Background and aims

Primary sclerosing cholangitis (PSC) is associated with an increased risk of hepatobiliary and colorectal cancer, but the risks of other cancer forms have not been explored. The aim of this study was to evaluate the risk of intestinal and extraintestinal cancers in a large, well-defined cohort of PSC patients.

Material and method

A matched cohort study of Swedish PSC patients was performed with up to ten comparators for each patient, matched for sex, age, and residency. The data were retrieved from national registers. Patients were followed from PSC diagnosis until cancer diagnosis, liver transplantation, first emigration date, death, or December 31, 2016. The risk of cancer was estimated using the Kaplan–Meier method and Cox regression models.

Results

In total, 1432 PSC patients with a verified diagnosis and 14,437 comparators were studied. The mean follow-up time was 15.9 years. Eighty-eight percent of the PSC patients had concomitant inflammatory bowel disease. PSC patients ran significantly increased risks of developing any cancer [HR 3.8, 95% confidence interval (CI) 3.3–4.3], hepatobiliary cancer (HR 120.9, 95% CI 72.0–203.1), colorectal cancer (HR 7.5, 95% CI 5.6–10.0), pancreatic cancer (HR 8.0, 95% CI 3.2–20.2), gastric cancer (HR 4.2, 95% CI 1.5–11.3), small bowel cancer (HR 21.1, 95% CI 3.5–128.2), and lymphoma (HR 3.0, 95% CI 1.6–5.7). PSC was not associated with a lower risk of any cancer form.

Conclusions

PSC patients have a four times overall increased risk of developing cancer compared to the general population, with increased risk of developing hepatobiliary, colorectal, and pancreatic cancer, as well as lymphoma.

中文翻译：

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原发性硬化性胆管炎患者患癌风险增加

背景和目标

原发性硬化性胆管炎 (PSC) 与肝胆癌和结直肠癌的风险增加有关，但尚未探索其他癌症形式的风险。本研究的目的是评估大型、明确的 PSC 患者队列中肠癌和肠外癌的风险。

材料和方法

对瑞典 PSC 患者进行了一项匹配队列研究，每位患者使用多达 10 个比较器，根据性别、年龄和居住地进行匹配。这些数据是从国家登记册中检索的。患者从 PSC 诊断到癌症诊断、肝移植、首次移民日期、死亡或 2016 年 12 月 31 日进行随访。使用 Kaplan-Meier 方法和 Cox 回归模型估计癌症风险。

结果

总共研究了 1432 名确诊的 PSC 患者和 14437 名对照者。平均随访时间为 15.9 年。88% 的 PSC 患者伴有炎症性肠病。PSC 患者发生任何癌症 [HR 3.8, 95% CI 3.3–4.3]、肝胆癌 (HR 120.9, 95% CI 72.0–203.1)、结直肠癌 (HR 7.5, 95% CI 5.6) 的风险显着增加–10.0)、胰腺癌 (HR 8.0, 95% CI 3.2–20.2)、胃癌 (HR 4.2, 95% CI 1.5–11.3)、小肠癌 (HR 21.1, 95% CI 3.5–128.2) 和淋巴瘤 (HR 21.1, 95% CI 3.5–128.2) HR 3.0, 95% CI 1.6–5.7)。PSC 与任何癌症形式的较低风险无关。

结论

与普通人群相比，PSC 患者患癌症的总体风险增加了四倍，患肝胆癌、结直肠癌和胰腺癌以及淋巴瘤的风险增加。