INTESTINE Vol.23 No.6(4-4)


特集名 全身性疾患における腸管病変─腸管ベーチェット病とその鑑別疾患
題名 腸管ベーチェット病の鑑別疾患 (4) Chronic enteropathy associated with SLCO2A1 gene (CEAS) と cryptogenic multifocal ulcerous stenosing enteritis (CMUSE)
発刊年月 2019年 11月
著者 漆久保 順 岩手医科大学内科学講座消化器内科消化管分野
著者 梁井 俊一 岩手医科大学内科学講座消化器内科消化管分野
著者 松本 主之 岩手医科大学内科学講座消化器内科消化管分野
【 要旨 】 非特異性多発性小腸潰瘍症の原因遺伝子として,プロスタグランジン輸送体をコードするSLCO2A1が同定され,本症の新名称としてchronic enteropathy associated with SLCO2A1 gene(CEAS)が提唱されている.一方,欧州ではcryptogenic multifocal ulcerous stenosing enteritis(CMUSE)と呼ばれる小腸潰瘍症の記載があり,主たる病態がプロスタグランジンの合成障害であることが確認されている.これら二つの疾患を合わせたプロスタグランジン関連腸症の概念が提唱されており,希少ではあるが腸管ベーチェット病を含む慢性小腸潰瘍症の重要な鑑別疾患である.
Theme Systemic disease-associated intestinal involvement -- Intestinal Behçet's disease and its differential diagnosis
Title Chronic enteropathy associated with SLCO2A1 gene (CEAS) and cryptogenic multifocal ulcerous stenosing enteritis (CMUSE)
Author Jun Urushikubo Division of Gastroenterology, Department of Internal Medicine, Iwate Medical University
Author Shunichi Yanai Division of Gastroenterology, Department of Internal Medicine, Iwate Medical University
Author Takayuki Matsumoto Division of Gastroenterology, Department of Internal Medicine, Iwate Medical University
[ Summary ] Chronic nonspecific multiple ulcers of the small intestine (CNSU) have been reported as an enteropathy characterized by multiple small intestinal ulcers of nonspecific histology. Recently, CNSU has been found to be caused by homozygous or compound heterozygous mutation of the SLCO2A1 gene, which encodes a prostaglandin transporter. Therefore, the disease has been referred to as chronic enteropathy associated with SLCO2A1 gene (CEAS). There has also been a description of an enteropathy, referred to as cryptogenic multifocal ulcerative stenosing enteritis (CMUSE), in which mutations of cPLA2α pathognomonic event. These findings suggest that there may be a clinical entity of enteropathy associated with prostaglandins in humans. These diseases should be considered as being differential diagnoses of intestinal Behçet's disease and simple ulcer of the small intestine.
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