The Role of Endomyocardial Biopsy in the Diagnosis and Treatment of Churg-Strauss Syndrome

Abstract

Churg-Strauss syndrome (CSS) is a rare vasculitis characterized by the clinical triad of asthma, peripheral eosinophilia, and systemic vasculitis. Pulmonary symptoms occur commonly, but gastrointestinal, renal, cardiac, and musculoskeletal manifestations may also occur. Disease activity and expression can be variable, and progressive organ failure may occur in the absence of other clinical or biochemical evidence of disease activity. We report the case of a 73-year-old man who presented with signs and symptoms of an acute myocardial infarction, eosinophilia, a pulmonary infiltrate, and recent onset asthma. The cardiac catheterization was normal, but an endomyocardial biopsy specimen revealed eosinophilic myositis, granuloma formation, and small vessel vasculitis. A repeat endomyocardial biopsy 1 month after the initiation of high dose prednisone therapy showed no evidence of inflammation and no significant fibrosis. We suggest that a endomyocardial biopsy is a safe and useful tool in the diagnosis and monitoring of therapy in patients with CSS cardiac disease.