Primary sclerosing cholangitis – a comprehensive review

Published on Dec 1, 2017in Journal of Hepatology18.95
· DOI :10.1016/j.jhep.2017.07.022
Tom H. Karlsen47
Estimated H-index: 47
(University of Oslo),
Trine Folseraas11
Estimated H-index: 11
(Oslo University Hospital)
+ 1 AuthorsMette Vesterhus15
Estimated H-index: 15
(Oslo University Hospital)
Summary 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.
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