Primary sclerosing cholangitis - a comprehensive review

[摘要] 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. (C) 2017 European Association for the Study of the Liver. Published by Elsevier B.V.

[发布日期] 2017-12-01

[关键词] Primary sclerosing cholangitis;IgG4-associated cholangitis;Cholangiocarcinoma