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DEVELOPMENT OF CRITICAL DIGITAL ISCHEMIA IN A PATIENT WITH DERMATOMYOSITIS AND ANTISYNTHETASE SYNDROME: CLINICAL CASE
Tripolka S.A.1, Golovach I.Yu.2
Summary. Description of a clinical case of diagnosis of antisynthetic syndrome in a young patient is presented; the above-mentioned syndrome manifested itself in the severe Raynaud’s рhenomenon, the development of occlusive vasculopathy, and critical digital ischemia that dominated the clinical picture. The disease debuted with skin lesion (heliotrope erythema, erythema rash on the skin) and fever, later joined by joint syndrome, muscles pain; lung damage was diagnosed (pulmonitis and pulmonary infiltrates). Immunological testing revealed antibodies to histidyl-tRNA synthetase (anti-Jo-1). The patient was diagnosed with 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 made it possible to reduce the activity of dermatomyositis, clinical and laboratory manifestations of myositis, and lung damage. However, the rapid progression of vasculopathy with the development of acute digital ischemia led to the development of dry gangrene and the loss of soft tissues.
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