心膜心筋炎を合併したChurg-Strauss症候群の1例

Report on a Case of Churg-Strauss Syndrome with Pericarditis and Myocarditis

　症例は40歳，女性．39歳時に喘息が出現．40歳になり皮疹と38℃台の発熱が持続し関節痛と浮腫および体重増加を認め入院．入院時心拡大（CTR58％）と，白血球増加（13,310／μ1）および好酸球増多（41％），Ig-E高値を示した．腎生検で糸球体にフィブリノイド壊死を伴う血管炎が認められた．喘息，好酸球増多，血管炎症候群の3徴よりChurg-Strauss症候群と診断した．心臓超音波検査で多量の心嚢液貯留，びまん性心筋腫大，心筋エコー輝度増強，心収縮能および拡張能の障害がみられた．心筋生検では間質に巣状の線維化と軽度のリンパ球浸潤が示され，合併症として心膜心筋炎の存在が示唆された．ステロイド療法により全身症状や心病変は著明に改善した．Churg-Strauss症候群の心筋障害の原因として，血管炎による微小心筋虚血や好酸球自体による直接的心筋障害が推察された．Churg-Strauss症候群で生前の心筋生検により心膜心筋炎の合併を診断し得た症例は極めて稀であり報告した．

A 40-year-old woman who had been suffering from asthma for a year was admitted to our hospital because of skin eruptions, low-grade fever, arthralgia and edema. Chest roentgenograms showed an enlarged car-diac silhouette. At that time the white blood cell count was 13,310 with 41% eosinophils, and elevated serum IgE was found. Renal biopsy specimens rhowed fibrinoid necronsis and crescents of glomeruli. She was diagnosed as having Churg-Strauss syndrome on the basis of her history of asthma, hypereosinophilia, and renal biopsy findings. Echocardiogram revealed the presence of a large effusion, hypertrophy and, in addi-tion, showed diffuse hypokinesis of the left ventricle. Based on a diagnosis of Churg-Strauss syndrome, the patient was treated initially with 40 mg of prednisolone daily. She responded well to steroid therapy, her symp-toms and pericardial effusion resolved and left ventricular function was improved. Six months after beginning steroid therapy, cardiac catheterization and myocardial biopsy was performed. The results of coro-nary angiography were normal. There was no regional left ventricular asynergy, but left ventricular ejection fraction was slightly reduced (EF56%). A myocardial biopsy specimen showed focal fibrosis and mild infiltration of lymphocytes.

This case of Churg-Strauss syndrome developed into pericarditis and myocarditis. It was suggested that the cause of the cardiac involvement was that microangiitis had induced myocardial necrosis and/or direct myocar-dial injury by eosinophils.