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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.
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
URL: https://doi.org/10.1016/S0140-6736(18)30300-3
DOI: 10.1016/S0140-6736(18)30300-3
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