INTESTINE Vol.20 No.3(5)

特集名 消化管ポリポーシス─診断と治療の進歩
題名 クロンカイト・カナダ症候群の特徴
発刊年月 2016年 05月
著者 渡辺 知佳子 防衛医科大学校内科学講座
著者 三浦 総一郎 防衛医科大学校内科学講座
著者 穂苅 量太 防衛医科大学校内科学講座
【 要旨 】 クロンカイト・カナダ症候群(Cronkhite-Canada syndrome;CCS)は,胃・大腸に過誤腫性のポリポーシスが分布し,高率に蛋白漏出性胃腸症を合併する,おもに高齢者の疾患で,消化管外症状として,爪の萎縮・脱毛・色素沈着など,特徴的な皮膚症状を伴う.CCSポリープは囊胞状拡張腺管を特徴とする過誤腫性ポリープで,ポリープ介在粘膜にも組織学的変化があることが,他の過誤腫性ポリポーシス疾患との鑑別ポイントとなる.
Theme Gastrointestinal polyposis -- progress in the diagnosis and treatment
Title Cronkhite-Canada syndrome
Author Chikako Watanabe Department of Internal Medicine, National Defense Medical College
Author Soichiro Miura Department of Internal Medicine, National Defense Medical College
Author Ryota Hokari Department of Internal Medicine, National Defense Medical College
[ Summary ] Cronkhite-Canada syndrome (CCS) is a rare, acquired gastrointestinal polyposis syndrome with dermatologic manifestations. Patients with chronic active CCS polyposis have a higher risk of developing GI cancer than the general population. Appropriate medical therapy and endoscopic surveillance can alter the natural history of CCS. Oral corticosteroid therapy was confirmed to be effective for active CCS. Its benefits, including clinical improvement and polyp regression are usually apparent within 12 months. Adjunctive nutritional therapy may decrease the overall duration of corticosteroid therapy. Durable polyp regression (endoscopic remission) is associated with a markedly improved prognosis with decreased cancer risk. To prevent the risk of cancer development, surveillance endoscopy is recommended to assess mucosal disease activity and to remove adenomas and other premalignant mucosal lesions, although no long-term surveillance data are available.