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文献考察１）：消化管amiloidosis，本邦集計29例．（表）
直腸穿孔をきたした透析アミロイドーシスの1例
Author：畝村泰樹(東京慈恵会医科大学 外科), 野尻卓也, 小川匡市, 三澤健之, 池内健二, 山崎洋次
Source：日本消化器外科学会雑誌(0386-9768)34巻2号 Page142-145(2001.02)
Abstract：71歳女.22年間の血液透析歴がある.以前より左腹部を中心とした腹痛が時にあり,大腸憩室および虚血性腸炎と診断されていた.1997年に大腸鏡下生検を施行し消化管アミロイドーシスと診断された.本年1月,下腹部痛のため緊急入院翌日に下部消化管穿孔と診断し緊急手術を施行した.直腸Ra後壁に径約1cmの穿孔があり,同部には径約3cmの硬便が存在していた.穿孔部を含む大腸部分切除および人工肛門造設術を施行した.粘膜下の小血管壁等にび漫性にβ2-microglobulin染色陽性のアミロイドの沈着を認めた.穿孔機序としては憩室炎,硬便による機械的圧迫の他に,アミロイドによる腸壁虚血の関与が示唆された.長期透析患者では,透析アミロイドーシスを消化管穿孔の危険因子として認識する必要がある．
追記：消化管へのアミロイドの沈着は続発性アミロイドーシスの70%，原発性アミロイドーシスの55%に発生するといわれる．粘膜の各層の小および中等大の動脈血管壁を中心に沈着し，特に粘膜下層の血管壁に最も高頻度に沈着する．沈着量が多くなると，血管壁以外に固有層の間質，粘膜筋板，固有筋層など壁内の随所で認められる．血管壁の肥厚・脆弱化あるいは血管内腔の狭小化・閉塞は出血，潰瘍や腸管壁の虚血を来す．そのため重篤な合併症として，消化管出血，梗塞および穿孔が起こる．
文献考察２）：消化管amiloidosisのCT所見：壁肥厚と腸管拡張が特徴だが，異常を認めない例も多い．
Abdom Imaging. 2000 Jan-Feb;25(1):38-44.
Amyloidosis of the alimentary canal: radiologic-pathologic correlation of CT findings.
Araoz PA, Batts KP, MacCarty RL.
BACKGROUND: The purpose of the study was to describe the computed tomographic (CT) findings of the alimentary canal and mesentery in amyloid infiltration of the gastrointestinal (GI) tract and to correlate the CT findings with histologic extent and distribution and with amyloid subtype. METHODS: Abdominal CT scans performed between 1988 and 1997 on patients with pathologically proven amyloidosis of the alimentary canal and mesentery. Histology was graded for extent of mucosal, submucosal, and muscularis propria involvement and for degree of interstitial and vascular distribution. CT findings were correlated with histologic extent, histologic distribution, and amyloid histochemical type. RESULTS: Twenty-three patients were included. Four (17%) had bowel wall thickening, which was associated with a higher submucosal extent and interstitial distribution than in patients with normal bowel by CT. Four (17%) patients had bowel wall dilatation without thickening, which was not associated with statistically significantly different histology than in patients with normal bowel by CT. There was no statistically significant correlation between CT findings and histochemical subtype. Mesenteric soft tissue infiltration was seen in two patients, and mesenteric adenopathy was seen in one patient. CONCLUSIONS: Normal bowel is a common abdominal CT finding in amyloidosis of the alimentary canal. When findings are present, GI wall thickening and/or bowel wall dilatation without wall thickening may be seen. Bowel wall thickening on CT correlates with submucosal extent and interstitial distribution of disease. Soft tissue infiltration and adenopathy are also occasionally seen.PMID: 10652919
３）Radiographics. 2004 Mar-Apr;24(2):405-16.
Amyloidosis: review and CT manifestations.
Georgiades CS, Neyman EG, Barish MA, Fishman EK.
Amyloidosis is a collection of disease entities that produce considerable morbidity and mortality and are increasing in prevalence. The imaging findings are problematically nonspecific and diverse. This lack of specificity is compounded by the fact that amyloidosis is strongly associated with and frequently coexists with many other chronic disease states that have their own imaging findings. Amyloidosis can involve any organ singly or in conjunction with other organs and can do so in the form of a focal, tumorlike lesion or an infiltrative process. In the proper clinical setting, that is, in a patient with chronic inflammatory disease and especially in a patient with multiple myeloma, amyloidosis should be considered as a possible cause of worsening or new symptoms or imaging findings. Occasionally, the radiologic findings may precede the clinical findings, thus providing the radiologist with the opportunity to contribute to the patient's care. However, to make a difference in patient care, the radiologist must be familiar with the diverse imaging findings of amyloidosis as well as the patient's clinical history, which could raise the suspicion of amyloidosis. Copyright RSNA, 2004．PMID: 15026589（full text）
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