UCLA-Olive View Internal Medicine Residency Program, Olive View-UCLA Medical Center, Sylmar, CA 91342, United States.
Office of the University Provost, Arizona State University, Phoenix, AZ 85004, United States.
World J Gastroenterol. 2019 Feb 14;25(6):659-671. doi: 10.3748/wjg.v25.i6.659.
Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease characterized by progressive fibroinflammatory destruction of the intra- and/or extrahepatic biliary ducts. While its features and disease course can be variable, most patients with PSC have concurrent inflammatory bowel disease and will eventually develop liver cirrhosis and end-stage liver disease, with liver transplantation representing the only potentially curative option. Importantly, PSC is associated with a significantly increased risk of malignancy compared to the general population, mainly cholangiocarcinoma, gallbladder carcinoma, hepatocellular carcinoma, and colorectal cancer, with nearly 50% of deaths in patients with PSC being due to cancer. Therefore, robust surveillance strategies are needed, though uncertainty remains regarding how to best do so. In this review, we discuss the epidemiology, prevention, and surveillance of cancers in patients with PSC. Where evidence is limited, we present pragmatic approaches based on currently available data and expert opinion.
原发性硬化性胆管炎（PSC）是一种罕见的胆汁淤积性肝病，其特征为肝内外胆管进行性纤维炎症性破坏。虽然其特征和疾病过程可能存在差异，但大多数 PSC 患者同时患有炎症性肠病，最终将发展为肝硬化和终末期肝病，肝移植是唯一潜在的治愈选择。重要的是，与普通人群相比，PSC 患者的恶性肿瘤风险显著增加，主要包括胆管癌、胆囊癌、肝细胞癌和结直肠癌，PSC 患者中有近 50%的死亡归因于癌症。因此，需要强有力的监测策略，但如何最好地进行监测仍存在不确定性。在本文中，我们讨论了 PSC 患者癌症的流行病学、预防和监测。在证据有限的情况下，我们根据现有数据和专家意见提出了实用的方法。
