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Review
, 67 (6), 1298-1323

Primary Sclerosing Cholangitis - A Comprehensive Review

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Review

Primary Sclerosing Cholangitis - A Comprehensive Review

Tom H Karlsen et al. J Hepatol.

Abstract

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.

Keywords: Cholangiocarcinoma; IgG4-associated cholangitis; Primary sclerosing cholangitis.

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