Antisynthetases syndrome associated with right heart failure

Abstract

Cardiac involvement is a complication of end stage polymyositis with left heart insufficiency reported to be the most frequent manifestation. We here describe an unusual clinical presentation of antisynthetases syndrome, beginning with right-sided cardiomyopathy associated with right heart failure. A 26 year-old Caucasian male experienced a 6-month clinical course of polyarthritis, fever, sweats, and myalgia. Laboratory studies showed elevated C reactive protein, elevated sedimentation rate, and myolysis associated with anti SSA and anti JO1 antibodies. Electromyography showed a myopathic pattern. Muscle biopsy confirmed the diagnosis of polymyositis. Chest X ray, chest scan, and cardiac echography were normal. One week after hospital admission, the patient developed acute right heart insufficiency, and magnetic resonance imaging showed a right ventricular myocarditis with myocardial inflammatory thickening. Treatment with corticosteroids rapidly improved both symptoms and biological abnormalities.