title: 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.
authors: Rheumatology Service, Cochin Hospital, Paris-Descartes University, AP-HP, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France.
authors: Latifa
authors: L
authors: Tahiri
authors: Sandra
authors: S
authors: Guignard
authors: Patricia
authors: P
authors: Pinto
authors: Muriel
authors: M
authors: Duclos
authors: Maxime
authors: M
authors: Dougados
mesh_terms: D000328:Adult; D000888:Antibodies, Anti-Idiotypic / Q000097:blood*; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D001706:Biopsy; D004576:Electromyography; D006333:Heart Failure / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D008025:Ligases / Q000276:immunology*; D008297:Male; D018482:Muscle, Skeletal / Q000473:pathology / Q000503:physiopathology; D017285:Polymyositis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D013902:Radiography, Thoracic; D000069283:Rituximab; D013577:Syndrome; D016896:Treatment Outcome
chemical_list: D000888:Antibodies, Anti-Idiotypic; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab; D008025:Ligases