Churg-Strauss syndrome with myopericardial involvement

Churg-Strauss syndrome (CSS) is a small-vessel necrotizing vasculitis typically characterized by asthma, lung infiltrates, extravascular necrotizing granulomas and hypereosinophilia. Cardiac disease is a major contributor to disease-related death in CSS. We describe a 38-year-old man with late-onset asthma, allergic rhinosinusitis, and high extravascular and peripheral blood eosinophilia, who presented with migratory pulmonary infiltrates and acute myopericarditis. Antineutrophilic cytoplasmic antibodies (ANCA) were negative. Early therapy with medium-dose methylprednisolone led to resolution of the pericardial effusion and significant clinical improvement. In the present case report, the importance of early recognition of CSS in patients with asthma and peripheral eosinophilia is discussed. Cardiac magnetic resonance imaging, besides electro- and echocardiography, may be helpful in early detection of cardiac involvement in CSS, enabling appropriate treatment aimed to prevent further disease progression and potentially fatal consequences.