ABSTRACT

Introduction: Primary sclerosing cholangitis (PSC) is a rare, heterogenous, chronic cholestatic liver disease that causes fibro-inflammatory destruction of the intra- and/or extrahepatic bile ducts. The disease course may be variable, though in many cases it ultimately leads to biliary cirrhosis and its associated complications. PSC is also associated with malignancies, in particular cholangiocarcinoma (CCA), a dreaded neoplasm of the biliary tract with a poor prognosis. Risk stratification and surveillance for this malignancy are important components of the care of patients with PSC.

Areas covered: In this review, we discuss important considerations in the clinical epidemiology, risk factors, diagnosis, and surveillance of PSC-associated CCA.

Expert opinion: Despite growing awareness of PSC, high-quality evidence regarding the management of PSC and its associated risk of CCA remains limited. Early diagnosis of PSC-associated CCA remains difficult, and treatment options are limited, especially when diagnosed at later stages. The recent introduction of recommendations for CCA surveillance will likely improve outcomes, though an optimal surveillance approach has yet to be validated prospectively. Further research is needed in the development of high-accuracy (and noninvasive) surveillance and diagnostic tools that may facilitate earlier diagnosis of CCA and potential disease cure.

摘要

简介：原发性硬化性胆管炎 (PSC) 是一种罕见的异质性慢性胆汁淤积性肝病，可导致肝内和/或肝外胆管的纤维炎症破坏。病程可能是可变的，但在许多情况下最终会导致胆汁性肝硬化及其相关并发症。PSC 还与恶性肿瘤有关，尤其是胆管癌 (CCA)，这是一种预后不良的胆道肿瘤。这种恶性肿瘤的风险分层和监测是 PSC 患者护理的重要组成部分。

涵盖的领域：在这篇综述中，我们讨论了 PSC 相关 CCA 的临床流行病学、危险因素、诊断和监测中的重要考虑因素。

专家意见：尽管对 PSC 的认识不断提高，但关于 PSC 管理及其相关 CCA 风险的高质量证据仍然有限。PSC 相关 CCA 的早期诊断仍然很困难，治疗选择也很有限，尤其是在晚期诊断时。最近引入的 CCA 监测建议可能会改善结果，但最佳监测方法尚未得到前瞻性验证。需要进一步研究开发高精度（和非侵入性）监测和诊断工具，以促进 CCA 的早期诊断和潜在的疾病治愈。