Sydney Medical School, The University of Sydney, Sydney, NSW, Australia.
Gastroenterology and Liver Services, Concord Hospital, Sydney, NSW, Australia.
Liver Int. 2017 Mar;37(3):442-448. doi: 10.1111/liv.13328. Epub 2017 Jan 24.
BACKGROUND & AIMS: Epidemiological data on primary sclerosing cholangitis (PSC) outside the Northern hemisphere are limited. Similarly, the impact of inflammatory bowel disease (IBD) on PSC outcomes remains unclear. We aimed to study the epidemiology and outcomes of PSC patients with and without IBD in an Australian cohort.
We retrospectively studied PSC patients attending two tertiary referral hospitals over 20 years. Diagnosis of PSC was made according to international guidelines by positive cholangiography and/or liver biopsy (for small duct PSC) with supporting clinical and laboratory evidence.
Of 208 PSC patients (61% male) were studied (2271patient-years follow-up). The median age of PSC diagnosis was similar for PSC-IBD and PSC-only patients (40 years vs 42 years, P = .35). All 33 deaths occurred in PSC-IBD patients while there were no deaths in PSC-only patients (21% vs 0%, P < .01). However, there were no significant differences in liver transplantation (PSC-only 25% vs PSC-IBD 31%, P = .45) and transplant-free survival between PSC-only and PSC-IBD patients (P = .43). On multivariate Cox regression, only elevated international normalized ratio (INR) was associated with a greater risk of death or liver transplant (HR 2.0, 95% CI 1.1-3.6, P = .02). Development of gastrointestinal malignancy was higher in the PSC-IBD group compared to PSC-only group (22% vs 2%, P < .01).
Australian PSC patients have similar characteristics compared to European and North American cohorts. IBD is a significant predictor of gastrointestinal malignancies. Deaths were more common in PSC-IBD but overall transplant-free survival remained similar in PSC-IBD and PSC-only groups. An elevated INR was an independent predictor of death or liver transplantation.
在北半球以外地区原发性硬化性胆管炎（PSC）的流行病学数据有限。同样，炎症性肠病（IBD）对 PSC 结局的影响也不清楚。我们旨在研究澳大利亚队列中伴有和不伴有 IBD 的 PSC 患者的流行病学和结局。
我们回顾性研究了 20 年来在两家三级转诊医院就诊的 PSC 患者。PSC 的诊断是根据国际指南通过阳性胆管造影和/或肝活检（对于小胆管 PSC）做出的，同时伴有支持临床和实验室证据。
共研究了 208 例 PSC 患者（61%为男性）（2271 患者年随访）。PSC-IBD 和 PSC 患者的 PSC 诊断中位年龄相似（40 岁对 42 岁，P =.35）。所有 33 例死亡均发生在 PSC-IBD 患者中，而 PSC 患者无一例死亡（21%对 0%，P <.01）。然而，PSC 患者的肝移植（PSC 患者 25%对 PSC-IBD 患者 31%，P =.45）和无移植生存率在 PSC 患者和 PSC-IBD 患者之间无显著差异（P =.43）。多变量 Cox 回归分析显示，只有国际标准化比值（INR）升高与死亡或肝移植的风险增加相关（HR 2.0，95%CI 1.1-3.6，P =.02）。与 PSC 患者相比，PSC-IBD 患者的胃肠道恶性肿瘤发生率更高（22%对 2%，P <.01）。
澳大利亚 PSC 患者的特征与欧洲和北美队列相似。IBD 是胃肠道恶性肿瘤的重要预测因素。PSC-IBD 患者的死亡率较高，但 PSC-IBD 和 PSC 患者的无移植生存率相似。INR 升高是死亡或肝移植的独立预测因素。
