Norwegian PSC Research Center, Department of Transplantation Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway; Institute of Clinical Medicine, University of Oslo, Norway; Research Institute of Internal Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway.
Norwegian PSC Research Center, Department of Transplantation Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway; Research Institute of Internal Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway.
J Hepatol. 2017 Dec;67(6):1298-1323. doi: 10.1016/j.jhep.2017.07.022. Epub 2017 Aug 10.
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.
原发性硬化性胆管炎（PSC）是一种罕见的疾病，其特征为多灶性胆管狭窄和进行性肝病。通常存在炎症性肠病，并且有发生胆管癌和结直肠癌的高风险。大多数患者最终需要进行肝移植，之后可能会发生疾病复发。由于治疗选择有限且缺乏经过证实的监测策略，患者目前存在显著的未满足需求。在本次研讨会中，我们全面回顾了该领域的现状。我们强调了与患者分层和疾病行为相关的进展，并从实际的、以患者为中心的角度概述了管理选择。我们调查了对 PSC 发病机制的理解方面所取得的进展，并总结了基于这些见解开发有效疗法的持续努力。
