高度の心機能低下を呈したChurg-Strauss症候群の1例

Abstract

A 46-year old male with Churg-Strauss syndrome (CSS) was admitted to our hospital because of congestive heart failure. Two years before admitsson, he developed nocturnal episodes of bronchial asthma for 2 weeks. One year before admission, purpura appeared on bilateral lower extremities. He noticed leg edema and exertional dyspnea one month before admission. Blood examination revealed increased eosinophil count (1,491/ μl). Serological test disclosed a marked increase in serum IgE. A chest X ray film showed increase of the cardiac silhouette and bilateral pleural effusion. Echocardiogram revealed an enlargement of the left ventricular dimensions and reduced ejection fraction (EF 31%). The first myocardial biopsy revealed moderate hypertrophy of cardiomyocytes and lymphocyte infiltration. These findings were compatible with myocarditis. Bronchial asthna, eosinophilia, and snymptom of angitis such as myocarditis and skin lesion con- firmed a diagnosis of CSS. Then he was given prednisolone, 20 mg daily. The second myocardial biopsy findings 11 days after prednisolone therapy showed improvement of the inflammation. Three months after corticosteroid administration, echocardiogram showed improvement of the ejection fraction (EF 55%). We report a case of CSS complicated with severe impairment of LV function. During corticosteroid therapy, histological improvement preceded the improvement of left ventricular systolic function.