消化管穿孔を来したChurg-Strauss症候群（アレルギー性肉芽腫性血管炎）の1例

Churg-Strauss Syndrome Presenting with Jejunal Perforations, Report of a Case

要旨　患者は62歳，女性．33歳時に気管支喘息，59歳時に肝硬変が出現．60歳から喘息発作が頻発しステロイドを服用したがコントロール不良．1994年4月，喘息重積発作に対するステロイド治療後，小腸穿孔を生じ，上部小腸を80cm切除した．切除標本にはUl-II～IVの多発性潰瘍と3か所に穿孔を認め，病理組織学的には血管周囲に肉芽腫様変化と好酸球浸潤を伴う血管炎がみられた．また，その後の消化管検査で，胃，小腸，大腸に浅い小～地図状の潰瘍を認めた．更に末梢血好酸球増多と末梢神経炎を認め，Churg-Strauss症候群と診断しta. predonisolone 60mgから治療を開始し，症状，末梢血の好酸球増多，多発潰瘍は改善したが，肝障害が進行し死亡した．剖検では，胃・小腸・大腸そして肝・胆の主として小動脈と静脈に瘢痕性の全層性血管炎を確認し，Churg-Strauss症候群に合致する所見であった．

　A 62-year-old woman was admitted because of abdominal pain and gastrointestinal bleeding following corticosteroid therapy for asthma exacerbation in April 1994. She had a history of bronchial asthma at the age of 33 and liver cirrhosis at the age of 59. At the age of 60, she started to take corticosteroids to manage severe asthma. On admission, perforation of the jejunum was recognized and partial resection of the jejunum was performed. Multiple ulcers with three perforations were found in the resected specimen. Additionally, radiographic and endoscopic examinations revealed multiple shallow ulcers in the small bowel, colon and stomach. Histology of the resected small bowel specimen demonstrated vasculitis accompanying granulomatous change and eosinophil infiltration around the blood vessels. On the basis of clinical and histologic features, the patient was diagnosed as having Churg-Strauss syndrome. Treatment with 60 mg prednisolone daily was effective against her symptoms, eosinophilia and gastrointestinal multiple ulcers. Nevertheless, despite this good response, she developed liver injury which led to death. At autopsy, cicatrical vasculitis was found to involve the whole layer of arteries and veins in the stomach, small bowel, colon, liver and gallbladder, a condition indicative of Churg-Strauss syndrome.