DERMATOMYOSITIS

From Big Medical Encyclopedia

DERMATOMYOSITIS (dermatomyositis ; grech, derma, dermat[os] skin + mys, myos a muscle + - itis; synonym: Wagner's disease, Wagner's disease — Unferrikhta — Heppa) — the disease which is characterized by disturbance of motive function as a result of systemic lesion cross-striped and to a lesser extent smooth muscles and also damage of skin. Treats group of diffusion diseases of connecting fabric.

The acute form D. is described by Wagner (E. Wagner, 1863), Unferrikhtom (H. Unverricht, 1887) and Hepp (P. Hepp, 1887), hron, a form — Petzhem and Klezha (G. Petges, S. of Clejat, 1906). As the diffusion general disease of connecting fabric D. is studied only from 40th of 20

EL meets at any age; prevails at women. Incidence 1: 200 000 — 1: 280 000 [Rose and Walton (A. Bose, J. Walton), 1966; Medsger, Doson, Ointments (T. Medsger, W. Dawson, A. Masi), 1970].

The etiology

the Aetiology is unknown. A number of authors considers D. as reaction of a sensitization to various antigens (microbic, tumoral, etc.). This concept is spoken well a wedge, by displays of a disease, such as knotty erythema (see. Erythema knotty ), small tortoiseshell (see), eosinophilia (see), quite often observable at the beginning of a disease. W. Norton with sotr. (1970), Klug and Zennikhsen (N. of King, N. Sonnichsen, 1973) found virus-like cytoplasmic inclusions in the struck fabrics (in cytoplasm of fibroblasts of skin, in an endothelium of capillaries of skin and muscles, a sarcoplasm of muscle fibers) and on the basis of it consider possible a role of viruses in D.'s etiology

the Pathogeny

the Pathogeny is found out insufficiently. The most recognized is the hypothesis of the autoimmune mechanism of development of. Existence of antibodies to skeletal muscles testifies to autoimmune disorders. Damage of muscles can be caused as well by cellular immune responses (reactions of the slowed-down type); such representation is confirmed by experimental data: at introduction to Guinea pigs of heterogeneous muscular suspension with a Freund's adjuvant (see. Adjuvants ) at animals the generalized miositis reminding D. at people develops.

Pathological anatomy

Fig. 1. Microdrug of the changed skeletal muscle at a dermatomyositis: the necrosis of muscle cells (1) and inflammatory infiltration are expressed (2).

At D. on autopsy generalized defeat of skeletal muscles is noted. Muscles are edematous, pale, gray or yellowish-brown color, with the centers of a necrosis, fibrosis and calcification (see). Microscopically morfol. changes in muscles are very variable and depend on a stage and rates of disease, and also age, in Krom there was a disease. It is noted focal proteinaceous dystrophy (see) and vacuolar dystrophy (see) myocytes with the subsequent necrosis and macrophagic reaction from a stroma (fig. 1). The centers of defeat are surrounded with the infiltrate consisting preferential of the small lymphocytes and plasmocytes which are located around vessels or diffuzno between muscle fibers. Further intersticial fibrosis develops, against the background of to-rogo the intensification of regenerative processes from myocytes is observed. Intensity of intersticial fibrosis depends on character of a current, duration and a stage of a disease. Fibrosis is more often observed at the acute massive necrosis of muscle cells developing at late begun treatment. In the outcome of a disease the atrophy of muscle fibers develops (see. Atrophy muscular ), alternating with their focal compensatory hypertrophy.

Electronic microscopically at an aggravation of process the focal degeneration of muscle cells, formation of cytoplasmatic hyaline little bodies is observed.

The thickening of an endothelium and a basal membrane of intramuscular arterioles and capillaries is observed. Quite often find the virus-like inclusions reminding those at in cells of an endothelium to a system lupus erythematosus (see).

In skin and hypodermic cellulose the centers of a necrosis and hypostasis are found with mucous dystrophy (see), and also fibrosis and calcification.

In a myocardium the changes similar to changes in skeletal muscles however expressed much more weakly are found. The endocarditis and a pericardis are observed extremely seldom. Fatty dystrophy of a liver is possible. Generally visceral changes come down to moderate inflammatory and sclerosing processes in a stroma, to vasculites (see. Vasculitis ) and to insignificant defeat of the unstriated muscles which are a part of bodies.

At D. changes from motor trailer nerves and their terminations are noted. In them dystrophic and regenerative processes are observed. It is noted also - dependence between weight of changes in muscles and in nerve fibrils.

For patomorfol, changes at D. at children dominance of destructive pan-vasculites which are not limited only to muscles or skin is characteristic, but extend and on went. - kish. path, heart, easy, peripheral nerves, etc. The hyperplasia of an intima and fibrosis of a wall of vessels conduct to their occlusion and hypoxemic changes in bodies.

The clinical picture

the Standard classification of D. does not exist. Allocate idiopathic, primary, and symptomatic, secondary, D. developing in response to tumoral antigens and D. at children.

The secondary dermatomyositis on a wedge, a picture essentially does not differ from primary. According to Williams (R. Williams, 1959), secondary D. is observed in 17% of cases; among sick D. 40 years are more senior secondary D.'s frequency increases to 50%. D.'s symptomatology for months or even years can precede displays of a tumor. Most often D. is noted at tumors of a lung, prostate, ovary, uterus, mammary gland and large intestine. Separate cases of D. at malignant lymphoma are described, and also at high-quality and malignant thymomas. On character of a current distinguish acute, subacute and hron, forms D. The acute form is characterized by fever from oznoba, quickly accruing generalized damage of the skeletal muscles progressing dysphagy (see), dysphonia (see), damage of heart and other bodies. Acute D. at adults is observed seldom. The subacute form differs in more drift. The disease begins with incremental weakness of muscles more often, edges comes to light at physical. to loading (overcoming high steps, washing of linen, etc.), is more rare from the phenomena of dermatitis. Later damage of muscles of a shoulder and pelvic girdle amplifies, the dysphagy, a dysphonia join. In 1 — 2 years from the beginning of a disease the developed picture D. with severe damage of muscles and visceral bodies is usually observed. Hron, a form D. proceeds cyclically, is long processes of an atrophy and sclerosis of muscles and skin prevail, involvement in process of the isolated groups of muscles of distal departments of extremities (a muscle of forearms, shins) is possible. Damage of muscles is often combined with hron, recurrent dermatitis (see).

fig. 4. Some clinical manifestations of a dermatomyositis — a paraorbital erythema and hypostasis (a symptom of „points“), puffiness of the person, it is cyanotic - pink coloring of skin and lips;

Damage of skin at D. polymorphic: prevail erythema (see) and swelled (see), it is preferential on open parts of a body. Petekhialny, papular, violent rashes are observed (see. Rashes ), teleangiectasias, centers of pigmentation and depigmentation, hyperkeratosis, etc. Skin, hl. obr. over the affected muscles, edematous, pasty or dense. The erythema is often localized on a face, a neck, a breast, over joints, on an outer surface of a forearm and shoulder, on a front surface of hips and shins; differs in big firmness, quite often is followed by a peeling and an itch. A peculiar periorbital hypostasis and an erythema is characteristic (tsvetn. fig. 4) — a symptom of «points». Trophic disturbances, a xeroderma, longitudinal striation and fragility of nails, a hair loss, etc. are quite often observed. More than at a half of patients simultaneous damage of mucous membranes in a look is noted conjunctivitis (see), stomatitis (see), hyperemia and hypostasis of a pharynx, and also voice folds. The skin syndrome usually precedes emergence of other signs of D., including and damage of muscles, however at a part of patients of change from skin are practically absent (actually a polymiositis).

Cardinal sign of D. is defeat of skeletal muscles. Damage preferential of muscles of proximal departments of extremities, a shoulder and pelvic girdle, a neck, a back, a throat, upper parts of a gullet, sphincters is characteristic. There are muscle pains, especially at the movement and a palpation; muscles are dense or testovata, are increased in volume. Steadily progressing muscular weakness is expressed in considerable restriction of active movements. Patients cannot independently rise, sit down, raise a leg on a step (a symptom of «bus»), to hold any subject in a hand, to brush the hair, put on (a symptom of «shirt»), easily fall during the walking; at damage of muscles of a neck and back cannot tear off the head from a pillow or hold in vertical position (the head falls on a breast); at damage of mimic muscles the maskoobraznost of the person appears. At height of a course of a disease (at an acute and subacute current) patients are almost completely immobilized; the movements are kept only in brushes and feet.

Involvement in process of pharyngeal muscles causes the phenomena of a dysphagy (a poperkhivaniye during the swallowing, liquid food pours out through it). Aspiration of food is possible. Damage of intercostal muscles and a diaphragm leads to restriction of mobility and decrease vital capacity of lungs (see). At damage of muscles of a throat the nasal shade of a voice, hoarseness appears; damage of muscles of an eye conducts to diplopias (see), to a ptosis (see); damage of muscles of sphincters — to disorder of their activity. Then the atrophy of the affected muscles or a picture of an ossifying miositis develops (see the Miositis). Calcification at D. is secondary and has reparative character. The centers of calcification are more often localized in the most affected muscles of a shoulder and pelvic girdle and in hypodermic cellulose in the form of plaques or massive deposits. The centers of calcification located superficially can be opened with allocation of limy weight.

The defeat of a nervous system noted at D. gave the grounds to Senator (H. Senator, 1888) to call a disease of a neurodermatomyositis. Changes are noted preferential in peripheral and the autonomic nervous system; defeat of c. the N of page is observed seldom and expressed in the form of astheno-depressive and asthenic syndromes (see. Asthenic syndrome ). On EEG rhythms of biopotentials come to light patol. Some authors note a possibility of development of meningitis and encephalitis with convulsive attacks.

Defeat of a peripheral nervous system can be shown by radicular pains, morbidity of nervous trunks, mono - and polyneurites (see the Polyneuritis). At polyneurites sensitivity, especially in distal departments of hands and legs is broken. Decrease in sensitivity, as well as increase it, does not happen deep. Reflexes are usually lowered, sometimes unevenly. Decrease or loss of tendon jerks can be result of the combined damage of muscles and peripheral motive neurone.

Vegetative disturbances are various — tendency to hypotonia, tachycardia, disturbance of thermal control, anorexia, etc.

Almost at a half of patients it is observed focal or diffusion myocarditis (see), sometimes with disturbances of a heart rhythm and the phenomena of congestive heart failure. The endocarditis and a pericardis meet seldom.

Damage of lungs is shown by vascular or intersticial pneumonia from the outcome in a pneumosclerosis (see. Pneumosclerosis ). Separate cases of development of calcification of lungs are described. Pulmonary insufficiency arises rather seldom and is caused mainly by damage of respiratory muscles and diaphragms.

Damage of smooth muscles went. - kish. a path leads to hypotonia of a gullet and intestines. At a part of patients the loss of appetite, abdominal pains, the phenomena is noted gastroenterocolitis (see). Zhel. - kish. bleedings and perforation of intestines at adult patients are rare. Moderate increase in a liver is observed approximately at 1/3 patients.

Cases of a heavy glomerulonephritis with hypertensia and a renal failure at D. are very rare, more often damage of kidneys is shown by passing proteinuria (see).

Also generalized lymphadenopathy, increase in a spleen belong to rare symptoms of D. Defeats of vessels of an eyeground are in some cases described.

From the general symptoms of a disease of the most frequent the lose of weight, sometimes considerable is (on 10 — 20 kg). Febrile temperature is noted at an acute current or D.'s aggravation; at subacute and hron, a current subfebrile temperature is registered.

Arthritises are rare. Approximately at 25% of patients are observed arthralgia (see) and hypostasis of periartikulyarny fabrics. Dysfunctions of joints are connected with damage of muscles. Sometimes D. is combined with Reynaud's syndrome (see. Reynaud disease ).

Laboratory researches at the acute and subacute course of a disease show moderate anemia, a neutrophylic leukocytosis, the eosinophilia), the accelerated ROE, increase alpha 2-1 and gamma-globulins is more rare a leukopenia. An indicator of sharpness and prevalence of damage of muscles is increase in blood of activity of enzymes — kreatinfosfokinaza, glutaminic and pyroracemic transaminases, a lactate - and malate dehydrogenases, and also emergence in urine of creatine. At hron, D.'s current of change of data of a laboratory research do not happen so accurate and expressed. At a number of patients the raised caption of a rhematoid factor is noted. Antinuclear antibodies and lupoid cells find exclusively seldom.

According to Pearson (S. M. of Pearson, 1972), at an elektromiografichesky research (see. Electromyography ) the characteristic triad is found: spontaneous fibrillation and positive potentials of muscular currents; the polyphase complex of potentials with a small amplitude appearing at any reduction of a muscle, volleys of high-frequency action potentials («psevdomiotoniya») after mechanical irritation of muscles.

Complications

the Most frequent and terrible complication, a cut is on the first place among causes of death at acute D., is heavy aspiration pneumonia (see), developing as a result of aspiration of food masses at disturbance of swallowing. Constant hypoventilation of lungs (see. Lung ventilation ) owing to damage of intercostal muscles and a diaphragm creates premises for development of bacterial pneumonia. In some cases severe damage of respiratory muscles with sharp restriction of an excursion of a thorax can conduct to accruing respiratory insufficiency (see) and to the phenomena asphyxia (see). At the immobilized patients can arise trophic ulcers (see), decubituses (see). Development of exhaustion is possible. A heart and renal failure at D. are rather rare.

The diagnosis

the Diagnosis is based on a wedge, displays of a disease, first of all on characteristic damage of muscles and skin. Have diagnostic value eosinophilia (see), increase in content of enzymes, creatinuria (see). For specification of the diagnosis of D. the large role is played by elektromiografichesky researches and especially these biopsies of muscles and skin. Separate sick D. can have similar to damage of skin changes of skin at a system lupus erythematosus; the morphology of muscles at D. is more characteristic.

In all cases of D., especially at senior citizens, it is necessary to carry out careful an all-wedge. inspection for an exception of a tumor.

Fig. 2. The roentgenogram of hips of the patient dermatomiozigy with the expressed calcification in muscles.

Radiological data at D. are not specific, however they can promote specification of extent of defeat of soft tissues and internals. Roentgenograms should be made by means of so-called soft radiation to receive structure of soft tissues. In an acute stage of a disease on such roentgenograms of a muscle look more transparent, enlightenments are noted. Hypodermic cellulose is very transparent, sometimes in it even small veins are visible. At hron. Typical is availability of calcificats in soft tissues (fig. 2). In hypodermic cellulose irregular shape calcificats most often are found, and on border of muscles and hypodermic cellulose the tape type of calcification is sometimes noted. In the field of a hip joint often there is extensive calcification — so-called pseudo-tumorous changes.

In lungs the picture of intersticial fibrosis of preferential basal departments comes to light. Calcificats in a pleura are sometimes noted. Heart is often increased in sizes.

Differential diagnosis at acute and subacute D. it is necessary to carry out with infectious and nevrol, diseases, system scleroderma (see), system lupus erythematosus (see).

At the acute beginning of D. when are available the fever, oznoba accelerated by ROE, the increasing muscular weakness allows to exclude infectious diseases (sepsis, typhus, an ugly face, etc.). The Bystry course of a disease, an obezdvizhennost, disturbance of swallowing imitate heavy polyneuritis (see). Specification of genesis and the nature of the observed defeats allows to differentiate false and neurologic symptomatology from true.

The scleroderma, as a rule, has no acute beginning. The leading symptom at it is the dense cutaneous dropsy without the phenomena of dermatitis.

Unlike a system lupus erythematosus, at D. visceral pathology is not so expressed, in a picture of a disease damage of muscles, other nature of skin changes, in blood prevails there are no lupoid cells.

Hron. Without skin syndrome (actually a polymiositis) should be differentiated with various myopathies: the progressing muscular dystrophy, a thyrocardiac myopathy, etc. (see. Myopathy ). The biopsy of a muscle often has crucial importance.

Treatment

At acute, subacute and an aggravation hron. Appoint corticosteroids, excepting triamsinolon which is contraindicated at D. since it can cause a myopathy. Use of Prednisolonum in adequate, usually big, doses is preferable: at an acute current of 80 — 100 mg, at subacute 60 mg, at an aggravation hron. 30 — 40 mg a day. These doses on condition of portability and lack of contraindications are appointed within 2 — 3 months and more to clear therapeutic effect. In the next months make very slow dose decline of Prednisolonum to supporting: at acute and subacute D. within the first year of a disease it shall be 30 — 40 mg; on the second and third year of a disease the maintenance dose decreases to 20 — 10 mg; in the period of deep a wedge, remissions full drug withdrawal is possible. At an exacerbation of a disease and stressful situations the dose is increased. Contraindications to use of corticosteroids (except a triamsinolon) at acute D. are practically absent.

Along with corticosteroids use cytostatic drugs. There are messages on good effect of use of a methotrexate, Azathioprinum, 6 Mercaptopurinum, cyclophosphamide. However the technique of treatment of D. is still insufficiently developed by cytostatic drugs. Duration of treatment is 2 — 6 months. At a combination to corticosteroids shorter use courses of treatment.

Aminoquinolinic drugs: Chingaminum (chloroquine, Resochinum, delagil), hydroxychloroquine (plaquenil) apply is long (for years), practically at all forms D. At acute and subacute D. purpose of these drugs during reduction of a dose of corticosteroids is reasonable, at hron. — from the moment of establishment of the diagnosis.

Depending on specific features of a disease in a complex with corticosteroids it is possible to apply non-steroidal anti-inflammatory drugs — salicylates (acetilsalicylic to - that) and indometacin in the standard doses.

Complex treatment of D. includes also cocarboxylase and vitamins of group B, and also anabolic hormones (Nerobolum, retabolil) which are especially shown at long use of corticosteroids or exhaustion of the patient. In the presence of calcification kompleksonoobrazovatel, in particular apply disodium salt ethylene diamine tetraacetic to - you (Na 2 EDTA).

At acute and subacute D. the bed rest, careful leaving is necessary, further — gradual expansion of volume of movements with careful use of separate receptions to lay down. physical cultures. Massage and physiotherapeutic methods of treatment can be applied only at clear decrease of the activity of process and transition of a disease in hron, a form. At dominance of processes of an atrophy and fibrosis of muscles with development of contractures by leaders in a therapeutic complex become to lay down. gymnastics, massage, physiotherapeutic procedures (paraffin applications, an electrophoresis with hyaluronidase, etc.). During the subsiding of activity of process the balneoterapiya, a resort therapy are possible.

Sick D.' food shall be full, protein-rich and vitamins.

The forecast

the Forecast at uncured acute and subacute D. bad. Under the influence of timely therapy by corticosteroids these forms get more high-quality, hron, a current, and at a part of patients there occur long-term full remissions with recovery of working capacity. The forecast for life at hron. Is more favorable, the forecast concerning working capacity bad.

At secondary D. the forecast depends on a current of a basic disease.

Prevention at D. — practically the prevention of an aggravation and progressing of process. It provides perhaps early diagnosis of a disease, timely and active treatment in a hospital, and then dispensary observation for patients, an adequate maintenance therapy, transfer into disability or employment with restriction physical. loadings and exception of allergenic factors.

The dermatomyositis at children

the Clinical picture

the Beginning is more often subacute. However acute, rapid development of a disease is quite often observed. Hron, and subacute D. at children can become more active.

A wedge, D.'s manifestations at children's age generally do not differ from those at adults, but a current more malignant. The wavy, progressing current with temperature increase, crushing systemic lesion of muscles, the expressed organ pathology is characteristic. The variety a wedge, syndromes is connected with involvement in patol, process of vessels of different caliber, first of all a microcirculator bed.

fig. 1. Some clinical manifestations of a dermatomyositis — a paraorbital erythema and hypostasis, moderate puffiness it is also cyanotic - pink coloring of lips, a bilateral ptosis (it is more on the right);
fig. 2. Some clinical manifestations of a dermatomyositis — symmetric erythematic rashes over interphalangeal joints, a red border at a nail;
fig. 3. Some clinical manifestations of a dermatomyositis — the widespread vascular drawing on skin, the extensive centers of a necrosis;

Skin changes meet big constancy (actually the polymiositis represents an extreme rarity) and, in addition to the paraorbital and located over joints erythema, often are followed by the deep necrosis of skin and hypodermic cellulose extended by hypostasis of N (tsvetn. fig. 1 — 3). During a disease the erythema fades, appear teleangiectasia (see), sites of a peeling, hyper - and the depigmented spots. Over joints skin loses elasticity, becomes wrinkled, rough or thinned, reminding so-called atrophic scars. Various changes of mucous membranes of an oral cavity, respiratory tracts, the conjunctivas which are shown catarral and ulcer reactions with hypostasis are often observed.

Fig. 3. The child suffering from a dermatomyositis. The diffusion muscular atrophy and tendinous and muscular contractures are expressed.
fig. 5. Some clinical manifestations of a dermatomyositis — the multiple kaltsinata which are moderately towering over skin, around some of them the wide invaded zone.

Diffusion weakness of skeletal muscles with pains and hypostasis limits an athletic ability of the child and quite often leads almost to a full obezdvizhennost. At children considerably more often than at adults, tendinous and muscular contractures (fig. 3) and calcification develop. While at adults development of calcification of muscles means the termination of an active phase of a disease, at children widespread calcification can be observed in combination with active D. Inogda kaltsinata partially act over a surface kozhn (tsvetn. fig. 5).

Damage of respiratory and pharyngeal muscles is shown dysphagy (see), dysarthtia (see), decrease in an excursion of a thorax. The progressing damage of respiratory muscles can lead to an apnoea.

At a half of patients the arthralgia is noted. Arthritises, sometimes with the subsequent deformation of joints can be observed.

Damage of lungs at D. is expressed most often by the vascular and intersticial pneumonia which is characterized by progressing of process, formation of pulmonary fibrosis, emergence of signs of a pulmonary heart and bronchial pneumonia. Stratification of consecutive infection, a pulmonary tuberculosis is possible. Pleurisy can be both dry, and exudative, is more often without considerable exudate, with development of adhesive processes.

Damage of heart is more often shown diffusion or focal myocarditis (see), a myocardial dystrophy and is more rare endomyocarditis, mioperikardity or pancarditis (see). Are sometimes observed a ptosis (tsvetn. fig. 1), exophthalmos.

In an active phase of a disease vessels of an eyeground in a varying degree are surprised.

Phenomena gastroenterocolitis (see) are noted at a half of sick children, sometimes with bleedings, ulcer processes up to perforation. An abdominal syndrome (see. Pseudoabdominal syndrome ) usually accompanies heavy a wedge, a picture and is a consequence of a widespread vasculitis and dystrophic changes (treatment by corticosteroids strengthens trophic disturbances).

Generalized damage of muscles quite often simulates nevrol, symptomatology, at the same time true defeat of the central and peripheral nervous system meets big constancy.

In most cases D. is followed in a varying degree by the expressed gepatosplenomegaliya (see. Hepatolienal syndrome ), lymphadenopathy. The accelerated ROE, proteinaceous, enzymatic and immunol, shifts, a small proteinuria with moderate changes of an urocheras are observed. Degree of manifestation of these changes matches activity of process.

Treatment

At the expressed D.'s activity is appointed Prednisolonum, by 1 — 3 mg to 1 kg of weight of the child a day. When dynamics of damage of respiratory and pharyngeal muscles happens torpid, use of «shock» doses is necessary (4 — 6 mg on 1 kg of weight a day). At stabilization of process (usually in 4 — 8 weeks) the dose is gradually reduced (individually) to supporting (from 20 to 5 mg a day). Patients receive a maintenance therapy is long, sometimes for years, being iod outpatient observation. Use of antikholinesterazny means, salicylates, pyrazolon and quinolinic drugs, and also the means improving exchange, a trophicity and regenerative processes in fabrics is reasonable (anabolic hormones, glutaminic and adenosine triphosphoric to - you, drugs of potassium, vitamins). Antibiotics are appointed according to indications.

At gormonorezistentny forms D. or development of complications in the course of treatment by corticosteroids (compression fractures of bodies of vertebrae, steroid diabetes, ulcers went. - kish. a path etc.) cytostatics, however in connection with frequent development of trophic disturbances, accession of consecutive infection with a great opportunity for development of sepsis are applied, their use is limited.

For treatment of kaltsinat disodium salt ethylene diamine tetraacetic to - you is recommended (Na 2 EDTA), a single dose a cut from 250 mg to 1 g (5 — 20 ml of 5% of solution). The drug is administered kapelno intravenously in 350 — 400 ml of isotonic solution of sodium chloride or in 5% solution of glucose daily within 5 days with the subsequent 5-day interval. In total on a course of 15 injections. The course repeats 2 — 3 times a year. At decrease of the activity of D. Na is applied 2 EDTA is frequent in combination with corticosteroid drugs in a moderate dose.

At an apnoea in connection with the progressing damage of respiratory muscles the managed breath is applied (see. Artificial respiration , artificial ventilation of the lungs).

At children's age in connection with frequent development of the progressing tendinous and muscular contractures the great value is attached to early appointment to lay down. gymnastics and massage with gradual complication of a complex (see. Physiotherapy exercises, at children ; Massage, at children ).

The forecast

In some cases at timely purpose of adequate therapy is possible not only to reduce activity of process, but also to achieve transition of a disease to an inactive phase with satisfactory compensatory opportunities of motive function. Hron., steadily progressing process with the expressed dystrophy, widespread calcification and irreversible tendinous and muscular contractures results in disability.

In those cases when it is not possible to stop violently and zlokachestvenno the current process having extremely bystry, «galloping» current, the disease in several months can lead to a lethal outcome. Also progressing trophic disturbances of skin, mucous membranes of respiratory tracts can be a cause of death and went. - kish. a path with development of consecutive infection from the outcome in sepsis or developing of profuse gortanopishchevodny bleedings and peritonitis. The phenomena of a pulmonary heart which developed against the background of the irreversible progressing damage of respiratory muscles can be a proximate cause of death.

Prevention

For D.'s prevention is necessary for the children having hypersensitivity to a number of factors of both surrounding, and internal environment persistent sanitation of the centers hron, infections. At them especially carefully (taking into account the previous allergic reactions) all types of vaccination, use of antibiotics, streptocides, plasma transfusion, blood, administration of gamma-globulin shall be carried out.

To children, sick D., preventive inoculations, insolation, physiotherapeutic and thermal procedures are absolutely contraindicated (the last especially in an active phase of a disease). Operative measures shall be carried out in an inactive phase D. (remission not less than 2 years) against the background of corticosteroid therapy. At an intercurrent infection courses acetilsalicylic to - you and other salicylates are recommended; antibiotics are appointed only according to absolute indications. Active dispensary observation for children with D. with ensuring comprehensive examination and a close check behind implementation of the recommended actions is necessary.


Bibliography: Gausmanova-Petrusevich I. Muscular diseases, the lane with polsk., page 303, Warsaw, 1971; And shch e of N'ko M. M. and Dorogy A. N. About clinic and histopathological changes in a nervous system at an acute dermatomyositis, Zhurn, a neuropath, and psikhiat., t. 74, No. 2, page * 209, 1974, bibliogr.; Spear-in and T. N., etc. Exchange of some enzymes of skeletal muscles at a dermatomyositis (a histochemical research), Arkh. patol., t. 34, No. 3, page 46, 1974; Mikheyev V. V. Collagenoses in clinic of nervous diseases, M., 1971; Nesterov A. I. and Sigidinya. A. Klinika of collagenic diseases, page 429, M., 1966, bibliogr.; Whether with about l about in e in and A. P. and In и^ legs r an adova O. M. Izlechim an acute «idiopathic» dermatomyositis? Ter. arkh., t. 47, No. 4, page 118, 1975; Solovyova A. The item, M about and with e e in V. S. and Chel-ts about in V. V. Cardiovascular pathology at a dermatomyositis, Cardiology, t. 15, No. 5, page 52, 1975, bibliogr.; T are of e e in E. M. Collagenoses, page 267, M., 1965, bibliogr.; Banzhaf M. and. G r e 1 W. Dermatomyositische Syndrome und ihre immunosuppressive Therapie, Z. arztl. Fortbild., S. 37, 1973; Bohn-d o r f W. u. S with h of p 1 F. Rontgen-befunde bei Dermatomyositis, Fortschr. Rontgenstr., Bd 112, S. 531, 1970; Haas D. C. a. A r n a s o n B. G. W. Cell-mediated immunity in polymyositis, Arch. Neurol., v. 31, p. 192, 1974; Logan R. G. a. o. Polymyositis, Ann. intern. Med., v. 65, p. 996, 1966; Medsger T. A., Dawson W. N. a. M a s i A. T. The epidemiology of polymyositis, Amer. J. Med., v. 48, p. 715, 1970; S o k o 1 o f f M. C., G o 1 d b e r g L. S. a. Pearson C. M. Treatment of corticosteroid-resistant polymyositis with methotrexate, Lancet, v. 1, p. 14, 1971; Wagner E. L. Fall einer seltenen Muskelkrankheit, Arch, d. Heilk. (Lpz.), Bd 4, S. 282, 1863.

At children — Isaeva L. A. and Zhvaniya M. A. The principles of treatment of a dermatomyositis at children, Vopr. okhr. mat. also it is put., t. 15, No. 12, page 3, 1970, bibliogr.; M about z about l of e in with to and y Yu. V. Differential diagnosis of a dermatomyositis at children, Zhurn, a neuropath, and psikhiat., t. 74, No. K); page 1472, 1974, bibliogr.; Banker B. Q. and. V i with t about M. of Dermatomyositis (systemic angiopathy) of childhood, Medicine (Baltimore), v. 45, p. 261, 1966; B i t n u m S. o. Dermatomyositis, J. Pediat., v. 64, p. 101, 1964, bibliogr.; R o g e t J. e. a. La dermatomyo-site de l’enfant, etude de 22 observations, Pediatrie, t. 26, p. 471, 1971; Sullivan D. B. a. o. Prognosis in childhood dermatomyositis, J. Pediat., v. 80, p. 555, 1972,

H. G. Guseva, G. P. Kurtinite, A. A. Matulis; M. A. Zhvaniya, L. A. Isaev (ped.).

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