RETROSPECTIVE OBSERVATION OF A PATIENT WITH DERMATOMOSITIS AND ANTISYNTHETASE SYNDROME: POLYMORPHISM OF CLINICAL MANIFESTATIONS AND SEARCH OF TREATMENT METHODS
Summary. A description of the clinical case of a three-year observation of the antisynthetase syndrome in a young patient is presented, which was clinically manifested by Raynaud’s phenomenon dominant clinical picture, the development of occlusive vasculopathy, critical digital ischemia, and interstitial lung disease.The disease debuted with a skin lesion (heliotrope erythema, erythema rash on the skin) and fever, later joined the joint syndrome, muscles pain, diagnosed lung damage (pulmonitis and pulmonary infiltrates). Immunological testing revealed antibodies to histidyl-tRNA synthetase (anti-Jo-1). The patient was exposed to an antisynthetase syndrome based on this symptom complex. Three months after the appearance of the first skin symptoms, severe vasculopathy developed with the development of critical digital ischemia of the fingers, later the area of the left heel. The use of combined pulse therapy with cyclophosphamide and methylprednisolone, treatment with intravenous immunoglobulin allowed to reduce the activity of dermatomyositis, clinical and laboratory appearance of myositis, lung damage, stop the progression of vasculopathy and achieve clinical remission. However, after the termination of an unplanned pregnancy, the patient again had an outbreak of disease activity with an emphasis on lung damage. Given the lack of effect of antibacterial, combined pulse therapy, therapy with intravenous immunoglobulin, it was decided to use rituximab, against which it was possible to achieve stabilization of the patient’s condition.
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