Fatal Myocarditis in a Male with Systemic Sclerosis

Abstract

Cardiac involvements in systemic sclerosis have a poor outcome. We report here a case of 28 year-old-man with history of systemic sclerosis developing a fatal myocarditis.Initially patient has disabling joint manifestation. Systemic sclerosis is diagnosed after progressive development of cutaneous, pulmonary, and gastrointestinal manifestations. One year after systemic sclerosis diagnosis, our patient develops heart failure signs when he is already treated with methotrexate (15 mg/week) and coticosteroids (15 mg/day). Echocardiography show global hypokinesia with low left ventricular ejection fraction (32%). Natriuretic peptides and troponin levels are high. Anti-centromere and anti-SRP antibodies are positive. Corticosteroids dose is increased to 1 mg/kg/day, and methotrexate was given 25 mg/week without improvement. Cyclophosphamide was started but patient died within six months. Despite immunosuppressive therapy, myocarditis outcome is poor since patient was in heart failure at presentation, and has anti-SRP antibodies.