresponds to an increase in the secondary (paraneoplastic) form of the disease.

Childhood (juvenile) DM (PM) is from 1/5 to 1/3 of the total number of cases of DM, idiopathic - 30-40% of cases and the next approximately 1/3 falls on the group of combined and secondary (paraneoplastic) forms of the disease, and the proportion of the latter increases in the older age group.

The predominant age of patients with idiopathic DM is from 30 to 60 years. In a review of 380 published cases of DM (PM), 17% of patients were under 15 years of age, 14% were between 15 and 30 years of age, 60% were between 30 and 60 years of age, and only 9% were over 60 years of age.

The predominance of women and an increase in the incidence during adolescence (juvenile DM), similar to that observed in RA and SLE, suggests the presence of common sex hormone-related factors in the development of these diseases.

What Causes Dermatomyositis?

The etiology of the disease is not well understood. The role of infection (viral, toxoplasmosis), genetic factors, and the immune theory of DM (TM) are discussed. As you know, in a number of viral diseases (influenza, rubella, etc.), a direct or indirect effect of viruses on muscle tissue is assumed, which manifests itself clinically (often myalgia) and morphologically. With DM, we are talking about the possibility of long-term persistence of the pathogen. There is no direct evidence of the viral etiology of DM, but indirect argumentation is quite extensive. One can think of at least three possible routes of exposure to the virus:

• direct damage to muscle tissue,
• through an immune response to viral antigens expressed on the surface of muscle fibers,
• antigenic mimicry, which causes the presence of cross-antibodies (autoantibodies) with the subsequent formation of immune complexes, etc.

The most common view is that a chronic viral infection persists in the muscles and causes a secondary immune response with the development of the PM pattern. The argument in favor of this hypothesis is the electron microscopic detection of virus-like particles (myxovirus-like and picornavirus-like) in the muscles (in the nuclei and cytoplasm) of DM patients. However, such particles are sometimes detected in the study of normal muscles and in other diseases, and most importantly, their detection may not have an etiological significance in DM (PM). Another proof is the discovery and experimental study of viruses with myotoxic properties. However, in patients with DM (PM), such viruses have not been identified, with the exception of individual observations, for example, isolation from the faeces of the Coxsackie A2 virus in a 14-year-old boy with chronic DM, echovirus in two brothers with acute PM. The virus was not isolated from the muscles of adult patients with DM, although the virus was isolated from individual newborns with myopathies and virus-like particles were found by electron microscopy.

An increase in the titer of antibodies to the Coxsackie B virus was noted in a controlled study in childhood DM, which is also regarded as an indirect argument in favor of the etiological role of a viral infection.

Currently, the model of PM in mice caused by the Coxsackie virus is successfully used in experimental studies. The tropism of the Coxsackie B virus to muscle tissue has been proven. With regard to individual picornaviruses in an experiment on mice, a connection between myositis and its characteristic Jo-1 antigen was shown.

A number of works also discuss the possible etiological role of toxoplasmosis, in particular, complement-fixing antibodies to Toxoplasma gondii are found much more often in patients with PM than in controls. Moreover, patients with high titers usually have a short duration of the disease (up to 2 years) and often specific antitoxoplasmic IgM antibodies, the level of which decreases during treatment with corticosteroids. However, the question remains whether it is contamination with toxoplasma infection, which stimulates the development of PM, or its direct participation in the pathogenesis of the disease. Summarizing the data on the infectious factor, one cannot exclude its possible adjuvant role, along with the above-mentioned ways of the possible participation of the virus in the development of immune responses and the pathological process as a whole.

Genetic factors undoubtedly play a role in the development of DM, similar to their participation in the genesis of other systemic connective tissue diseases, i.e., within the framework of the multifactorial theory of inheritance. This provides for the presence of a predisposition to the disease, which is realized only in combination with various exogenous and endogenous factors (environmental, infectious, immune, endocrine, etc.). For DM, such disease initiating factors can be, for example, Coxsackie 2 and other groups of viruses in interaction with the immune (autoimmune) shifts caused by them or preexisting.

Although the molecular basis of disease predetermination has not been established, there is a number of circumstantial evidence in favor of the involvement of genetic factors in its development. This is the presence, although infrequent, family cases of DM, including in twins, the detection of other rheumatic diseases in relatives of patients with DM (in every seventh family, various allergic and autoimmune syndromes, laboratory changes - an increase in the level of immunoglobulins, antinuclear antibodies, RF in families of patients with DM. Thus, E. M. Tareev observed a family where cases of acute DM, discoid lupus erythematosus and constitutional hypergammaglobulinemia were combined, and A. P. Solovyov observed two sisters, one of whom had DM, the other had RA. observed a combination of DM and scleroderma in two families.During the examination of 45 close relatives of 33 patients with DM, 13 other autoimmune diseases were detected, and in these families and in patients, the average level of serum IgG was reduced, and the C3 component of complement was increased.However, there is an observation of a family couples where the wife had severe DM with a fatal outcome 5 years after the onset of the disease, and the husband had myalgia, muscle hardening and increased serum oral creatine phosphokinase, which again returns to the hypothesis of the involvement of an infectious factor in the development of the disease.

There are few special immunogenetic studies with the study of the relationship of histocompatibility antigens (HLA) with DM or they have been carried out on a small material. Nevertheless, the identified association of DM (PM) with B8, B14, and DR3 antigens in the European population and the association with B7 and DRW-6 in blacks should be noted. Somewhat later, F. C. Arnett et al. noted an association between anti Jo-1 (DM-specific antibodies) and HLA-DR3. All anti Jo-1 positive DM patients were also DR3- or DRW-6 positive. A negative relationship with the HLA-DRW-4 antigen, which is characteristic of patients with seropositive RA, was noted. Association with the B8 antigen is well known for various immune (autoimmune) conditions and confirms the involvement of immune factors in the development of DM. Perhaps it is the presence of certain haplotypes that explains the characteristics of the clinical forms of DM, combinations with other connective tissue diseases (for example, more frequent with scleroderma and rare with RA), the severity of the immune component, etc. The association with HLA-B8 and DR3 is most pronounced in juvenile DM is currently considered as a genetic marker of diseases.

Pathogenesis (what happens?) during Dermatomyositis

The immune theory of the pathogenesis of DM is the leading one and is closely intertwined with the genetic and viral (infectious) ones, it is proved by pronounced disorders of cellular and humoral immunity, which are actively involved in the development of the pathological process. With DM, a wide range of antinuclear antibodies, circulating and fixed immune complexes, an increase in the level of serum immunoglobulins, an imbalance in the populations of T and B blood lymphocytes, a cytotoxic effect of lymphocytes on muscle tissue, etc. are detected. A high frequency of combinations with tumors, where DM usually acts as the second disease, with other autoimmune diseases and syndromes, including Hashimoto's thyroiditis, Segren's syndrome, etc., the development of "secondary" DM (PM) in trichinosis after revaccination, the provocative role of photosensitization and drug hypersensitivity confirm the involvement of immune mechanisms in the pathogenesis of the disease.

There is no doubt an important role of cellular immunity in the development of DM (PM), which is argued by the following data:

• lymphoid infiltrates in muscles consist predominantly of immune lymphocytes of the T-helper phenotype;
• upon exposure to the muscle antigen, the lymphocytes of patients with DM (PM) are transformed and increase the production of macrophage-inhibiting factor (MYF);
• lymphocytes in DM (PM) reveal a high cytotoxic effect on muscle cells compared to control lymphocytes;
• they release lymphotoxin, which can impair muscle metabolism, and a specific factor that inhibits calcium ions associated with the sarcoplasmic reticulum and muscle contractility;
• lymphocytes of animals with experimental DM have a cytotoxic effect on skeletal muscles.

It should be emphasized that not all of these reactions are specific to DM; they can also be observed in viral myositis and some myopathies, which, however, does not exclude their pathogenetic significance. Recent studies have shown that peripheral mononuclear cells in DM have a damaging effect on skin fibroblasts in tissue culture. This suggests the involvement of cellular reactions in the connective tissue damage in DM and the pathogenesis of the disease.

Changes in the immune response in DM are indicated by the presence of antinuclear antibodies (determined by immunofluorescence), precipitating antinuclear antibodies, antimuscle, antimyosin, antimyoglobin and anticytoskeletal antibodies, circulating and fixed in the vessels of immune complexes. In recent years, interest in these studies has increased, more detailed characteristics of the isolated antibodies have appeared, however, even now their pathogenetic role, the ability of antibodies to mediate the autoimmune process, remain unproven. Some of the above antibodies are also detected in the blood serum of patients with other muscle diseases, which allows us to consider them rather as a consequence, rather than the cause of muscle damage.

Previously, it was believed that the formation of antinuclear antibodies is not typical for DM, at least in comparison with SLE, in which their presence is regarded as a diagnostic sign of the disease. Currently, using more sensitive substrates such as HEp-2 cells, antinuclear antibodies are detected with a high frequency in both SJS and DM. In particular, the immunofluorescent method makes it possible to detect the presence of antinuclear antibodies with the use of HEp-2 in SLE and SJS in approximately 100%, and in DM (PM) in 78%. Antibody heterogeneity was found. The most specific antibodies, according to recent studies, are antibodies to PM-1, Ku-, Jo-1 and Mi-2 antigens. . .

PM-1 antibodies, giving nuclear and nucleolar fluorescence, were found in 60% of patients with DM, more often with a combination of DM and SJS. With further purification of the PM-1 antigen, the frequency of its detection in DM decreased to 9-12%; in patients with RA and SLE, this antigen was not detected, but it was found in 2 out of 32 patients (6%) with SJS. M. Reichlin et al. confirmed the typicality of PM-1 antibodies in overlap syndrome (DM-SSD) and their relative rarity in DM. It is proposed to refer to this phenomenon as "DM-SSD-antibodies". In a study of 77 patients with DM in combination with SJS, RNP antibodies (29%), SSA antibodies (14%), SSB antibodies (5%), Scl-70 antibodies (10%), DNA antibodies (6 %) and Sm-antibodies (10%), however, the association of PM-1 antibodies with other antibodies was rarely observed. In patients with the presence of Sm-antibodies, signs of SLE were also observed. Thus, the presence of PM-1 antibodies confirms the existence and characterizes the immunological features of the cross form of DM with scleroderma, which we can also identify on the basis of clinical data.

Anti-Ku antibodies are also observed mainly in patients with signs of DM (PM) and SJS: therefore, they are often found in association with anti-RM-1 antibodies. However, the Ku system is differentiated from PM-1 antibodies by immunodiffusion and other physical and chemical properties.

Anti-Jo-l antibodies directed to a nuclear soluble antigen are considered specific for DM. M. C. Hochberg et al. found anti-Jo-l in 23% of patients with DM (PM) and in none of the cases of SLE and SJS. Most often, these antibodies are detected in PM (in 47%), including overlap syndrome. Jo-1 antibodies are directed to histidyl RNA transfer synthetase and may therefore pose an immune response to viral agents associated with this enzyme. It was noted that in anti-Jo-l-positive patients, interstitial lung damage is more common and there is an association with DR-3 and DRW-6 antigens characteristic of adult DM.

We can talk about the presence of a subgroup of patients with DM (PM), HLA-, DR3- and Jo-1-positive, who often have interstitial lung disease. Mi-2 antibodies represent the first type of precipitating antibody described as specific for DM. They occur in approximately 25% of patients with DM (less often in the absence of skin changes); in other diseases of the connective tissue were not detected.

Thus, anti-Mi2 are more typical for DM, and anti-Jo-1, on the contrary, for PM, while anti-PM-1 is characterized mainly by the combination or intersection of DM (PM) with SJS.

Immune complexes were found in the vascular wall in children with DM with vasculitis, which suggests their pathogenetic significance. At the same time, circulating immune complexes (CIC) are one of the characteristic laboratory tests of the activity of the pathological process, they correlate with other indicators of activity and the presence of immune disorders. A retrospective analysis showed that CEC-positive DM (PM) patients needed higher doses of prednisolone (on average 2 times) than CEC-negative ones. This indicates the diagnostic (in determining activity) and, to some extent, the prognostic significance of the CEC in DM (PM). The level of the CEC can also be used to monitor the effectiveness of treatment: with the use of adequate doses of corticosteroids, it decreases in most patients.

In a comparative study of the CIC in two groups: the first with idiopathic DM (PM) and the second with DM in combination with other diffuse connective tissue diseases, it was found that in the second group the percentage of detection of the CIC and binding to Clq is somewhat higher than in the first. In both groups, an increase in the CIC correlated with higher laboratory indicators of process activity, but in the second group, positive autoimmune tests were more often detected: LE cells in 10% of patients in the first group and in 38% in the second, antinuclear factor in 40 and 69%, RF - in 40 and 85%, respectively.

The pathogenetic role of the CEC is discussed in connection with their interaction with the Fc receptors of lymphocytes, which causes an increase in the biosynthesis of immunoglobulins (and again a subsequent increase in the CEC, i.e. a vicious circle), and the release of lymphokines involved in the development of inflammation and muscle damage.

The deposition of immune complexes in tissues (muscles, skin, blood vessels, etc.) leads to the development of immune complex inflammation.

All this testifies to the undoubted participation and leading role of immune disorders in the local and general pathogenesis of DM (PM).

In about half of the patients, the onset of the disease was preceded by insolation, cooling, emotional stress, vaccination, administration of tetanus toxoid, sensitization with epoxy resins, photosolvents, drugs (penicillin, sulfonamides, chlorpromazine, insulin, vitamins B1, B6, B12), etc. Such a relationship with previous , predisposing or provoking disease factors are detected more often in acute onset of DM.

Symptoms of Dermatomyositis

The onset of the disease can be acute, but more often the symptoms develop gradually, characterized mainly by skin and muscle manifestations: edema and hyperemia in the periorbital region, on exposed parts of the body, myalgia, increasing muscle weakness, sometimes arthralgia, low-grade fever. With an acute onset - fever up to 38-39 ° C, a sharp deterioration in the condition, more generalized and bright erythema on the face, trunk, limbs, rapidly increasing muscle weakness, up to immobility in the first month of the disease. There are also observations of chronic DM, when skin symptoms long precede muscle damage, which develops gradually and is usually not as pronounced as in its acute and subacute forms. With PM, there is no skin lesion, but already from the onset of the disease, characteristic muscular symptoms develop acutely or gradually. A very slow development of muscle weakness (within 5-10 years) is also possible as a reflection of the picture of chronic PM, which is sometimes difficult to differentiate from progressive muscular dystrophy. At the onset of the disease with Raynaud's syndrome or joint stiffness, sometimes preceding febrile conditions, which are later joined by a characteristic picture of PM, it is usually a combination of PM with other connective tissue diseases, more often SJS (overlap syndrome).

Clinical signs

• Increase in body temperature
• Skin lesion:
  • • erythema
    • periorbital edema
    • capillaritis
• Raynaud's syndrome
• Generalized skeletal muscle disease:
  • • weakness
    • myalgia
    • contractures
    • calcification
• Dysphagia
• Mucosal damage
• Arthritis/arthralgia
• Heart damage:
  • • myocardium
    • endocardium
    • pericardium
• Interstitial pneumonia, pulmonary fibrosis
• Adhesive pleurisy
• Nephritis
• Hepatomegaly (fatty degeneration)

The detailed picture of the disease is characterized by polysystemic and polysyndromic with prevailing lesions of the skin and muscles, which leads to a peculiar appearance of patients with DM and increasing immobility. Often the mucous membranes are involved in the process; joint damage, as well as visceral pathology, is usually mild and not as frequent as, for example, in SLE and SJS.

Skin lesion with DM it is polymorphic: erythema, edema and dermatitis predominate, mainly on open parts of the body; papular, bullous, sometimes with ulcerations, petechial rashes, telangiectasias, foci of pigmentation and depigmentation, hyperkeratosis, etc. are observed. Periorbital edema and erythema are characteristic, having a kind of lilac "heliotropic" hue (a symptom of "glasses"), which plays an important diagnostic and differential diagnostic role in DM. Bright erythema is more often localized on the face, neck, décolleté, over the joints, especially over the proximal interphalangeal and metacarpophalangeal (Gottron's syndrome), on the outer surface of the forearm and shoulder, the anterior surface of the thighs and lower legs. Such skin changes, especially with capillaritis, resemble skin lesions in SLE, but are more resistant, bluish in color, and may be accompanied by peeling and itching. Sometimes dermatitis has a squamous character and resembles seborrhea or psoriasis. Swelling of the face and extremities, predominantly over the affected muscles, has a doughy or dense character, sometimes resembling a skin lesion in scleroderma. Trophic disorders are often observed in the form of dry skin, longitudinal striation and brittleness of nails, hair loss, etc. Nail folds can be hyperemic due to dilatation of capillaries and a sludge phenomenon detected by capillaroscopy. Sometimes a variety of chronic DM is distinguished - poikilodermatomyositis, which is characterized by skin lesions of the type of poikiloderma, when there are foci of pigmentation and depigmentation, multiple telangiectasias, thinning of the skin, dryness, areas of hyperkeratosis. Less often, poikiloderma develops as an outcome of erythematous, bullous, petechial and other rashes, more characteristic of an acute and subacute course, indicating a kind of chronic process that occurred spontaneously or under the influence of ongoing therapy.

Approximately half of the patients have conjunctivitis, stomatitis, sometimes accompanied by increased salivation, hyperemia, swelling of the pharynx, and true vocal cords. The skin syndrome may precede the appearance of other signs of DM, including muscle damage, but in patients with PM, skin changes are practically absent. Rarely, skin changes for a number of years are practically the only sign of the disease.

Thus, although the changes in the skin and mucous membranes are different, the characteristic signs and the predominant localization of the process often make it possible to suspect DM already at the first glance at the patient.

Skeletal muscle damage is the leading symptom of DM. Characterized by the development of severe, often necrotic myositis with a predominant lesion of the muscles of the proximal limbs, shoulder and pelvic girdle, neck, back, pharynx, upper esophagus, sphincters.

Clinically, pain in the muscles, density or test character of the affected muscles, their increase in volume, pain on palpation are noted. The dominant sign of PM (DM) is steadily progressive muscle weakness, which is expressed in a significant limitation of the active movements of patients who cannot stand up, sit down, raise their legs on a step (the "bus" symptom), hold any object in their hand, comb their hair, get dressed (symptom of "shirt"), easily fall when walking. With damage to the muscles of the neck and back, patients cannot raise their heads from the pillow or hold them while sitting (the head falls on the chest), they cannot sit up and get out of bed on their own. All movements associated with the participation of the proximal muscles of the limbs (shoulder and pelvic girdle) are practically difficult ), while in the distal limbs (in the hands and feet), satisfactory strength and full range of motion are maintained.

The gradual involvement of the muscles of the neck and back in the process exacerbates the severity of the condition of patients who, due to increasing disability and immobility, require constant care.

Involvement of the pharyngeal muscles in the process causes dysphagia (choking when swallowing), aspiration of food into the trachea is possible. In contrast to the dysphagia seen in SJS, DM patients have difficulty swallowing both solid and liquid food, which sometimes spills out through the nose. The upper parts of the esophagus, the muscles of the soft palate, and the tongue are predominantly affected; developing pseudobulbar symptoms mimic a neurological disease.

Damage to the intercostal muscles and diaphragm leading to limited mobility and reduced lung capacity, contributes to the development of pneumonic complications - one of the main causes of death in DM.

When the muscles of the larynx are affected, a nasal tone of voice (dysphonia), hoarseness, up to aphonia appear. The defeat of the muscles of the sphincters leads to a breakdown in their activity. The severity of the condition and disability of patients with DM are also due to the frequent subsequent development of tendon-muscle contractures, atrophy and calcification of previously affected muscle groups.

The rate of development of symptoms depends mainly on the nature of the course of the disease. In acute cases, severe muscle weakness may appear within the first 2–3 weeks, often associated with myoglobinuria. More often, the symptoms of PM develop gradually - within 3-6 months (subacute course). Muscle weakness may increase over a number of years when it comes to chronic DM (PM). At the same time, the characteristic localization of the process is preserved - the proximal parts of the muscles of the limbs.

Facial muscles are affected extremely rarely, the involvement of the eye muscles in the process is practically not observed with PM. However, G. Serratrice and A. Schiano also refer to the DM (PM) variant as a regional form - segmental polymyositis with damage to certain muscle groups (shoulder, shoulder blade, femur) of a sclerosing or inflammatory nature. .Here they also include orbital myositis, in which ptosis, diplopia, and a number of other local myositis are observed, which, in our opinion, is redundant. the degree of "muscular consumption" (myophthisis) in severe cases of the disease.In 6 of 39 patients, along with signs of DM, D. Beaurain et al. observed scapular retractile capsulitis, 4 of these 6 patients also had signs of scleroderma and all 6 - a positive antinuclear factor (overlap syndrome), so it could be assumed that retractile capsulitis of the shoulder was associated with both PM and scleroderma.

It is quite clear that the nature of muscle damage, the severity and localization of pathology vary both within the studied group of patients and in the picture of an individual patient. It depends largely on the duration of the disease, the degree of evolution of the pathological process and the nature of the course of the disease, the therapy, etc.

Morphological changes in biopsied muscles presented below, as well as electromyography data, studies of muscle enzymes vary depending on the severity, severity and activity of myositis, however, they have features characteristic of this disease that allow verification of the diagnosis.

Calcinosis also serves as one of the characteristic, although rather secondary, signs of DM and has a dystrophic or metabolic ("reparative") character. Affected tissues are calcified, in which inflammatory and even necrotic changes were noted earlier: calcium metabolism is not disturbed (the level of calcium and phosphorus in the blood remains normal).

Calcinosis more common in DM in children, but can also complicate the course of DM in adults, especially in the absence of adequate and timely corticosteroid therapy. In juvenile DM, it develops approximately 16 months after the onset of the disease. Usually areas of fascia, subcutaneous tissue adjacent to the affected muscles, i.e., mainly in the area of ​​the shoulder and pelvic girdle, are usually calcified, but there may also be calcifications in the area of ​​the elbow and other joints. Massive areas of calcification, sometimes sharply painful, or their spread to the periarticular tissues lead to immobility and disability of patients. When located subcutaneously, calcifications are partially rejected in the form of crumbly masses, leading to ulceration and sometimes suppuration. Quite typical clinically, they are confirmed radiographically, which is especially important for detection when they are located deep. We observed 4 young patients with extensive calcifications in the pelvic and shoulder girdle, who were practically immobilized and suffered from pain. Therefore, we cannot agree with the authors who regard calcification as a good prognostic sign, although in adult patients it really indicates the transition of an acute course to subacute and even chronic, spontaneously or during therapy. Of course, tissue calcification is not so pronounced in all patients with DM (PM); it can develop gradually and be practically painless or be felt only in a certain position, for example, sitting - with relatively small calcifications in the gluteal areas.

Calcification, taking into account the nature and localization, has a certain diagnostic and differential diagnostic value.

Treatment of patients with calcification is difficult and usually ineffective. Attempts to surgically remove individual calcifications do not solve the problem as a whole. Therapeutic agents (corticosteroids, diphosphonates, MagEDTA infusions, etc.) also do not give tangible results. With small superficial calcifications, local use of DMSO with Trilon B gives some effect. In single patients, a partial improvement was noted from the use of probenecid and colchicine. In rare cases, spontaneous resorption of calcifications is observed. Articular syndrome is less typical for DM (PM), usually expressed as arthralgia or damage to periarticular tissues, arthritis is rare. Dysfunction of the joints and contractures are more often associated with muscle damage. X-ray examination sometimes reveals moderate osteoporosis of the bones. In juvenile DM (TM), joint damage, including in the form of arthritis, is more pronounced. In adults, articular syndrome was noted by A.P. Solovieva in 27.7%: pain during movement, especially in large joints of the limbs, dysfunction - limitation of both active and passive movements, rigidity and sometimes swelling in the joints. Elbow, shoulder, knee joints and hands are most commonly affected. Joint damage is observed in 1/3-1/2 patients with DM (PM), more often in combination with other connective tissue diseases (overlap syndrome). The intensity of pain is moderate; they occur more often at night and continue in the morning; always recede into the background compared to muscle symptoms. They usually appear at the onset of the disease and are quickly stopped (both arthralgia and arthritis) with corticosteroids, which should also be taken into account in the diagnosis and differential diagnosis of DM (PM).

Raynaud's syndrome can also be observed in DM, but it is not as characteristic and frequent as in SJS. It is noted in approximately 1/4-1/3 patients with DM (PM), more often in children, in whom it is included in the picture of vasculitis characteristic of this form. Typical for combined forms of DM with scleroderma. In idiopathic DM, it often has a two-phase character with a predominance of acroasphyxia, usually often pronounced and does not lead to trophic ulcers and necrosis of the fingers, with the exception of cross-forms with SJS, in which it characterizes the latter and may be the first sign of the disease.

Capillaroscopy reveals microcirculation disorders, combined with both Raynaud's syndrome and vasculitis: expansion of capillary loops, slowing of blood flow and sludge syndrome, avascular fields, although the latter are more characteristic of SJS. These changes are more often found in DM than in PM. They do not have a clear correlation with the severity and activity of myositis, although they decrease with prolonged remission; more often found in patients with Raynaud's syndrome, lesions of the skin, joints and lungs, with overlap syndrome.

Damage to internal organs usually moderately pronounced, occurs in most patients with DM, but does not prevail in the picture of the disease, as, for example, in SJS and SLE. Part of the visible visceritis is due to or aggravated by the muscular pathology inherent in the disease. This applies primarily to damage to the respiratory and digestive tract. Other visceral manifestations of DM (PM) are due to the development of a pathological process in the interstitial tissue and organ vessels, which confirms the interest of the connective tissue and the systemic nature of the process characteristic of this group of diseases. Myocardial damage of an inflammatory and dystrophic nature, the development of interstitial pneumonia or diffuse interstitial fibrosis of the digestive tract (dysphagia, vasculitis, damage to the sphincters) are more often observed, and the kidneys are less often affected.

Damage to the heart, especially myocardium, is often observed in patients with DM, and in systemic cases can be the cause of death. It is characterized by diffuse or focal changes in the heart muscle (during functional and morphological examination), conduction disorders, arrhythmias, and rarely, heart failure. According to different authors, clinical and (or) electrocardiographic anomalies are found in 30-50% of patients with DM (PM). ECG changes in children with DM often indicate a poor prognosis.

Clinical, functional and morphological comparisons showed the relative scarcity of clinical symptoms and the important role of instrumental methods in the detection of pathology. Heart damage develops more often during the active period of DM (PM) and is expressed by tachycardia, moderate expansion of the boundaries of the heart, muffled tones, more often in the apex region, arrhythmias, and hypotension. These signs indicate the predominant damage to the myocardium, which is confirmed by special studies.

Infection of the endocardium and pericardium are rarely detected, however, with the use of echocardiography and other instrumental methods of investigation, the number of observations of DM (PM) with involvement of the pericardium and endocardium in the pathological process, including individual cases of heart disease, mitral valve prolapse, has increased.

New non-invasive methods for examining the heart made it possible to confirm the frequency and different nature of its damage in DM (PM). So, when using echocardiography, daily monitoring, perfusion scintigraphy with 201Tl and the study of central hemodynamics, A. Askari revealed changes in the heart in all examined patients, at the same time they also had a high level of cardiac fraction of creatine phosphokinase.

In an electrocardiographic study, rhythm and conduction disturbances are most characteristic - blockades of various degrees, changes in the T wave and displacement of the ST segment. A. Askari revealed ventricular extrasystoles, atrial fibrillation, bigeminia, which were sometimes observed at different times in the same patient, often associated with intraventricular conduction disturbances - blockade of the left or right leg of the atrioventricular bundle, etc. Rhythm disturbances such as atrial and supraventricular paroxysmal tachycardia, bigeminy, were not detected by conventional electrocardiographic examination, but were detected by 24-hour Holter monitoring. Sometimes changes in the terminal part of the ventricular complex on the ECG were similar, as well as changes in the study with 201Tl, to those observed in infarction, but angiography and postmortem examination showed no coronary occlusion, which, however, does not exclude the interest of the microvasculature in the genesis of pathology.

Pathological anatomical examination and biopsy revealed changes in the myocardium that were largely similar to those found in skeletal muscles. This is mononuclear infiltration, sometimes necrosis and atrophy of muscle fibers. Fibrosis is also observed, not associated with coronary occlusion, similar to how it is characteristic of SJS, but less pronounced. The genesis of these changes in DM (PM) is explained by the presence of myocarditis, but it is possible, at least in part, due to ischemic changes due to damage to small vessels, similar to the role of microcirculation disorders in SJS. The term "polymyositis cardiopathy" is sometimes used to refer to this pathology.

In the process of observation, there is a dynamics cardiac pathology, including a decrease in the symptoms of carditis during treatment with corticosteroids in a number of patients, which, apparently, proves a predominantly inflammatory nature in this group. sick. In other observations, where such an improvement was not noted, one could assume the predominance of dystrophic changes or myocardial fibrosis. Usually, the development of carditis correlates with active damage to the peripheral muscles, although it is usually in the background in terms of the timing and severity of the pathology, and against the background of adequate therapy, positive cardiac dynamics retains parallelism with the usually prevailing damage to the peripheral muscles. However, there is an observation of a relatively late acute myocarditis with severe ventricular arrhythmia, which ended in death, in a 65-year-old woman with typical PM and a positive response to treatment with corticosteroids and azathioprine, which has already been carried out for 7 weeks. An autopsy revealed myocarditis with a clear improvement in the condition of skeletal muscles. The authors draw attention to the lack of parallelism with peripheral muscle pathology.

A recently published observation also illustrates the possibility of developing constrictive pericarditis in a patient with DM. Previously, the great rarity of pericardial involvement in DM was emphasized, although isolated cases of acute pericarditis have been described. We also observed the development of constrictive pericarditis with heart failure in a 32-year-old patient, in whom severe manifestations of PM were combined with signs of SJS.

Thus, cardiac pathology is observed quite often in DM (PM) and can be the cause of death or increasing heart failure in individual patients with myocardial necrosis or combined damage to all three layers of the heart, which can be conditionally designated as pancarditis. It should also take into account the role of microcirculation disorders in the development of pathology, the frequent involvement of metabolic processes that may come to the fore in patients with intensive and prolonged corticosteroid therapy. Lung damage in patients with DM is due to a number of factors and includes the participation of muscle syndrome (hypoventilation), infectious agents, aspiration in swallowing disorders, along with proper pulmonary pathology such as interstitial pneumonia and fibrosing alveolitis. In some cases, drugs used to treat patients with PM (eg, methotrexate) can cause pulmonary fibrosis.

Muscle weakness extending to the respiratory muscles, including the diaphragm, may be the cause of a decrease in the ventilation function of the lungs, and therefore a control study of the vital capacity of the lungs in seriously ill DM (PM) over time is recommended.

According to N. M. paun et al., significant worsening of respiratory functions and the involvement of the respiratory muscles in the process was noted in 3/4 of the observed patients (53 people). In 16 out of 53 these changes were combined with lung damage, in 37 there was no actual pulmonary pathology and muscle weakness was combined with a decrease in total vital capacity and maximum pulmonary ventilation, an increase in residual volume and arterial CO2 content, more frequent atelectasis and pneumonia. The authors emphasize the importance of using vital capacity indicators, a decrease in which below 55% may be associated with hypercapnia and further complicate the course of the disease and muscle condition. Decreased function in DM (PM) concerns both inspiratory and expiratory muscles, which distinguishes this group of patients from those suffering from amyotrophic lateral sclerosis, muscular dystrophy, and myasthenia gravis. Clinically, more frequent and shallow breathing is noted, shortness of breath appears, which indicates the development of ventilation insufficiency. X-ray reveals a high location of the diaphragm, sometimes atelectasis. Deterioration of the function of the pharyngeal muscles leads to impaired swallowing - dysphagia, which, along with a decrease in the intensity of coughing and aspiration of liquid or food, causes the development of aspiration pneumonia, which, with hypoventilation and a severe general condition of patients, is difficult to treat and can lead to death.

Actually, lung damage often occurs in the form of moderate interstitial pneumonia or by the type of fibrosing alveolitis.

Pulmonary fibrosis observed in 5-10% of patients and is detected mainly by X-ray examination. Pulmonary functional tests indicate mainly a restrictive type of disorders with a decrease in total and vital lung capacity; hypoxemia is characterized by a moderate decrease in the diffusion capacity of the lungs. Shortness of breath and cough, wheezing and crepitus are observed with severe lung damage.

Morphological examination reveals alveolar-septal fibrosis, interstitial mononuclear infiltrates, consisting mainly of lymphocytes, a small number of large mononuclear and plasma cells, type I hyperplasia of the alveolar epithelium, an increase in the number of free alveolar macrophages. The affected tissue alternates with apparently unaltered areas. There are often also interstitial edema and vascular changes with thickening of the intima and media of the artery wall and arterioles. If inflammatory changes in the alveolar walls are detected using a lung biopsy (usually in an acute course), then the therapeutic effect is better, and the presence of fibrosis in the absence of inflammation is a poor prognostic sign. In some patients, despite treatment with corticosteroids, rapidly progressive fatal pulmonary insufficiency may develop. In cases where a patient with acute DM, immobilized, with hypoventilation of the lungs (sometimes requiring the connection of an artificial respiration apparatus), severe dysphagia and choking, the phenomena of severe pneumonia increase, usually we are talking about a mixed nature of pulmonary pathology: 1) damage to the interstitial tissue of the lungs and vasculitis , 2) damage to the respiratory muscles and 3) aspiration pneumonia.

It should be borne in mind the possibility of a tumor, often metastatic, process in the lungs.

Changes gastrointestinal tract are often noted and are manifested by increasing dysphagia, lack of appetite, sometimes - abdominal pain and gastroenterocolitis.

Dysphagia, of course, can only conditionally be attributed to the visceral signs of the disease. There is a decrease in the contractile strength of the pharyngeal muscles and muscles of the upper esophagus, impaired peristalsis, weakness of the muscles of the soft palate and tongue. This causes choking, a violation of swallowing solid and liquid food, which can pour out through the nose. The voice becomes nasal. Dysphonia is often combined with dysphagia and in seriously ill patients sometimes turns into aphonia.

In some patients, there is also dysfunction of the cricopharyngeal muscles with spasm, sometimes leading to constriction, fibrosis and requiring surgical intervention. With the involvement of the esophageal sphincter in the process, the development of reflux esophagitis is possible.

Pharyngeal-esophageal dysphagia is an important diagnostic, differential diagnostic sign of DM (PM). Unlike SJS, the upper esophagus and the pharyngeal ring are affected, so the clinical and radiological picture is different. In particular, with scleroderma, liquid food passes well, does not spill out through the nose, but at the same time, radiological signs of damage and complications of scleroderma esophagitis are often more pronounced. The prognostic significance of this localization of the process should also be kept in mind.

Severe progressive dysphagia, when solid food is regurgitated and liquid is poured out through the nose, due to the possibility of aspiration, poses an immediate threat to the life of the patient and is a direct indication for urgent therapy with maximum doses of corticosteroids.

Separate cases of DM with gastrointestinal bleeding, gastric perforation, which are based on vasculitis and necrosis along the digestive tract, are described.

A moderate increase in the liver with a change in functional tests is observed in approximately 1/3 of patients, less often - hepatolienal and glandular-splenic syndromes.

Renal involvement is relatively rare in DM (PM). In the acute course, severe persistent myoglobinuria can lead to the development of renal failure. Some patients observed diffuse glomerulonephritis, vascular pathology of the kidneys with fibrinoid changes in arterioles, thrombosis; glomerulitis. Clinically, 31 out of 130 patients with DM observed by A.P. Solovieva (1980) had transient proteinuria and only 3 had severe renal pathology. Among children with DM, 41.5% had transient proteinuria with microhematuria and cylindruria. When clarifying the causes of proteinuria, one should keep in mind the possible connection with the activity and severity of the disease, the influence of steroid and other therapy, tumor damage to the kidneys, infection, etc.

Damage to the nervous and endocrine systems is also rare. More often we are talking about pseudo-neurological symptoms, although some patients may develop mild polyneuritis and even CNS lesions due to vasculitis. Occasionally observed mental disorders, emotional instability of patients are more likely to be associated with taking high doses of corticosteroids. The most common are vegetative disorders. Changes in the endocrine sphere (decrease in the functions of the gonads, pituitary-adrenal syndrome, etc.) can be associated with both the severity of the disease and vasculitis, and ongoing steroid therapy.

• The course of the disease

The course of DM is undulating, progressive and highly variable. Allocate acute, subacute and chronic forms.

The acute course is characterized by fever, a catastrophically increasing generalized lesion of the striated muscles up to complete immobility, widespread erythematous rashes, progressive dysphagia, dysphonia, damage to the heart and other organs. Without treatment with corticosteroids, these patients usually died during the first year of the disease, and sometimes after 2 months from its onset. The cause of death in patients with acute DM was more often aspiration pneumonia, which easily arises and quickly spreads in conditions of hypoventilation of the lungs, pulmonary heart and kidney failure. An acute course is also characteristic of DM in children and adolescents with characteristic angiopathy underlying multiple infarcts, ischemia, and muscle atrophy. In the most acute cases of DM in children, inflammatory changes in the muscles are not pronounced, necrosis and vascular pathology predominate.

Due to the catastrophically rapid development of acute DM with immobility and swallowing disorders that mimic severe polyneuritis and pseudobulbar disorders, these patients are often hospitalized in neurological hospitals, with fever and hemorrhagic rashes on the skin - in infectious and much less often in dermatological and therapeutic ones. In the absence of timely diagnosis, emergency and adequate therapy (massive doses of corticosteroids), patients die from the main manifestations of the disease and complications.

Currently, with the help of corticosteroids, it is usually possible to stop the progression of the process and improve the condition of patients, up to clinical remission.

The subacute course is characterized by a slower increase in the symptoms of DM, but after 1-2 years from the onset of the disease, there is usually an already developed picture of DM (PM) with severe muscle damage, erythema or dermatitis and visceritis, tissue calcification is possible. In the subacute course, the disease often begins with gradually increasing muscle weakness, which is detected during exercise, less often with dermatitis. Later, a characteristic clinical picture of the disease develops with a predominant lesion of the muscles of the shoulder and pelvic girdle, dysphagia, dysphonia, and sometimes myocardium, lungs and kidneys.

The prognosis of this variant of the course of DM (PM) was also unfavorable in the precorticosteroid era. Most patients died or became disabled with multiple flexion contractures, widespread calcification, and sometimes complete immobilization.

Modern therapy causes the reverse development of symptoms, prevents the development of tissue calcification and allows achieving remission.

In a chronic course, the disease usually proceeds cyclically, for a long time; the processes of muscle atrophy and sclerosis predominate; their local defeat is possible, including the distal extremities. Often, patients with chronic DM develop dermatitis, itching, hyperpigmentation, hyperkeratosis. Visceral lesions are rare. The prognosis of this form of DM is favorable.

• Complications

The most frequent and formidable complication (ranks first among the causes of death in patients with DM) is aspiration of food masses in violation of swallowing with the development of severe aspiration pneumonia against the background of limited mobility of the chest due to damage to the intercostal muscles and diaphragm. Lung hypoventilation also creates the prerequisites for the development of pneumonia due to intercurrent infection. In some cases, severe damage to the respiratory muscles with a sharp restriction of chest excursion can lead to progressive respiratory failure and asphyxia, which requires the use of mechanical ventilation. Cardiac and especially renal failure in DM is relatively rare. Immobilized patients often develop ulcers, bedsores, which are easily infected; dystrophy, exhaustion are possible.

• Pediatric (juvenile) dermatomyositis

DM (PM) in childhood occurs with approximately the same frequency in boys and girls, according to some authors, it may even prevail in boys. The ratio of DM and PM is approximately 2:1. DM in children often develops at the age of 4-10 years and in 50% of cases has an acute onset.

A. Bohan and J. Peter singled out DM (PM) in children as a special form due to the severity and frequency of vasculitis in this group. The prognosis of DM in childhood is estimated differently. A. Roze and J. Walton consider it to be better than in adults with DM: among 19 patients under the age of 20 they observed, there were no deaths compared with 39% of mortality in adults. Clinical and laboratory manifestations are generally similar to the picture of DM (PM) in adults, however, there are some features associated with severe vasculitis and microangiopathies, often with a more acute onset and an exudative component (edema, synovitis, etc.), followed by the development of widespread tissue calcification.

The disease begins more often with fever, sharp pain in the muscles, hands and feet, increasing muscle and general weakness, progressive weight loss.

Skin lesions are noted in most patients in the form of a lilac hue of the face or characteristic heliotropic erythema in the periorbital areas, rashes in the forehead, eyelids, sometimes cheeks, neck, anterior and posterior chest, extremities. Often, edema of the skin, subcutaneous tissue, and periarticular tissues develops in parallel, sometimes imitating or actually combined with synovitis. In the area of ​​the nail bed, there are sometimes micronecrosis (vasculitis), telangiectasia; over the joints of the hand - Gottron's erythema (with a characteristic cyanotic whitish tint, atrophy and waxy peeling or brighter). In severe vasculitis, ulceration and necrosis of the skin, visceral organs (intestines, etc.) are possible.

Muscle damage is characterized by an increase in muscle weakness and immobility of patients, often with a more pronounced pain component, which is sometimes difficult to differentiate from polyarthritis. Emerging dysphagia and dysphonia no longer allow one to doubt the diagnosis of DM (PM), but sometimes suggest neurological symptoms. Growing damage to the respiratory muscles with development is especially unfavorable.

Diagnosis of Dermatomyositis

Despite the characteristic clinical presentation of the disease, diagnostics it, especially at the beginning, presents great difficulties. The diagnosis of DM (PM), as a rule, is preceded by erroneous diagnoses, with skin symptoms predominating "dermatological", and with muscular - "neurological" diagnoses. The most typical among them are dermatitis, allergic edema, erysipelas, neurodermatitis, erythroderma, infectious myositis, polyneuritis, poliomyelitis, pseudobulbar syndrome, myasthenia gravis, etc. Diagnoses of allergic and infectious diseases, other systemic connective tissue diseases, more often - SLE are also not uncommon.

Among the patients with DM(HGM) observed by us, almost all of them went through the "phase" of erroneous diagnoses. A.P. Solovieva presented an analysis of misdiagnosis in 100 patients with idiopathic and 30 patients with tumor DM. Erroneous diagnosis occurred in almost all patients, and the correct diagnosis in a number of them was preceded by 3-4 or more erroneous ones. Particularly tragic is the fact that patients with late diagnosis of DM (PM), especially children, become lifelong invalids (persistent contractures, generalized calcification), and in the acute course of the disease, both children and adults can die, despite the relatively effective modern therapy for condition of early and adequate treatment.

We have to admit that even with the classical picture of the disease, doctors of various profiles (therapists, dermatologists, neuropathologists, etc.), to whom patients turn, incorrectly evaluate the symptoms and the disease as a whole, obviously due to insufficient knowledge in this area. At the same time, there are cases of DM (PM) that are really difficult to diagnose, atypically occurring or combined with other diseases, when not only knowledge is needed, but also experience, monitoring the course of the disease.

In recent years, a reverse trend has been revealed towards overdiagnosis of DM (PM) in the presence of other diseases of the rheumatic group, muscle damage of a different nature, various endocrine and neuropathies. This trend is also unfavorable and sometimes dangerous for the patient due to the unjustified prescription of high doses of corticosteroids, resulting steroid dependence and complications. A.P. Solovieva identifies 4 main groups of patients with the most frequent overdiagnosis of DM (PM):

• rheumatic and related diseases (rheumatism, systemic lupus erythematosus, SJS, periarteritis nodosa, RA, hemorrhagic vasculitis, Loeffler's fibroplastic endocarditis, recurrent urticaria, erythema nodosum, panniculitis, Beck's sarcoidosis);
• endocrine diseases (myxedema, thyrotoxicosis, diabetes mellitus with diabetic polyneuritis, obesity, etc.);
• various muscular and neuromuscular diseases (myasthenia gravis), myotonia, various polymyositis, polyfibromyositis;
• neuropsychiatric disorders (vegetopathy, psychopathy, schizophrenia, etc.).

All this indicates the need for further development of the basics of diagnosis and differential diagnosis of DM. The diagnosis of DM (PM) is usually based on the characteristic clinical and laboratory signs of the disease, with the clinical picture being the leading one. There are no officially accepted international criteria for DM, but, based on the most commonly used diagnostic criteria, no, one can distinguish 7 main diagnostic criteria for DM (PM):

• Typical skin changes.
• Progressive weakness in the symmetrical parts of the proximal muscles of the limbs according to the anamnesis and examination.
• Increased concentration of one or more serum muscle enzymes.
• Myopathic changes in electromyography.
• Typical picture of polymyositis on muscle biopsy.
• Increased creatinuria.
• Objective signs of improvement in muscle weakness during treatment with corticosteroids.

The first five criteria for DM, in the presence of the first and any three of the following four criteria, one can speak of a "definite" diagnosis of DM. In the presence of the first and any two of the following four criteria, it is proposed to regard the diagnosis of DM as "probable", and in the presence of the first and one of the following - as "possible". With PM, the presence of four criteria (2nd, 3rd, 4th and 5th) allows diagnosing "definite", in the presence of any three of the four criteria - "probable", and any two of the same four criteria - " possible" PM. According to T. Medsger and A. Masi, the diagnosis of PM is definite in the presence of the 2nd and 5th criteria or the 2nd, 4th and 3rd (or 6th) criteria; the presence of the 2nd and 4th or 2nd and 3rd (or 6th) criteria makes it possible to speak of a "probable", and the 2nd and 7th - of a "possible" diagnosis of PM.

Viral polymyositis. With viral infections, myalgias are often observed, which may be due to inflammatory changes in the muscles. Acute PM is described in viral influenza more often in childhood. On the electromyogram, no special changes are found, but the level of creatine phosphokinase in the blood serum increases significantly (10-15 times in 2/3) of cases), a muscle biopsy reveals a picture of nonspecific myopathy or inflammatory infiltration with necrosis of muscle fibers. Differences in morphological characteristics, apparently, reflect the activity and severity of muscle pathology, which correlates to a large extent with clinical parameters. Subacute myositis is sometimes observed with measles, rubella, and with vaccination using a live vaccine. Coxsackievirus infection is also accompanied by inflammatory muscle damage, and ECHO viral infection is accompanied by acute vacuolar myopathy. Virus-like particles are often found in chronic PM.

Bacterial pyogenic myositis (PM) in the form of abscesses in the muscle area is more often associated with streptococcal and staphylococcal flora. In rare cases of gas gangrene and leprosy, muscles become infected with the development of myositis.

Focal nodular myositis can be acute or chronic, the latter being closer in clinical manifestations to DM (PM); histologically, in addition to inflammatory changes, skeletal muscle infarcts are sometimes detected.

Giant cell myositis usually serves as a syndrome of various granulomatous conditions, including tuberculosis, sarcoidosis, in rare cases it appears to be independent. May be associated with giant cell myocarditis, myasthenia gravis and thymoma. Muscle biopsy reveals multinucleated muscle fibers, regenerative changes involving myofibroblasts, and, in rare cases, granulomas.

In polymyalgia rheumatica, which is often combined with giant cell temporal arteritis, pain predominates rather than muscle weakness, as in DM (PM); there is no picture of true PM, laboratory signs, but limitation of movements is expressed, which sometimes causes an erroneous diagnosis of idiopathic PM or DM.

PM is also possible with other connective tissue diseases, in particular with SJS, SLE, in some cases - with RA and sarcoidosis. With the development of drug allergies and serum sickness, myositis also often develops (No. as one of the manifestations of the general reaction.

It should also be borne in mind the possibility of developing secondary PM in various myopathies: primary muscular dystrophies, including fasciocapulofemoral, etc. Biopsy may reveal inflammatory infiltration, often interstitial, but sometimes perivascular. The use of corticosteroids (even in high doses and for a long time) in these patients usually does not give a significant effect, despite a decrease in the level of creatine phosphokinase in the blood serum. Suggests the possibility of an autoimmune response to constantly releasing] muscle antigens, which should also be suppressed, but the treatment of the underlying pathological process is, of course, decisive.

Various endocrinopathies (hypercorticism, hyper- and hypothyroidism) and metabolic disorders may accompany the development of myopathy. Known alcoholic myopathy, myopathies associated with impaired lipid metabolism or deficiency of carnitine palmitin transferase, etc.

The effect or lack of effect of corticosteroid treatment (ex juvantibus) can be used to differentiate between these conditions. However, the opposite situation should also be taken into account, when drug therapy (corticosteroids, D-penicillamine, aminoquinoline drugs, etc.) causes myopathy, which, however, is relatively rare.

Without dwelling on other primary and secondary myopathies, to facilitate the differential diagnosis of DM (PM), especially with an atypical picture of the latter, we present a list of the main groups of diseases with muscle damage of another origin, proposed by W. padley.

This list of diseases can be supplemented by granulomatous myositis (sarcoidosis), myopathies in psoriasis, panniculitis, diffuse fasciitis, steroid therapy, etc., however, even in the presented form, it illustrates a wide range of muscle damage of an inflammatory, dystrophic, and other nature.

Thus, the diagnosis and differential diagnosis of DM (PM) is often difficult due to its variability and a large number of diseases accompanied by muscle damage or having muscle, neuromuscular pathology of a different origin. However, it is striking that with a typical clinical picture of DM with a characteristic lesion of muscles and skin, in most cases, the diagnosis of the disease (especially at its onset) is erroneous. Hypo-, as well as the currently noted overdiagnosis of DM, is very fraught for patients with adverse consequences and complications due to the peculiarities of treatment and prognosis. Noting the undoubted progress in the treatment of patients with DM (PM) in recent decades, it must be emphasized that the cornerstone of the effectiveness of therapy is the early diagnosis of the disease. With the established diagnosis of DM (PM), it is vital for the patient to differentiate between primary (idiopathic) and secondary (tumor) DM, which determines the tactics of treatment and prognosis.

Laboratory data. Laboratory studies mainly characterize the overall activity of DM, and only the appearance of creatine in the urine and an increase in the level of creatine kinase, aminotransferases and aldolase in the blood testifies directly to the severity and prevalence of muscle damage. Some patients with DM have moderate anemia, leukocytosis, less often - leukopenia, eosinophilia, increased ESR, increased levels of a2- and g-globulins, seromucoid, ceruloplasmin. Immune anomalies are frequent: detection of various antinuclear and other antibodies, sometimes rheumatoid and lupus factors (often in a small titer), immune complexes, etc. When combined with neoplasm and especially with DM (PM) as part of the overlap syndrome, dysproteinemia and changes in protein fractions usually more pronounced. Of the biochemical tests, the most characteristic is an increase in the serum level of muscle enzymes, reflecting the severity of muscle damage. A good indicator of muscle pathology, which is also used as a control of the effectiveness of therapy in patients with DM (PM), is creatine phosphokinase, to a lesser extent - aldolase, aminotransferases, and the content of creatine phosphokinase can exceed the normal level by 80 times, on average it increases by 5-10 times. At the same time, individual patients with DM (PM) are described without an increase in the level of serum creatine phosphokinase (before the start of therapy), including in combination with neoplasm. Each such observation requires verification of the diagnosis and confirmation of its clear clinical, morphological and electromyographic data.

A variety of serological changes in the immune nature reflect the activity of the process, but are more often observed in DM in combination with other connective tissue diseases, especially SLE, when LE cells can be detected along with a wide range of antinuclear antibodies. Idiopathic DM (PM) is characterized by the detection of various antibodies - antinuclear, antimuscle, antimyosin, antimyoglobin, etc. Among antinuclear antibodies specific for DM (PM), according to recent studies, are PM-1, Ku, Jo-1 and Mi-2 antibodies, moreover, the latter are more frequent in DM, Jo-1 - in PM, and PM-1 is often found when PM is combined with SJS (see Pathogenesis).

In addition to the diagnostic value, the pathogenetic significance of antibodies and immune complexes, their involvement in damage to the vascular wall with the development of vasculopathy, which is characteristic, especially for juvenile DM, are discussed.

Electrophysiological studies. With the help of electromyography, a decrease in the amplitude and shortening of the duration of the biopotentials of the affected muscles, polyphasicity, sometimes - spontaneous activity such as fibrillation, pseudomyotonic disorders, etc. are revealed. According to S. M. Pearson, the following triad of electromyographic changes is characteristic of DM (PM): and positive potentials, as in muscle denervation; 2) a polymorphic complex of potentials that appears during voluntary muscle contraction, the amplitude of which is much less than normal; 3) volleys of high-frequency action potentials ("pseudomyotonia") after mechanical stimulation of the muscle. The diagnostic significance of electrophysiological studies causes conflicting opinions. Indeed, electromyography data are not strictly specific for DM (PM), may change during the course of the disease, and by themselves do not allow differentiating DM (PM) from a number of other myopathies, but in combination with the clinical picture and other studies, they are widely used to diagnose DM. (PM).

Preference is given to needle electromyography. The importance of individual signs is emphasized both for confirming the muscle damage itself and for clarifying its nature. So, polyphasic potentials along with other myogenic features is an argument in favor of the "myositis" process; the number of biphasic complexes predominates over three-phase ones.

Along with the data characteristic of PM, sometimes with repeated loads, a progressive decrease in the amplitude of myasthenic-type potentials is noted, which suggests a pseudomyasthenic form of PM or its combination with myasthenic syndrome.

It should be borne in mind that electromyography can cause muscle changes, so the biopsy should be performed in a different part of the skeletal muscle.

Morphological studies. When a muscle biopsy is performed in the affected area (muscles of the shoulder, thigh, etc.), pronounced inflammatory and degenerative changes are usually detected: cellular infiltration with a predominance of lymphocytes, the participation of histiocytes and plasma cells between muscle fibers and around small vessels, necrosis of muscle fibers with loss of transverse striation, degenerative changes, phagocytosis and elements of regeneration (Fig. 6.5). As a rule, vascular pathology is noted in the form of segmental proliferative vasculitis, thickening of the intima and sclerosis of the wall of small vessels, narrowing of the lumen, and thrombosis. More pronounced vasculopathy is characteristic of juvenile DM (PM).

Regeneration is characterized by the presence of small fibers with large nuclei, vesicular and nucleolar structures; the cytoplasm of these fibers is basophilic due to the accumulation of RNA. In a chronic process, the number of fibers of different sizes increases, the number of nuclei inside the fibers increases, endo and perimysial fibrosis increases. Atrophy of muscle fibers (mainly perifascicular) clearly predominates over hypertrophy. Along with this, there are distinct signs of interstitial fibrosis.

In a pathoanatomical study, changes in the skeletal muscles are already detected visually: the muscles are edematous, pale, the color of boiled meat, dull, atrophic; in severe cases, they are difficult to detect (total atrophy) at autopsy.

Electron microscopy reveals typical changes in muscle fibers with rupture of the sarcolemma, disruption of the structure, arrangement of myofibrils, lysis, sometimes total necrosis with infiltration by phagocytes and proliferation of phospholipid membranes in spheromembranous bodies, signs of regeneration and neoplasms of myofibrils.

In the skin with DM, vasculitis and necrosis of the vascular walls are noted, which is especially characteristic of juvenile or childhood DM. In acute cases, the dermis may be edematous (especially the papillary layer), contain lymphohistiocytic infiltrates and other components of an inflammatory-degenerative nature. In a chronic course, changes similar to those observed in SLE are possible. Poikiloderma is characterized by atrophy of the epidermal layers, degeneration of the basal cell layer, and vascular dilatation. Sometimes they do not find the actual vascular changes, but find perivascular and interstitial inflammatory cell infiltration along with thrombosis of the skin capillaries. Differences in the morphological picture reflect the clinical polymorphism of the dermatological manifestations of DM. In cases of PM, skin changes may also be absent on morphological examination. Immunofluorescent studies are more often negative and can be used to differentiate from SLE. Calcification (crystals are hydroxyapatite) at the biopsy site is detected by morphological examination.

It should be emphasized that the pathology detected by skin and muscle biopsy is not specific and should be taken into account in the diagnosis and differential diagnosis of the disease only in combination with clinical and laboratory signs of DM (PM).

Treatment of Dermatomyositis

Substantiating the progress in the study and treatment of patients with DM (PM), leading rheumatologists of the CSA identified the following main achievements: the creation of the A. Bohan and J. Peter classification, improved diagnosis, treatment with corticosteroids, cytostatics (azathioprine, methotrexate), the introduction of a test for creatine phosphokinase, clarification of the role B-Coxsackievirus infection in children, survival studies. When using the scoring system, the highest score was given to corticosteroid therapy, which is recognized as the main one in the treatment of patients with DM (PM).

Treatment with corticosteroids improves the condition of almost every patient with DM, radically - with primary DM and partially - with secondary (paraneoplastic), where effective surgery and other types of therapy remain decisive. The results of treatment of patients with idiopathic DM are especially striking with the timely and long-term use of adequate doses of prednisolone, when complete or almost complete regression of the disease and practically recovery of the patient are possible. It should be emphasized that the timeliness of treatment provides for early diagnosis of the disease. An equally important condition is the duration of therapy with the initial use of the maximum suppressive doses of corticosteroids, which serve as the drug of choice in acute and subacute forms of the disease. Having an anti-inflammatory and immunosuppressive effect, corticosteroids in sufficiently large doses are able to suppress the inflammatory and immune (autoimmune) process in muscle tissue, preventing the development of necrosis and subsequent fibrous-atrophic and dystrophic changes. Recovery (regeneration) of muscle fibers requires a long period (at least 6 months). ), which should be taken into account when monitoring patients and assessing the overall effectiveness of therapy throughout the first year of treatment. Initially, a large dose of prednisolone is given in 2-4 doses, with the morning dose being the highest. Upon reaching a certain clinical effect, the doses are gradually reduced, choosing adequate supportive ones, which patients have been taking for years. Alternative therapy with corticosteroids every other day is also possible. In chronic forms of DM, significantly lower doses of prednisolone (20–30 mg/day) are recommended, with a gradual decrease to maintenance doses (10–5 mg/day) or course treatment during an exacerbation of the disease. The effectiveness of treatment is monitored by clinical and laboratory tests, including studies of creatine phosphokinase; use electromyographic, sometimes morphological data.

Quite often already in the first weeks of treatment the state of health of patients improves, the erythema, hypostases, pains in muscles decrease or the further progressing of process stops. If there is no improvement, the initial dose of prednisolone should be increased. After 1.5-2 months of adequate therapy, the effect of treatment becomes apparent, after which a gradual decrease in the dose of prednisolone can be started. Observations have shown that in acute and subacute DM, the effectiveness of therapy is higher if during the entire first year of the disease the patient receives large doses of prednisolone, which are reduced to 40 mg in acute and up to 30 mg in subacute DM, and maintenance doses (20-15- 10-5 mg) are "worked out" already in the second and subsequent years of treatment. This dosage is maintained for a number of years, is selected individually and should be increased during exacerbation, which makes it necessary to carefully monitor patients. With a forced decrease in the dose of corticosteroids, an exacerbation of the process usually occurs, and then an increase in the dose to the original, and sometimes even higher, is inevitable. There are various schemes of therapy and dose reduction of the drug that can be taken into account, but an individual approach always remains decisive with an assessment of the initial state of the patient, monitoring the effectiveness of treatment, tolerance of the selected drug (s), complications, etc. The dose of prednisolone is always reduced gradually while maintaining the general rule: the lower the dose, the longer the interval before the next step-like decrease in it. So, at a dose of 100-80 mg of prednisolone per day, it is possible to reduce it by ½ tablet every 3-5 days, at 70-40 mg - ½ tablet in 5-10 days or ¼ tablet in 3-4 days, at 30 mg - ¼ tablet in 7-10 days, at 20 mg - ¼ tablet in 3 weeks; further more slowly. Thus, in the course of long-term therapy, an individual maintenance dose is selected, which is taken for years, but with a stable clinical remission, it can be further reduced and even canceled. The doctor observing the patient is always faced with the dilemma of choosing the most effective dose and duration of treatment, on the one hand, and the need to reduce the dose of corticosteroids. connection with their frequent concomitant side effects - on the other.

Patients with DM usually tolerate high doses of prednisolone well, but complications may occur during long-term therapy - Itsenko-Cushing's syndrome (obesity, striae, etc.), osteoporosis and steroid spondylopathy ("fish vertebrae"), sometimes with a compression fracture of the spine, steroid diabetes , gastrointestinal bleeding, infectious complications, myocardiopathies, etc. Iatrogenic complications in large series of observations are relatively rare.

Sometimes, against the background of taking high doses of corticosteroids, palpitations, gastralgia appear, blood pressure increases, excitability, the psyche is disturbed, which requires symptomatic therapy, and sometimes dose reduction and combination with other drugs (immunosuppressants, NSAIDs, etc.).

The second problem of long-term therapy is corticodependence developing in a number of patients, addiction, and therefore the withdrawal of the drug when using sometimes even small doses causes the onset of a withdrawal syndrome and exacerbation of the disease.

These difficulties are faced by virtually all physicians with long-term use of corticosteroids in patients with various diseases.

An alternative treatment option (usually taking a single dose of corticosteroids every other day in the morning) allows you to avoid or reduce the risk of complications, which can be recommended when a certain effect is achieved on classical therapy and when initial signs of cushingoid appear, which is sometimes interpreted as an additional argument in favor of the effectiveness of treatment. Additional intake of calcium (0.5 g per day) and vitamin D (50,000 IU 1-2 times a week), anabolic steroids can slow down the development of osteoporosis. During treatment with high doses of corticosteroids, potassium preparations and antacids are indicated; with fluid retention - potassium-sparing diuretics, with a tendency to hypertension - antihypertensive therapy. In the presence of foci of infection and a history of tuberculosis, antibiotics, nystatin, anti-tuberculosis drugs, etc. are recommended.

Previous attempts to treat DM with separate courses or relatively small doses of corticosteroids were unsuccessful: the prognosis of these. patients are significantly worse than when using high doses. Some authors have managed to achieve improvement in PM in children by prescribing corticosteroids at a dose of 1-1.5 mg/kg per day, with their long-term use and subsequent reduction. However, in general, the prognosis of this form, especially with the development of severe and torpid vasculitis, remains unfavorable, in some cases fatal. We also observed sick children, "sparingly" treated, who developed severe contractures, widespread calcification, partial or complete immobilization of the limbs. The predominance of tissue atrophy, sclerosis, and fibrosis gave them scleroderma-like features, which created additional diagnostic difficulties. Unfortunately, the treatment of this category of patients, already disabled, is unpromising; increasing the dose or prescribing corticosteroids has very little effect and more often leads to complications.

Prednisolone is preferred for the treatment of patients with DM, which is effective, well tolerated and easy to use with long-term use and slow dose reduction. If it is necessary to replace it with another drug from the group of corticosteroids, the use of drugs of the triamcinol group, which themselves can have a damaging effect on muscle tissue (iatrogenic myopathies), should be immediately abandoned. Dexamethasone, especially at a high dose, quickly leads to weight gain, the development of cushingoid and other complications, including mental disorders. ACTH previously used by some authors in patients with DM is ineffective. Other steroid therapy options may be used.

Parenteral administration of corticosteroids is possible as an additional and (or) temporary measure, but cannot be recommended for long-term treatment of patients with DM.

Use of steroid pulse therapy- high doses of methylprednisolone (1000 mg each), administered intravenously for three days - is evaluated ambiguously; the number of observations is still small. On the one hand, there is a certain effect, which is subsequently maintained by taking oral prednisolone, and on the other hand, the number of often severe side complications in patients with DM (PM) is increasing. Courses of pulse therapy can be repeated after a month or a number of months. Our limited experience with pulse therapy in three patients with acute DM using megadoses of corticosteroids (1000 mg of metipred) is not very encouraging. We did not notice a rapid or significant effect (apparently due to the fact that muscle recovery requires a significant time period), the need for further therapy with relatively high doses of prednisolone orally and intramuscularly (with severe swallowing disorder) remained necessary, two of three patients developed subsequent Cushingoid and spondylopathy. It seems that pulse therapy with corticosteroids can be performed in acute DM, especially for health reasons, but its wider use in DM (PM) is inappropriate.

It should be emphasized that the frequent progression or exacerbation of the disease with an insufficient dosage of corticosteroids (prednisolone) causes the patient, and sometimes the doctor, to have an erroneous idea that there is no effect, which leads to unreasonable cancellation or replacement of the drug with subsequent adverse and sometimes irreversible consequences.

With adequate (dose and duration) therapy with corticosteroids, on the contrary, the prevailing number of patients show improvement, up to the complete recovery of some of them. E. M. Tareev et al. suggest the following categories of effective therapy:

• complete cure,
• convalescence with a defect,
• persistent remission,
• significant improvement.

Complete cure involves the absence of clinical and laboratory signs of the disease after discontinuation of maintenance doses of corticosteroids for 2 years or more. By "recovery with a defect" is meant a practical cure, but with the preservation of minor muscle atrophy or individual effects of steroid therapy. "Sustained remission" implies a significant improvement in the condition with signs of regression of erythema and muscle damage, but the possible persistence of moderate muscle weakness and atrophy in the absence of creatinuria and increased levels of muscle enzymes. With "significant improvement" there is a clear positive trend along with remaining weakness, muscle atrophy, mild skin manifestations, and low creatinuria when patients continue to take moderate doses of prednisolone.

A number of researchers remain skeptical about the success of steroid therapy in DM (PM), noting its effectiveness in 40-50% of patients. However, differences in the composition of patients, in the duration of therapy and the timing of its appointment, the selected doses, methods for assessing the effectiveness of treatment, etc., should be taken into account. In general, corticosteroid therapy retains its leading place in the treatment of patients with DM (PM).

The second group of drugs actively used in DM is immunosuppressants used alone or in combination with corticosteroids. The most commonly used are methotrexate and azathioprine. The indication for their appointment is usually steroid resistance or lack of effect from corticosteroid therapy, which is rare, the presence of contraindications for use, complications. The use of immunosuppressants allows, if necessary, to reduce the dose of corticosteroids. These drugs should also be used for a long time, although, as you know, their range of side effects is much wider. There are various. schemes for the use of cytotoxic drugs. So, methotrexate can be administered intravenously and orally - 25-50 mg per week. According to another scheme (similar to treatment for RA), small doses of the drug are used: 7.5 mg per week orally at first, then 5 and 2.5 mg weekly for a long time, under the control of blood, urine, liver and lung tests, taking into account possible toxic effects. action of methotrexate.

Another drug, also quite often used in DM, is azathioprine at a dose of 2-3 mg/(kg daily). The drug gives fewer hematological complications, which allows it to be used for a long time, on an outpatient basis, but also with mandatory medical supervision. Since months of treatment are sometimes necessary before the onset of the effect, it is advisable to combine the drug with prednisone.

Cyclophosphamide and chlorambucil are used less frequently (daily dose 150-300 mg/day orally), as they have more pronounced side effects than methotrexate and azathioprine. Attempts of intravenous administration of cyclophosphamide were unsuccessful: complications were observed much more often than the effect of treatment. In some patients with DM (PM), the effectiveness of treatment with cyclosporine was noted, but the number of such observations is small.

The effectiveness of immunosuppressant treatment is difficult to assess, since they are more often used in combination with corticosteroids and the number of series of isolated observations is small. However, this group of drugs also gives a certain therapeutic effect in DM, apparently due to their pathogenetic inhibitory effect on the immune component of the pathological process, but is inferior to the relatively rapid and more demonstrative results of corticosteroid treatment, which retain a leading role in the treatment of patients with DM (PM). In the absence or insufficient effectiveness of corticosteroids, in the presence of contraindications or complications, cytostatic drugs come to the fore and can be combined with each other (at lower doses).

The most common side effect of cytostatics is associated with bone marrow suppression (manifested mainly by leukopenia), hepatotoxicity, gastrointestinal complications, skin rashes, decreased resistance to infection, etc. When prescribing cyclophosphamide, alopecia and bladder hemorrhages are also observed. These complications significantly limit the use of immunosuppressive therapy. Questions remain about possible genetic damage and an increased risk of malignant diseases, but there are no real statistics in DM (PM) in this regard.

Aminoquinoline drugs(plaquenil, delagil, etc.) can also be used in DM (PM), especially with a decrease in activity, with a chronic course and in combination with other therapy.

NSAIDs in active DM (PM) are ineffective and are indicated only as maintenance, additional therapy for long-term treatment of the disease or as a component of complex therapy for chronic DM (PM). Unfortunately, it is a common mistake to prescribe NSAIDs at the onset of the disease, which slows down the use of corticosteroids needed by patients and thereby worsens (sometimes irreversibly) the prognosis.

Encouraging results in the treatment of patients with DM (PM) are provided by plasmapheresis, although there have been almost no strictly controlled studies of its effectiveness. Nevertheless, in a number of cases with refractoriness or intolerance to corticosteroids and immunosuppressants, a clearly positive reaction to repeated courses of plasmapheresis or leukocytapheresis was noted, and subsequently the tolerability and effectiveness of drug therapy often improved. In some cases, general or local (in the area of ​​the lymph nodes) irradiation was successfully carried out.

Along with drug therapy, other methods of extracorporeal treatment can also be used, for example, repeated courses of carbohemosorption to remove immune complexes and other possible damaging factors, affect microcirculation, improve corticosteroid tolerance, etc.

When calcifications appear, colchicine is treated at a dose of 0.65 mg 2-3 times a day, Na2EDTA is injected intravenously, Trilon B is locally administered, sometimes surgical removal of individual calcifications is recommended. Unfortunately, this DM (PM) complication is difficult to treat, and the doctor's task is to prevent it by adequate, i.e., active, and sometimes "aggressive" therapy.

It is important to identify as early as possible, surgical and other active treatment of the tumor, which determines the prognosis of the patient with paraneoplastic DM (PM). As a rule, in this case, the reverse development of signs of DM is also noted, although they do not always disappear completely.

The complex treatment of patients with DM also includes repeated courses of administration of ATP, cocarboxylase, vitamin E, prozerin (during the recovery period), anabolic steroids (nerobol, retabolil), especially with prolonged use of corticosteroids, symptomatic therapy.

Patients with DM are shown a full-fledged diet with limited salt load when using high doses of corticosteroids, special diets are used only in the presence of complications. Patients with swallowing disorders require great attention, with severe dysphagia and aphagia, feeding of patients and the introduction of the necessary drugs is carried out through a probe.

With active DM (acute, subacute), at first, the motor regimen is limited, but soon, when clear clinical and laboratory changes appear on the background of treatment, one should carefully, and then more decisively, include physiotherapy exercises with exercises for the muscles of the limbs (in order to avoid contractures) in the complex of measures , respiratory and other affected muscle groups. After 1.5-2 months of treatment, it is also possible to add a massage, but not deep and not traumatic to the tissue. With the predominance of the processes of muscle atrophy and fibrosis with the development of contractures, therapeutic exercises, massage, physiotherapeutic procedures (paraffin, hyaluronidase electrophoresis, etc.) are leading in the therapeutic complex, it is possible (with the exclusion of activity) the use of balneotherapy, resort treatment.

Forecast

Before the era of corticosteroids, the prognosis of DM(GTM) was considered unfavorable, fatal in almost 2/3 of patients. With the use of corticosteroid drugs, the prognosis of the disease has improved significantly, although the opinions of scientists about the effectiveness of treatment are divided. A number of authors, positively evaluating corticosteroids in DM, note only a moderate improvement in prognosis, but most emphasize the high efficiency of this type of therapy.

When studying the survival of 144 long-term observed patients with DM 5 and 10-year survival of patients was 73 and 66%, respectively. The prognostic value of the age of patients has been established: the most favorable prognosis is in persons who fell ill at the age of up to 20 years, the lowest survival rate was noted in older age groups. If the levels of 5 and 10-year survival of patients in the first group were 100%, then in patients older than 50 years they were 57 and 38%. The worsening of the prognosis of DM in the elderly is also noted by other authors. So, in the observations of M. Hochberg et al. The 8-year survival rate of patients with DM (PM) was 56.7% in persons over 45 years of age and 96.6% in the group of patients under 45 years of age. It is quite obvious that worsening prognosis in older age groups is due to an increase in the number of patients with tumor DM. Comparison of 5 and 10-year survival rates in patients with idiopathic (89 and 81%) and tumor (15 and 11%) DM clearly illustrates the poor prognosis of the latter. In addition, one should take into account the often more severe course of DM, often complicated by the development of pneumonia, in the elderly.

There were no significant differences in the survival of patients with DM (PM) depending on gender.

A significant role in determining the prognosis is played by the nature of the course of the disease, which is also well illustrated by survival rates. So, according to M. A. Zhanuzakov, 5 and 10-year survival of patients with chronic DM remained at the level of 100%, and in acute and subacute course it was 71 and 63%, respectively.

With active forms of DM, of course, the prognosis is also determined by the duration of the disease (before the start of adequate therapy), the severity of muscle and visceral manifestations. So, in the presence of immobility, 5 and 10-year survival was 77 and 69%, and while maintaining the range of movements necessary for self-service, it was 95 and 88%. In the presence of dysphagia, the same indicators were 76 and 70%, and in patients without dysphagia - 97 and 88%. The addition of pneumonia is even more unfavorable prognostic: in the group of DM patients with pneumonia, 5 and 10-year survival rates decreased to 66 and 32% compared with 93 and 89% in the absence of pneumonia.

An important factor that improved the prognosis of patients with acute and subacute idiopathic DM should be considered timely and adequate treatment, primarily with sufficiently high doses of corticosteroids (at least 1 mg/kg of body weight). Such treatment led to the preservation of 5 and 10-year survival at the level of 96 and 90%, while in patients who did not receive adequate therapy (insufficient doses and / or periods of treatment), these figures were 70 and 56%.

In tumor DM, surgical intervention in combination with treatment with corticosteroids is decisive. This tactic contributed to maintaining survival after 5 and 10 years in this category of patients at the level of 32 and 27%.

Of the 209 patients with DM observed by E. M. Tareev and A. P. Solovieva for 25 years, there were 162 patients with idiopathic DM (group I) and 40 patients with tumor DM (group II). Most of the patients in group I received adequate drug therapy, including corticosteroids, which led to a relatively favorable prognosis. Of the 162 patients with idiopathic DM, 17 (10.5%) died, and in 5 of them the cause of death was not directly related to the underlying disease (myocardial infarction, influenza complications, etc.), in 8 it was due to complications of corticosteroid therapy (gastrointestinal bleeding). , pancreatic necrosis, infection). In group II (40 patients with paraneoplastic DM), 36 died; in 4, timely removal of the tumor led to a cure. In some operated patients, relapses or neoplasia of another localization occurred, which was accompanied by activation and growth of signs of DM, although during the period of severe tumor intoxication, the signs of DM often clearly decreased.

In the retrospective observations of J. Benbassat et al. In 94 patients with DM (TM), in order to analyze the prognostic factors of the disease, the mortality rate was 32.6%, and it was also the highest in the group of patients with tumor DM (TM). The most common causes of death were malignant tumor, pulmonary complications, coronary heart disease. The highest mortality was observed during the first year from the moment of diagnosis. Prognostically unfavorable factors include uncontrolled activity of the process and the inability to achieve remission of the disease, advanced age, as well as such clinical and laboratory signs as skin rashes, dysphagia, fever above 38 °C and leukocytosis. Sex, the presence of arthritis or arthralgia, Raynaud's syndrome, ECG changes, histological changes in muscle biopsy, an increase in the level of muscle enzymes in the blood serum, an increase in ESR, changes in the electromyogram, the level of hemoglobin, the presence of antinuclear antibodies did not affect survival. Thus, summarizing our own observations and literature data, we can conclude that the causes of death in patients with idiopathic DM (PM) are often complications of the disease (most often hypostatic and aspiration pneumonia) or treatment, changes in the general condition (cachexia, dystrophy) or internal organs ( heart with the development of heart failure, etc.). Often, a fatal outcome is associated with the addition of a concomitant disease (infection, etc.) against the background of the general serious condition of the patient.

In paraneoplastic DM (PM), the cause of death is usually a malignant tumor, although other complications should be considered.

Naturally, the term "recovery" is used to a certain extent conditionally, since patients, even after returning to an active lifestyle, require further (at least once a year) observation and employment with the exclusion of physical activity, night shifts, business trips, chemical and temperature influences, any allergenic factors, etc. Similarly, all adverse factors should be eliminated in all patients with DM, which is a kind of prevention of exacerbation of the disease. In acute and subacute cases, patients are transferred to disability group I or II, and only after a year or more, when a lasting effect is achieved, the issue of resuming study or work (with the above restrictions) can be discussed. In the chronic course of DM (PM), it is possible to maintain labor activity, subject to medical supervision and the necessary medical procedures.

Prevention of Dermatomyositis

Prevention of DM- mostly secondary, preventing exacerbations and further generalization of the process. It provides for the possible early diagnosis of the disease with the exclusion of provoking factors, timely and active treatment in a hospital, and then on an outpatient basis, dispensary observation, adequate supportive therapy, transfer to disability or employment with a load limit and the exclusion of allergenic factors. In the process of dispensary observation of patients, issues of pregnancy, treatment of focal and other infections, career guidance (for adolescents) and retraining, and rehabilitation measures are resolved. It should be noted that in intercurrent diseases and surgical interventions, corticosteroids should not be canceled.

Pregnancy in patients with DM (PM) before a stable remission is not recommended.

At present, long-term observation and treatment of patients with DM (PM), subject to timely diagnosis and adequate therapy, allow, according to M. A. Zhanuzakov et al. 3% of patients.

In cases of tumor DM, the timely detection and radical therapy of neoplasm is decisive, and DM is not a contraindication to surgical intervention.

It is desirable to observe patients by the same specialists (in a hospital, polyclinic, family doctor) in order to carry out a clear correction of treatment with the condition of patients. This applies both to specific issues of reducing the doses of corticosteroids, their cancellation with a real possibility or need for treatment with cytostatics, etc., and general treatment and rehabilitation tactics that determine the life and work prognosis of patients with DM.

In acute and subacute cases, patients are transferred to group I or II disability, but with a stable remission or “recovery”, they can return to work (study). At the same time, it is very important to eliminate allergenic factors, physical and mental overload, cooling and other situations that provoke exacerbation, which is also included in the concept of secondary prevention of DM (PM). In addition to proper employment, it is necessary to continue dispensary observation of patients, examination at least 2 times a year with a favorable course and outcome.

For primary prevention of DM in childhood, it is advisable to isolate and monitor a group of children with increased sensitivity to various exogenous and endogenous factors. Vaccination, as well as the introduction of gamma globulin, plasma and blood transfusions, antibiotic treatment in these children should be excluded or carried out with extreme caution. The risk group conditionally includes also persons with the presence of rheumatic diseases in families. In the future, with the widespread use of immunogenetic studies, it will obviously be possible to specify the predisposition to DM. However, at present, the earliest possible diagnosis of the disease, timely active therapy and prevention of exacerbation are real and important, which, along with systematic dispensary observation of patients, undoubtedly improves the prognosis and outcome of DM.

Which doctors should you contact if you have Dermatomyositis

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- a diffuse inflammatory pathology of the connective tissue with a progressive course, characterized by damage to smooth and striated muscle fibers with impaired motor functions, involvement of the skin, small vessels and internal organs. In the absence of skin syndrome, the presence of polymyositis is said to be present. The clinic of dermatomyositis is characterized by polyarthralgia, severe muscle weakness, fever, erythematous-spotted rash, skin calcifications, and visceral symptoms. Diagnostic criteria for dermatomyositis are clinical, biochemical, electromyographic parameters. The main therapy is hormonal, the course of dermatomyositis is undulating.

General information

- a diffuse inflammatory pathology of the connective tissue with a progressive course, characterized by damage to smooth and striated muscle fibers with impaired motor functions, involvement of the skin, small vessels and internal organs. In the absence of skin syndrome, the presence of polymyositis is said to be present. The clinic of dermatomyositis is characterized by polyarthralgia, severe muscle weakness, fever, erythematous-spotted rash, skin calcifications, and visceral symptoms. Diagnostic criteria for dermatomyositis are clinical, biochemical, electromyographic parameters. The main therapy is hormonal, the course of dermatomyositis is undulating.

An etiological relationship of dermatomyositis with a viral infection (picornaviruses, Coxsackieviruses) and genetic conditioning is assumed. Chronic persistence of viruses in muscles and antigenic similarity between viral and muscle structures causes an immune response with the formation of autoantibodies to muscle tissue. The starting points for the development of dermatomyositis can be hypothermia, infectious exacerbation, stress, hyperthermia, hyperinsolation, drug provocation (vaccination, allergies).

Classification of dermatomyositis

The defining symptom in the clinic of dermatomyositis is the defeat of the striated muscles. Characterized by weakness in the muscles of the neck, proximal lower and upper limbs, leading to difficulty performing daily activities. In severe lesions, patients hardly rise in bed, cannot hold their head, move independently and hold objects in their hands.

Involvement of the musculature of the pharynx and upper digestive tract is manifested by impaired speech, swallowing disorders, choking; damage to the diaphragm and intercostal muscles is accompanied by a violation of lung ventilation, the development of congestive pneumonia. A characteristic sign of dermatomyositis is a skin lesion with various manifestations. There is a development of periorbital edema, an erythematous-spotted rash over the upper eyelids, in the cheekbones, nasolabial folds, wings of the nose, upper back, sternum, joints (knee, elbow, metacarpophalangeal, interphalangeal).

Typically, the presence of a symptom of Gottron - scaly erythematous spots on the skin of the fingers, peeling and redness of the palms, brittleness and striation of the nails, periungual erythema. The classic sign of dermatomyositis is the alternation of foci of depigmentation and pigmentation on the skin in combination with telangiectasias, dryness, hyperkeratosis and atrophy of skin areas (poikilodermatomyositis).

On the part of the mucous membranes with dermatomyositis, the phenomena of conjunctivitis, stomatitis, edema and hyperemia of the palate and posterior pharyngeal wall are noted. Sometimes there is an articular syndrome with damage to the knee, ankle, shoulder, elbow, wrist joints, small joints of the hands. With juvenile dermatomyositis, intradermal, intrafascial and intramuscular calcifications may appear in the projection of the pelvic, shoulder girdle, buttocks, and joints. Subcutaneous calcifications can lead to ulceration of the skin and the release of calcium deposits to the outside in the form of a crumbly mass.

Among the systemic manifestations of dermatomyositis, heart lesions (myocardiofibrosis, myocarditis, pericarditis) are observed; lungs (interstitial pneumonia, fibrosing alveolitis, pneumosclerosis); Gastrointestinal tract (dysphagia, hepatomegaly); kidneys (glomerulonephritis), nervous system (polyneuritis), endocrine glands (hypofunction of the adrenal glands and gonads).

Diagnosis of dermatomyositis

The main diagnostic markers of dermatomyositis are the characteristic clinical manifestations of skin and muscle lesions; pathomorphological transformation of muscle fibers; increased levels of serum enzymes; typical electromyographic changes. Additional (auxiliary) criteria for the diagnosis of dermatomyositis include dysphagia and calcinosis.

The reliability of the diagnosis of dermatomyositis is not in doubt in the presence of 3 main diagnostic criteria and skin rash, or 2 main, 2 auxiliary criteria and skin manifestations. The possibility of dermatomyositis cannot be ruled out when skin lesions are identified; with a combination of any 2 other main manifestations, as well as with a combination of any main and 2 auxiliary criteria. To establish the fact of polymyositis, it is necessary to have 4 diagnostic criteria.

The blood picture is characterized by moderate anemia, leukocytosis, neutrophilic shift of the leukocyte formula to the left, an increase in ESR in accordance with the activity of the process. Biochemical markers of dermatomyositis are an increase in the level of α2- and γ-globulins, fibrinogen, myoglobin, sialic acids, haptoglobin, seromucoid, transaminases, aldolase, reflecting the severity of muscle tissue damage. An immunological blood test in dermatomyositis reveals a reduced complement tyr, a decrease in the number of T-lymphocytes, an increase in the level of IgG and IgM immunoglobulins with a decrease in IgA, a small number of LE cells and antibodies to DNA, a high content of myositis-specific antibodies, the presence of nonspecific antibodies to thyroglobulin, myosin, endothelium etc.

In the study of musculoskeletal biopsy specimens, a picture of severe myositis, fibrosis, degeneration, inflammatory infiltration of muscle fibers, and loss of transverse striation are determined. An electromyogram with dermatomyositis fixes increased muscle excitability, short-wave polyphase changes, fibrillar fluctuations at rest. Soft tissue radiographs show areas of calcification; X-ray of the lungs is determined by an increase in the size of the heart, pleural calcifications, interstitial fibrosis of the lung tissue. The bones show moderate osteoporosis.

Treatment of dermatomyositis

In case of damage to the respiratory muscles and palatine muscles, it is necessary to ensure the functions of adequate breathing and swallowing. To suppress inflammation in dermatomyositis, corticosteroids (prednisolone) are used under the control of blood serum enzymes and the clinical condition of the patient. In the course of treatment, the optimal dosage of corticosteroids is selected, the drugs are taken for a long time (1-2 years). It is possible to conduct steroid pulse therapy. The anti-inflammatory regimen for dermatomyositis can be supplemented by the appointment of salicylates.

Forecast and prevention of dermatomyositis

With an advanced course of dermatomyositis, mortality in the first 2 years of the development of the disease reaches 40%, mainly due to damage to the respiratory muscles and gastrointestinal bleeding. With a severe protracted nature of dermatomyositis, contractures and deformities of the limbs develop, leading to disability. Timely intensive corticosteroid therapy suppresses disease activity and significantly improves the long-term prognosis.

Measures to prevent the development of dermatomyositis have not been developed. Among the measures of secondary prevention of dermatomyositis are dispensary control of a rheumatologist, maintenance therapy with corticosteroids, reduction of reactive hypersensitivity of the body, sanitation of focal infection.

Dermatomyositis is a chronic rheumatic inflammatory disease of the muscles and skin. Another name for the disease is polymyositis, it is mainly used to refer to an ailment without symptoms of skin lesions (25% of all cases). Dermatomyositis is a rare disease, on average, according to world statistics, it is diagnosed in 5 people per million people per year. Children under 15 years of age and persons over 55 years of age are more often ill. Women get sick 2 times more often than men. In this article, we will look at the symptoms and treatment of this disease.

Why does dermatomyositis develop?

As in the case of other rheumatological diseases, the underlying cause of the disease has not been found. It is expected to be associated with the following factors:

1. Chronic viral infection (for example, Coxsackie virus, herpes zoster,).
2. , 30% of patients with dermatomyositis have an oncological diagnosis. Here, both the autoimmune reaction (when the body attacks both tumor cells and its own cells) and the direct toxic effect of the decay products of tumor cells are important.
3. genetic predisposition. In individuals with dermatomyositis, the blood test shows the accumulation of HLAB8, which is associated with a variety of immune disorders.

Clinical variants of dermatomyositis

Classification of forms of dermatomyositis:

1. Primary idiopathic polymyositis. The term "idiopathic" means that the cause of the disease or condition is unknown.
2. Primary idiopathic dermatomyositis.
3. Dermatomyositis in combination with tumors.
4. Dermatomyositis in combination with.
5. Dermatomyositis in combination with.

Dermatomyositis in women

Most often, women from 30 to 50 years old get sick with polymyositis. Typical manifestations: gradual increase in symptoms, skin rashes, arthralgia.

Dermatomyositis affects mainly women of the same age group, but, unlike polymyositis, the disease begins and proceeds acutely, there is a severe musculoskeletal syndrome.

Dermatomyositis in children

In children, the variant of dermatomyositis is most often found in combination with vasculitis. The disease is acute, often recurs.

Dermatomyositis, combined with tumors, affects equally boys and girls.

Course of the disease

The course of the disease determines the scope and nature of the treatment. Allocate:

• Acute course of dermatomyositis. Within six months, the patient is involved in the process of O most of the muscles. Because of this, a person can no longer move, swallow himself, and sometimes even speak. The patient suffers from fever and poisoning with toxic decay products of his own muscles. The cause of death at this stage is aspiration pneumonia (for example, when vomit enters the lungs) or against the background of heart damage.
• Subacute flow. Dermatomyositis regularly recurs, worsening the patient's condition. Gradually, manifestations of damage to internal organs increase. The patient also becomes immobilized over time. Against the background of specific treatment, a long-term remission (periods of relative health) is possible. Its duration depends on the condition of the patient and on how carefully he fulfills medical prescriptions. With proper attention to yourself, the patient can live for many years, being only slightly limited in movement.
• Chronic course. The most favorable variant of the course of dermatomyositis. The disease affects only some muscle groups, so the patient feels relatively well and is able to work productively and live a full life. The exceptions are young men, who may develop large areas of calcification in the skin and muscles. This leads to immobility of the limb or joint, and therefore significantly worsens the quality of life of the patient.

Symptoms of dermatomyositis

Muscular manifestations

1. Pain in the muscles during movement and at rest.
2. Myalgia caused by pressure on the muscle.
3. Increasing muscle weakness, leading to disability of the patient. Over time, weakness increases so that the patient loses the ability to get up, sit down, eat independently. In the end, he is completely immobilized.
4. The pathological process also extends to the muscles of the face, so the patient is completely deprived of the opportunity to express his emotions through facial expressions.
5. The muscles of the larynx, pharynx, soft palate are affected. Because of this, a person's voice changes, and there may be problems with swallowing food and water.
6. The defeat of the intercostal muscles and the diaphragm leads to respiratory failure, hypoventilation and the development of pneumonia.

Skin lesions

1. In 40% of patients, erythema occurs on open parts of the body (face, neck, limbs).
2. Rashes such as papules and large blisters (bull).
3. Telangiectasia.
4. Hyperkeratosis (excessive keratinization).
5. Hyperpigmentation.
6. Purple puffy spots around the eyes - dermatomyositis glasses.
7. Scaly red spots over the joints of the hands - Gottron's syndrome.

Raynaud's syndrome

Raynaud's syndrome is accompanied by numbness, a feeling of coldness, a feeling of goosebumps and is accompanied by pain in the hands; between attacks, the hands may remain cold and bluish. In addition to the extremities, manifestations of the syndrome can be observed in the area of ​​the tip of the nose, chin, earlobes and tongue. The duration of the attack ranges from several minutes to several hours.

Raynaud's syndrome occurs in 10% of patients.

Joint damage

1. When moving, pain in the joints occurs, which torments and limits the patient.
2. Sometimes the muscles are affected so quickly and severely that a person cannot bend his arm at the elbow or leg at the knee precisely because of the formation of "ankylosis of a muscular nature." Ankylosis is the inability to move a joint.

Damage to the cardiovascular system

Damage to the gastrointestinal tract

Occurs in 50% of patients. Anorexia is noted. Due to damage to smooth muscles, hypotension of the esophagus, edema and necrosis in the walls of the stomach and intestines can develop.

Diagnosis of dermatomyositis

Blood tests

Elevated levels of leukocytes (a sign of inflammation), eosinophils (a sign of an allergic reaction), high ESR, anemia. High levels of creatine phosphokinase, C-reactive protein, fibrinogen.

Muscle biopsy

The histologist detects thickening of muscle fibers and areas of necrosis in the tissue sample.

The diagnosis is made based on the presence of the following symptoms:

1. Progressive muscle weakness.
2. Skin syndrome (manifestations typical of dermatomyositis).
3. Increased activity of muscle enzymes (high levels of creatine phosphokinase).
4. Typical changes in muscle tissue, established by the results of a biopsy.

Treatment of dermatomyositis

The drugs of choice in the treatment of patients with dermatomyositis are. These are substances with high anti-inflammatory activity. They are especially effective when prescribed in an adequate dose for the stage of the disease, preferably in the early stages.

The main glucocorticosteroid in the treatment of dermatomyositis is prednisolone. It is prescribed in tablets, at a dose of up to 100 mg per day, in 4-6 doses. Usually, in 1-2 weeks of hormone therapy, the patient's condition improves significantly: the timbre of the voice is restored, the patient stops choking when eating, pain and weakness in the muscles decrease.

After reaching the maximum effect, the dose of prednisolone is gradually reduced. This is done in order to find the number of tablets per day that will help the patient to maintain a satisfactory condition and avoid periods of exacerbation.

Unfortunately, prednisolone, like other glucocorticosteroids, has a number of side effects:

1. Education .
2. Accession of infections.