Department of Laboratory Medicine and Pathology, Toronto General Hospital, University of Toronto, 200 Elizabeth Street, Toronto, ON, M5G 2C4, Canada.
Dig Dis Sci. 2013 Sep;58(9):2608-14. doi: 10.1007/s10620-013-2697-7. Epub 2013 May 14.
Inflammatory bowel disease (IBD) associated with primary sclerosing cholangitis (PSC) is reported to be mild and prone to right-side predominance with rectal sparing. However, no dedicated studies evaluating patterns of presentation of liver disease with respect to IBD are available.
We performed a detailed histological examination of the colonic biopsies in the context of PSC, identifying 97 patients [89 with ulcerative colitis and ten with Crohn's disease (CD)] stratified into two groups, based on their initial disease presentation: hepatic/biliary (group 1-PSC-IBD; n=56) versus colonic (group 2-IBD-PSC; n=41).
Inflammatory bowel disease that preceded PSC had a tendency to have a "pan-colitis" distribution; this group included all patients with CD. Inflammatory bowel disease diagnosis that followed PSC presentation was more likely to be right-sided, sparing the descending, sigmoid and rectal regions (p=0.002). In both groups, colitis was mild with focal deep plasmacytosis and occasional mild cryptitis. Active cryptitis with crypt abscesses, surface erosion and ulceration were not identified in any of the patients.
Colitis associated with PSC shows mild disease activity and the colitis pattern is associated with disease presentation, i.e. colitis preceding PSC (IBD-PSC cohort) typically have a pancolitic distribution, while colitis following PSC (PSC-IBD cohort) demonstrates right-sided predominance. Awareness by pathologists and clinicians of these patterns of inflammatory bowel disease is important and of use in directing appropriate investigations for patients.
据报道，与原发性硬化性胆管炎（PSC）相关的炎症性肠病（IBD）较轻，且易于右半结肠受累而直肠不受累。然而，目前尚无专门研究评估 IBD 患者肝脏疾病表现模式。
我们对 PSC 患者的结肠活检标本进行了详细的组织学检查，确定了 97 例患者[89 例溃疡性结肠炎和 10 例克罗恩病（CD）]，根据其初始疾病表现分为两组：肝/胆（PSC-IBD 组，n=56）和结肠（IBD-PSC 组，n=41）。
IBD 先于 PSC 出现时倾向于呈“全结肠炎”分布，这组包括所有 CD 患者。PSC 后出现的 IBD 更可能为右半结肠受累，降结肠、乙状结肠和直肠不受累（p=0.002）。在两组中，结肠炎均为轻度，表现为局灶性浆细胞浸润和偶尔轻度隐窝炎。任何患者均未发现活动性隐窝炎伴隐窝脓肿、黏膜表面糜烂和溃疡。
PSC 相关结肠炎活动度较轻，且结肠炎的分布与疾病表现相关，即先于 PSC 的结肠炎（IBD-PSC 组）通常呈全结肠炎分布，而继于 PSC 的结肠炎（PSC-IBD 组）则表现为右半结肠优势分布。病理科医生和临床医生了解这些 IBD 表现模式很重要，有助于指导对患者进行适当的检查。
