Dermatomyositis

Dermatomyositis (polymyositis) is a systemic disease of skeletal and smooth muscles and skin. Etiology. Assume a viral (Coxsacki B2) etiology of dermatomyositis. Provoking factors - cooling, insolation, trauma, pregnancy, drug intolerance. Tumor dermatomyositis is observed in 20 - 3% of patients.

Pathogenesis: a variety of immunopathological disorders. The predominance of women (2: 1), the two age peaks of the disease (transitional and climacteric periods) indicate the importance of neuroendocrine reactivity. Perhaps a family-genetic predisposition.

Symptoms, course. The disease begins acutely or subacute with muscle syndrome (myasthenia gravis, myalgia), arthralgia, fever, skin lesions, dense, widespread edema. Later the disease acquires a recurring course. The defeat of skeletal muscles is observed in all patients in the form of myalgia during movement and even at rest, with pressure and increasing muscle weakness. The muscles of the shoulder and pelvic girdle thicken, increase in volume, the active movements are significantly disturbed, the patient can not sit down alone, lift limbs, head off the pillow, keep her sitting or standing. With a significant spread of the process, patients are essentially completely immobilized, and in severe cases are in a state of complete prostration. The myasthenic syndrome does not decrease from taking prozerin and its analogues. The spread of the pathological process to the facial muscles leads to the masking of the face, the destruction of the pharyngeal muscles - to the dysphagia, and the intercostal and diaphragm - to the violation of breathing, a decrease in the vital capacity of the lungs, hypoventilation and frequent pneumonia. Oculomotor musculature can be affected with the development of diplopia, strabismus, bilateral ptosis of the eyelids, etc. In the early stages of the disease, the muscles are painful and often swollen, later on the site of dystrophy and myolysis of the muscle fibers develop myofibrosis, atrophy, contractures, and less often calcification. Calcium can also be observed in subcutaneous fat, especially in young people, and is easily detected by X-ray examination. With electromyography, the changes are nonspecific.

Lesions of the skin are diverse (erythematous, papular, bullous eruptions, purpura, telangiectasias, hyperkeratosis, hyper- and depigmentation, etc.). In a number of cases, rashes are accompanied by itching. Very characteristic and pathognomonic is the presence of periorbital edema with purpurnililovoy (heliotrope) erythema - the so-called dermatomyositis of eyeglasses.

Polyartralgia during movement and limitation of joint mobility up to the development of ankylosis are mainly due to muscle damage. There is a lesion of myocardium of inflammatory or dystrophic nature, which is manifested by persistent tachycardia and pulse lability, arterial hypotension, cardiac enlargement to the left, muffling of tones, systolic murmur at the apex. Electrocardiographic changes are expressed by a decrease in voltage, disturbance of excitability and conduction, depression of the ST segment, inversion of the T wave. When diffuse myocarditis develops a severe picture of heart failure. Y '! Of patients with Raynaud's syndrome. Lung infection is rarely associated with the underlying disease, it is more often due to a banal infection, to which patients are predisposed due to hypoventilation of the lungs, aspiration of food due to dysphagia. The gastrointestinal tract is involved in the process in almost half of the patients. As a rule, anorexia, abdominal pain, gastroenterocolitis symptoms, hypotension of the upper third of the esophagus are noted. Possible damage to the mucous membrane of the stomach and intestines with the formation of necrosis, with swelling and hemorrhages, up to severe gastrointestinal bleeding, perforation of the stomach, intestines; Sometimes there are symptoms that stimulate intestinal obstruction.

Laboratory data are nonspecific. Usually there is moderate leukocytosis with severe eosinophilia (up to 25 - 70%), persistent, although moderate increase in ESR, hypergammaglobulinemia. Biochemical blood and urine tests and muscle biopsies are of diagnostic importance, especially in chronic and subacute flow (thickening of muscle fibers with loss of transverse striation, fragmentation and dystrophy up to necrosis is observed, there is a significant cellular reaction - accumulation of lymphocytes, plasma cells, etc.) ).

In the acute course, there is a catastrophically increasing generalized lesion of the transverse striated musculature up to complete immobility, the phenomena of dysphagia and dysarthria. There is a general severe febrile-toxic state with a variety of skin rashes. The cause of the fatal outcome, which occurs in untreated cases after 3 to 6 months, is usually aspiration pneumonia or pulmonary heart failure due to severe lung or heart damage. Subacute flow is cyclical, but nevertheless steadily increase adynamia, lesions of the skin and internal organs. The chronic course of the disease is the most favorable form in which only single muscles are affected. Therefore, despite a significant number of exacerbations, the general condition of patients remains satisfactory and they remain permanently able to work. The exception is young people who develop extensive calcifications in the skin, subcutaneous tissue, muscles with the formation of persistent contractures and almost complete immobility of patients.

Treatment. In the presence of a malignant tumor, its removal leads to a stable remission. In acute and subacute flow, glucocorticoids are shown in large daily doses (the suppressive dose of prednisolone is 60 to 80 mg or more, if necessary). After reaching the effect, which does not occur soon, the dose of corticosteroids is reduced very slowly (by half a tablet every 7 - 1 0 days), to a maintenance dose against a background of deligil (0.25 g), plaquenyl (0.2 g) 1 tablet after dinner. Only with the development of persistent remission of glucocorticoids can be completely abolished. Triamcinolone is contraindicated because of its ability to enhance the myasthenic syndrome.

In therapy, non-steroidal anti-inflammatory drugs retain their importance at generally accepted doses, especially when doses of corticosteroids are lowered. In complex treatment, B vitamins and ascorbic acid are recommended. With pronounced fatigue of the muscles, proserine and its analogs in usual doses, ATP and cocarboxylase, and methandrostenolone (nerobol) are shown. In the development of calcitants recommended disodium salt ztilendiaminetetraakusnoy acid, able to form a complex with calcium ions and remove them from the body. Enter it intravenously in a 5% solution of glucose (per 500 ml of glucose 2-4 g of the drug) drip for 3 to 4 hours or fractional every 6 hours. The course of treatment is 3 to 6 days, after a 7-day break, the courses can be repeated. The drug is contraindicated in the defeat of the kidneys and liver. In the period of calming down acute phenomena, careful therapeutic gymnastics and massage of the extremities can be recommended. In a chronic stage with pronounced atrophies and contractures, persevering medical gymnastics and massage are shown.

At early treatment with adequate doses of corticosteroids in patients with acute dermatomyositis, a persistent recovery occurs.

In subacute flow, it is usually possible to achieve only a remission supported by glucocorticoids. In chronic dermatomyositis, the disease becomes undulating.