非特異性多発性小腸潰瘍症/CEASの臨床病理学的特徴と鑑別疾患

Clinical and Pathological Characteristics and Screening of Nonspecific Multiple Ulcers of the Small Intestine and Chronic Enteropathy Associated with SLCO2A1 Gene（CEAS）

要旨●非特異性多発性小腸潰瘍症本邦報告例および自験例46文献62症例について集計し，臨床病理学的特徴の再確認を行った．診断年齢の平均は39.3歳で，推定発症年齢の平均は22.5歳であったが，50歳以上の比較的高齢発症と考えられる症例もあった．男女比は23：39で女性に多かった．姉妹発症は4組8例，両親がいとこ同士の結婚である症例が10例あった．貧血，低蛋白血症共におよそ9割の症例にみられ，便潜血陽性所見と合わせ，本症を疑ううえで重要な所見であった．多くの症例が回腸末端は保持されていた．潰瘍の形態は輪状，輪走，斜走，横走，帯状，全周性などの記載があった症例が79.3%と大半を占め，狭窄は81.0%にみられた．大多数がUl-IIまでの浅い潰瘍で，当院経験例では腸間膜対側で潰瘍の幅が広い傾向にあった．上記臨床的特徴をおおよそ満たし，さらに特徴的な小腸潰瘍（curved ulcer）の確認が可能であった報告例は12例であった．

　We collated data from 46 reports in Japan involving 62 patients with nonspecific multiple ulcers of the small intestine（including our patients）and reconfirmed the clinical and pathological characteristics of these nonspecific multiple ulcers. The average age of the patients at the time of diagnosis was 39.3 years, and although the estimated average age at the time of onset was 22.5 years, a late onset age of ≧50 years was also suspected in some patients. There were more female patients than male patients（male：female ratio, 23：39）, four pairs of sisters（eight patients）, and 10 patients whose parents had married their cousins. Both anemia and hypoproteinemia were observed in 90% of the patients, which appear to be a significant diagnostic finding for this condition along with the presence of occult blood in feces. Many patients also don't have lesson in the terminal ileum. Regarding the shape of the ulcer, the ulcer was ring-shaped, circular, oblique, horizontal, belt-shaped, or circumferential in 79.3% of the patients and stenosis was observed in 81.0% of the patients. A majority of patients had shallow ulceration of up to UI-II, and there was a tendency for the width of the ulcer to be wider on the antimesenteric side among our patients. Twelve patients mostly satisfied the above clinical criteria and also exhibited a curved ulcer that is characteristic of this condition.