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Stomach and Intestine(Tokyo) Volume 49, Issue 3 （March 2014）

胃と腸 49巻3号（2014年3月発行）

Japanese English

今月の主題消化管アミロイドーシスを見直す

主題研究

炎症性腸疾患に伴う二次性アミロイドーシスの臨床的検討―Crohn病症例の合併率，形態診断，長期経過を中心に Secondary Amyloidosis Associated with Inflammatory Bowel Disease 平井郁仁 ¹ , 高田康道 ¹ , 佐藤祐邦 ² , 高橋晴彦 ³ , 矢野豊 ¹ , 高津典孝 ¹ , 松井敏幸 ¹ , 今村健太郎 ¹ , 池田圭祐 ⁴ , 岩下明德 ⁴ , 宮岡正喜 ³ Fumihito Hirai ¹ , Yasumichi Takada ¹ , Yuho Satoh ² , Haruhiko Takahashi ³ , Yutaka Yano ¹ , Noritaka Takatsu ¹ , Toshiyuki Matsui ¹ , Kentaro Imamura ¹ , Keisuke Ikeda ⁴ , Akinori Iwashita ⁴ , Masaki Miyaoka ³ ¹福岡大学筑紫病院消化器内科 ²田主丸中央病院消化器内科 ³済生会二日市病院内科 ⁴福岡大学筑紫病院病理部 ¹Department of Gastroenterology, Fukuoka University Chikushi Hospital, Chikushino, Japan ²Department of Gastroenterology, Tanushimaru Central Hospital, Kurume, Japan ³Department of Internal Medicine, Saiseikai Futsukaichi Hospital, Chikushino, Japan ⁴Department of Pathology, Fukuoka University Chikushi Hospital, Chikushino, Japan キーワード：炎症性腸疾患 , 二次性アミロイドーシス , Crohn病 , 潰瘍性大腸炎 , Behçet病 Keyword: 炎症性腸疾患 , 二次性アミロイドーシス , Crohn病 , 潰瘍性大腸炎 , Behçet病 pp.345-357

発行日 2014年3月25日 Published Date 2014/3/25

DOI https://doi.org/10.11477/mf.1403114096

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要旨　当科において診療したCrohn病（CD），潰瘍性大腸炎（UC），Behçet病患者（BD）を対象とし，生検および手術標本における病理組織学的所見の結果から，二次性アミロイドーシス（SA）合併の有無を検討した．CDに関してはデータベースを用いて患者数，臨床像，臨床経過および長期予後について解析し，さらにSA合併症例の詳細とSAの合併有無別の比較検討を加えた．IBD患者におけるSA合併率は1.1％（CD：1.6％，UC：0.3％，BD：3.4％）であった．CD症例においては，（1）診断では十二指腸病変の認識と生検が有用であること，（2）SA合併率は1.6％で，近年やや下降傾向であること，（3）累積生存率はSA診断後50か月で79.5％，131か月で53.0％と生命予後が不良であること，（4）SA合併例は悪性疾患の既往の頻度が15.4％で非合併例より有意に高かったことが明らかとなった．

　[Background and Aims] Amyloidosis often leads to systematic organ dysfunction and is considered a life-threatening disorder. However, little is known regarding secondary amyloidosis（SA）associated with inflammatory bowel disease（IBD）. The aim of this study was to evaluate the incidence, clinical course, and prognosis of SA in a large cohort of IBD patients at a referral center.

　[Methods] We conducted a cohort study using the IBD database at our department. We investigated the incidence, clinical features, long-term outcome and prognosis of patients with SA associated IBD.

　[Results] Among a total of 1,450 IBD patients（Crohn's disease, CD : 800, Ulcerative colitis, UC : 621, Behçet disease, BD : 29）, 16 cases of SA were identified. Thirteen of the 16 cases had CD, 2 had UC, and 1 had BD. Therefore, the incidences of SA for IBD, CD, UC, and BD were 1.1％, 1.6％, 0.3％, and 3.4％, respectively. In CD patients, the cumulative incidence of SA was 0.7％ at 10, 2.9％ at 20, and 9.3％ at 30 years after onset. Specific endoscopic findings of SA, such as rough mucosa, granularity, abnormalities of the Kerckring fold, and edema, were frequently observed in the duodenum（10/12, 83.3％）and small bowel（6/13, 46.2％）. Biopsy specimens taken from the stomach（9/10, 90.0％）and duodenum（11/11, 90.0％）of CD patients with SA showed high detection rates of AA type amyloid. The cumulative survival rates of CD patients with SA were 9.5％ at 50 months, 66.3％ at 94months, and 53.0％ at 131 months after onset. History of malignant disease was more likely to be observed in CD patients with SA（2/11, 15.4％）than in those without SA（17/787, 2.2％）.

　[Conclusion] In our large cohort, incidence of SA in IBD patients was not high. However, when considering the poor prognosis of SA-complicated CD, systematization including gastroduodenoscopy and biopsy for early diagnosis of SA is mandatory. For IBD patients who have risk factors, -such as long disease duration, relapsing course, and history of malignant disease, gastroenterologists should take the complication of SA into consideration.