Primary sclerosing cholangitis (PSC) is a rare disorder characterised by multi-focal bile duct strictures and progressive liver disease. Inflammatory bowel disease is usually present and there is a high risk of cholangiocarcinoma and colorectal cancer. Most patients ultimately require liver transplantation, after which disease recurrence may occur. With limited therapeutic options and a lack of proven surveillance strategies, patients currently have significant unmet needs. In the present seminar, we provide a comprehensive review of the status of the field. We emphasise developments related to patient stratification and disease behaviour, and provide an overview of management options from a practical, patient-centered perspective. We survey advances made in the understanding of PSC pathogenesis and summarise the ongoing efforts to develop an effective therapy based on these insights.

中文翻译：

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原发性硬化性胆管炎——综合回顾

原发性硬化性胆管炎 (PSC) 是一种罕见的疾病，其特征是多灶性胆管狭窄和进行性肝病。炎症性肠病通常存在，胆管癌和结直肠癌的风险很高。大多数患者最终需要肝移植，之后可能会出现疾病复发。由于治疗选择有限且缺乏经过验证的监测策略，患者目前有大量未满足的需求。在本次研讨会中，我们全面回顾了该领域的现状。我们强调与患者分层和疾病行为相关的发展，并从实用的、以患者为中心的角度提供管理选择的概述。