Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark.
Department of Gastroenterology, Herlev Hospital, University of Copenhagen, Herlev, Denmark.
Liver Int. 2018 Mar;38(3):532-541. doi: 10.1111/liv.13548. Epub 2017 Sep 5.
Inflammatory bowel disease (IBD) may be complicated by primary sclerosing cholangitis (PSC). We aimed to assess the characteristics of Danish PSC-IBD patients and to compare their prognosis with IBD patients without PSC.
A retrospective nationwide population-based cohort of 257 PSC-IBD patients was assessed through Danish national registries and manual scrutiny of patient files.
For all PSC-IBD patients diagnosed after 1976 (n = 222) and 8231 IBD controls (ie, without PSC), the cumulative probability of resective surgery, liver transplantation, cancer, and survival from 1977 through 2011 was estimated and compared by log-rank test and Cox regression. PSC-IBD patients primarily had ulcerative colitis (UC) (72%), were diagnosed in young adulthood (median age at IBD diagnosis, 23 years), and 9% were smokers. Among PSC-UC patients 78% had pancolitis at diagnosis. Among patients with PSC and Crohn's disease (CD) 91% had colonic involvement. The PSC-IBD patients had a significantly higher probability of receiving resective surgery (HR; 2.13, 95% CI: 1.50-3.03); of developing colorectal cancer (CRC) (HR; 21.4, 95% CI: 9.6-47.6), of cholangiocarcinoma (HR; 190, 95% CI: 54.8-660), and of dying (HR; 4.39, 95% CI: 3.22-6.00) as compared to non-PSC-IBD controls. The 25-year cumulative risk of liver transplantation was high (53%).
This unselected population-based study shows that PSC-IBD patients not only have an extensive phenotype of IBD, they are also treated more intensively than other patients with IBD. However, the prognosis remains poor and without any apparent improvement over calendar time.
炎症性肠病（IBD）可能并发原发性硬化性胆管炎（PSC）。本研究旨在评估丹麦PSC-IBD 患者的特征，并将其与无 PSC 的 IBD 患者的预后进行比较。
通过丹麦国家登记处和患者病历的人工审查，对 1976 年后诊断的 257 例 PSC-IBD 患者进行回顾性全国性人群队列评估。
对于所有 1976 年后诊断的 PSC-IBD 患者（n=222）和 8231 例 IBD 对照患者（即无 PSC），通过对数秩检验和 Cox 回归比较并估计了 1977 年至 2011 年期间接受切除术、肝移植、癌症和生存的累积概率。PSC-IBD 患者主要患有溃疡性结肠炎（UC）（72%），在成年早期被诊断（IBD 诊断时的中位年龄为 23 岁），9%为吸烟者。在 PSC-UC 患者中，78%在诊断时患有全结肠炎。在 PSC 和克罗恩病（CD）患者中，91%有结肠受累。PSC-IBD 患者接受切除术的概率显著更高（HR：2.13，95%CI：1.50-3.03）；结直肠癌（CRC）（HR：21.4，95%CI：9.6-47.6）、胆管癌（HR：190，95%CI：54.8-660）和死亡（HR：4.39，95%CI：3.22-6.00）的风险更高，与非 PSC-IBD 对照相比。25 年的肝移植累积风险很高（53%）。
这项未选择的基于人群的研究表明，PSC-IBD 患者不仅具有广泛的 IBD 表型，而且比其他 IBD 患者接受更强化的治疗。然而，预后仍然很差，且没有随着时间的推移而明显改善。
