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Primary sclerosing cholangitis

Lookup NU author(s): Dr Jess Dyson, Professor David Jones, Dr Mark Hudson


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© 2018 Elsevier Ltd. Primary sclerosing cholangitis is a rare, chronic cholestatic liver disease characterised by intrahepatic or extrahepatic stricturing, or both, with bile duct fibrosis. Inflammation and fibrosis of bile ducts and the liver are followed by impaired bile formation or flow and progressive liver dysfunction. Patients might be asymptomatic at presentation or might have pruritus, fatigue, right upper quadrant pain, recurrent cholangitis, or sequelae of portal hypertension. The key diagnostic elements are cholestatic liver biochemistry and bile duct stricturing on cholangiography. Genetic and environmental factors are important in the cause of the disease, with the intestinal microbiome increasingly thought to play a pathogenetic role. Approximately 70% of patients have concurrent inflammatory bowel disease and patients require colonoscopic screening and surveillance. Primary sclerosing cholangitis is associated with increased malignancy risk and surveillance strategies for early cholangiocarcinoma detection are limited. No single drug has been proven to improve transplant-free survival. Liver transplantation is effective for advanced disease but at least 25% of patients develop recurrent disease in the graft.

Publication metadata

Author(s): Dyson JK, Beuers U, Jones DEJ, Lohse AW, Hudson M

Publication type: Article

Publication status: Published

Journal: The Lancet

Year: 2018

Volume: 391

Issue: 10139

Pages: 2547-2559

Print publication date: 23/06/2018

Online publication date: 13/02/2018

Acceptance date: 02/04/2016

ISSN (print): 0140-6736

ISSN (electronic): 1474-547X

Publisher: Lancet Publishing Group


DOI: 10.1016/S0140-6736(18)30300-3


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