Mixed connective tissue disease
Mixed connective tissue disease (Sharpe's syndrome) is characterized by a combination of individual signs of systemic scleroderma, polymyositis and systemic lupus erythematosus.
Etiology is unknown, and the peculiarity of pathogenesis is a peculiar impairment of immunity, manifested by the circulation of antibodies to RNP, immune complexes and changes in immunoregulatory functions of T-lymphocytes. A feature of pathogenesis is also the development of proliferative processes in the intima and (or) in the media of large vessels with signs of Raynaud's syndrome and pulmonary hypertension. Mostly women are ill. .
Symptoms, course. A combination of Raynaud's syndrome accompanied by dense edema, polyarthritis and polymyositis, hypotension of the esophagus and disturbance of the function of external respiration is characteristic. Reynaud's syndrome is not only the most frequent, but often early symptom of the disease, occurring without the tendency to severe ischemic necrotic changes in the terminal phalanges of the hands. Often it is accompanied by a dense edema of the brushes, but without the outcome of sclerodactyly. Very often recurrent polyarthritis with development in 1/3 of patients with erosive changes and in a number of patients with rheumatoid nodules with the advent of rheumatoid factor. Muscle syndrome in the form of myalgias, muscular densities and weakness of proximal muscle groups was noted in 2/3 of the patients. Of visceral lesions, hypotension of the esophagus, development of primary pulmonary hypertension and, more rarely, diffuse glomerulonephritis. The nervous system is seldom affected, but severe aseptic meningitis, trigemini, and others can occur.
A number of patients develop anemia, leukopenia, and also there is hypergammaglobulinemia and the presence of high-titer antibodies to RNP, which has diagnostic value. The disease flows more benignly than each of these separately. The prognosis is unfavorable with the development of pulmonary hypertension. .
Treatment. Glucocorticosteroids are prescribed in medium and small doses - from 30 to 10 mg per day and only in severe polymyositis to 60 mg or more. The intake of hormones in the maintenance dose lasts for years. If steroids are ineffective, azathioprine can be additionally recommended 1 mg / kg for a long time.
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