Churg-Strauss Syndrome Presenting with Pericarditis

Abstract

Background: Churg-Strauss syndrome (CSS) is a rare systemic vasculitis, that affects medium-sized vessels, involves multiple organs and in the majority of cases is related to asthma and eosinophilia. We report the case of a young woman with acute pericarditis.

Case History: A 22-year-old woman with a history of allergic rhinitis, asthma and nasal polyposis was admitted at our emergency department for chest pain and bilateral lower extremity weakness with paresthesia. Laboratory tests showed eosinophilia, elevated C-reactive protein (112 mg/dl) and elevated biochemical markers of myocardial injury (troponin 0.46 ng/ml). The electrocardiogram was negative. Transthoracic echocardiography revealed a small pericardial effusion with no other abnormalities. Hearth magnetic resonance imaging was permormed and confirmed a diagnosis of pericarditis. Immunologic study showed ANA and ANCA positive, in particular ANA 1:100, p-ANCA 134 U/l, and elevated IgE. Electromyography revealed sensory-motor symmetric axonal neuropathy. She had also arthritis of the right wrist. This presentation led to a diagnosis of CSS and appropriate therapy with corticosteroid, colchicine and mepolizumab resulted in a remission of disease activity.