Primary sclerosing cholangitis is associated with a distinct phenotype of inflammatory bowel disease

Primary sclerosing cholangitis is associated with a distinct phenotype of inflammatory bowel disease
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DOI:
10.1002/ibd.22938
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发表时间:
2012-12-01
影响因子:
4.9
通讯作者:
Ponsioen, Cyriel Y.
Ponsioen, Cyriel Y.
中科院分区:
医学2区
文献类型:
--
作者:
Boonstra, Kirsten;van Erpecum, Karel J.;Ponsioen, Cyriel Y.

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背景:原发性硬化性胆管炎(PSC)与炎症性肠病(IBD)密切相关。本研究的目的是使用内窥镜和组织病理学标准,在一个基于表型良好的人群的大型PSC队列中评估与PSC相关的IBD表型。方法:在荷兰地理定义地区的39家医院中,识别并确定了符合既定标准的PSC病例。根据蒙特利尔分类记录IBD位置。由于该分类未考虑节段性炎症、回肠炎或直肠保留,因此在80例病例和80例年龄和性别匹配的IBD对照中进行了额外的亚组分析,审查了2000年至2010年期间提交的所有内窥镜检查和病理学报告。在总共579名PSC患者中,共有380名(66%)合并IBD,主要是溃疡性结肠炎(UC)(75%)。总体而言,207例(83%)PSC-UC患者患有全结肠炎,32例(13%)左侧结肠炎,9例(4%)仅直肠炎。70例(95%)PSC-克罗恩病(CD)患者仅发生(回肠)结肠炎和4例(5%)回肠炎。在亚组分析中,确定了53例(66%)PSC-UC患者、24例(30%)PSC-CD患者和3例(4%)PSC-IBD-U患者。50例(94%)PSC-UC患者有全结肠炎,而32例(62%)匹配的UC患者有全结肠炎(P < 0.001)。在16例(31%)UC对照和1例PSC-UC患者中观察到左侧结肠炎(P < 0.001)。反冲洗性回肠炎和直肠保留是罕见的发现(
Background: Primary sclerosing cholangitis (PSC) is strongly associated with inflammatory bowel disease (IBD). The aim of this study was to assess the IBD phenotype associated with PSC in a large well-phenotyped population-based PSC cohort using endoscopic and histopathologic criteria.Methods: PSC cases were identified and ascertained, fulfilling well-established criteria, in 39 hospitals in a geographically defined region of The Netherlands. IBD location was recorded according to the Montreal Classification. As this classification does not consider segmental inflammation, backwash ileitis, or rectal sparing, an additional subgroup analysis was performed in 80 cases and 80 age- and sex-matched IBD controls, reviewing all endoscopy and pathology reports filed between 2000 and 2010.Results: In all, 380 (66%) of a total of 579 PSC patients had coexistent IBD, mainly ulcerative colitis (UC) (75%). Overall, 207 (83%) of the PSC-UC patients had a pancolitis, 32 (13%) a left-sided colitis, and 9 (4%) a proctitis only. Seventy (95%) PSC-Crohn's disease (CD) patients had an (ileo)colitis and four (5%) ileitis only. In the subgroup analysis 53 (66%) PSC-UC patients were identified, 24 (30%) PSC-CD patients, and three (4%) PSC-IBD-U patients. Fifty (94%) PSC-UC patients had a pancolitis, compared with 32 (62%) matched UC patients (P < 0.001). Left-sided colitis was seen in 16 (31%) UC controls and in one PSC-UC patient (P < 0.001). Backwash ileitis and rectal sparing were rare findings (