好酸球性副鼻腔炎術後に発症したＣｈｕｒｇ‐Ｓｔｒａｕｓｓ症候群

Abstract

Churg-Strauss syndrome (CSS) is a systemic vasculitic disorder affecting small blood vessels of the upper and lower respiratory tract. Subjects with CSS often have otorhinolaryngological manifestations, but clinical characteristics have not been detailed. We report a case of CSS associated with chronic hypertrophic eosinophilic sinusitis. A 66-year-old man treated for bronchial asthma and seen for a 1-month history of nasal obstruction was found in physical and radiological tests to have chronic pansinusitis with nasal polyposis. Clinical laboratory tests showed marked peripheral blood eosinophilia (35%, 3080/μl). When conservative treatment was ineffective, the patient underwent endoscopic transnasal polypotomy. Surgical specimens histopathologically showed conspicuous eosinophilic infiltration consistent with chronic hypertrophic eosinophilic sinusitis. His postoperative clinical course was uneventful. Three months later, he developed purpura of the extremities and general fatigue with deteriorated of peripheral blood eosinophilia (58%, 11020/μl). The defintive diagnosis was CSS. He was administered steroid pulse therapy-methylprednisolone of 1,000 mg/day for 3 days followed by tapered doses-combined with transoral cyclophosphamide. Following complete remission, he remains currently free of theh disease. Chronic hypertrophic eosinophilic sinusitis morbidity has been increasing in the last decade, and diagnosticians must be aware that this prevalent sinusitis may be a manifestation of a rare condition such as CSS.