Primary sclerosing cholangitis (PSC) is a rare obliterative fibrotic condition of the bile ducts. We assessed PSC epidemiology and natural history within the UK Clinical Practice Research Datalink (CPRD). Incidence and natural history of PSC were evaluated in a retrospective cohort study using linkage of CPRD, Hospital Episode Statistics, and Office for National Statistics data. Data from age, sex, and general practice-matched population controls provided a context for the incident PSC patients. Liver disease other than PSC was defined as autoimmune hepatitis, hepatitis, hepatomegaly, liver failure, cirrhosis, portal hypertension, cholangiocarcinoma, or hepatobiliary cancer. The age-standardized incidence of PSC was 0.68 (95% confidence interval [CI] 0.45–0.99) per 100,000 person-years and the age-standardized prevalence was 5.58 (95% CI 4.82–7.35) per 100,000 during 1998 to 2014. In all, 250 incident PSC patients met the inclusion criteria and each was matched with 5 controls (mean age 54 ± 18 years, men 63.2%). A higher percentage of PSC patients had a history of inflammatory bowel disease (54% vs 2%) and liver disease other than PSC (22% vs 1%) than controls (standardized differenceweighted >0.1). During a median follow-up of 5 years, PSC patients were more likely to develop adverse health outcomes. The mortality rate per 1000 person-years was 3-fold higher in PSC than population controls (49.5 vs 16.1; incidence rate ratio 3.1, 95% CI 2.2–4.2). The incidence and prevalence of PSC observed in the UK CPRD were either comparable with or higher than previous studies. Compared with the general population, PSC patients had worse health outcomes including PSC disease progression, complications, and higher mortality.
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机译:原发性硬化性胆管炎(PSC)是一种罕见的胆管闭塞性纤维化病。我们在英国临床实践研究数据链(CPRD)中评估了PSC流行病学和自然史。在一项回顾性队列研究中,使用CPRD,医院情节统计数据和国家统计局数据的链接对PSC的发病率和自然史进行了评估。来自年龄,性别和与常规操作相匹配的人群控制的数据为事件中的PSC患者提供了背景。除PSC以外的肝病定义为自身免疫性肝炎,肝炎,肝肿大,肝衰竭,肝硬化,门脉高压,胆管癌或肝胆癌。在1998年至2014年期间,PSC的年龄标准化发病率为每100,000人年0.68(95%可信区间[CI] 0.45–0.99),年龄标准化的患病率为每100,000人5.58(95%CI 4.82–7.35)。所有250例PSC入组患者均符合纳入标准,每例均与5名对照(平均年龄54±18岁,男性63.2%)匹配。与对照组相比,有较高百分比的PSC患者有炎症性肠病史(54%vs 2%)和除PSC以外的肝病(22%vs 1%)(标准化差异加权 sub 0.1)。在5年的中位随访期间,PSC患者更有可能出现不利的健康结局。在PSC中,每千人年的死亡率比人口对照组高3倍(49.5比16.1;发病率3.1,95%CI 2.2-4.2)。在英国CPRD中观察到的PSC的发生率和患病率与以前的研究相当或更高。与普通人群相比,PSC患者的健康结局更差,包括PSC疾病进展,并发症和更高的死亡率。
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