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Introduction
Primary sclerosing cholangitis (PSC) is a rare cholestatic disorder with a prevalence of 16.2 per 100 000 population. It is characterised by progressive inflammation and destruction of the intrahepatic and extraheptic bile ducts culminating in progressive fibrosis and cirrhosis.1–3 The course of PSC is complicated by biliary strictures, recurrent cholangitis and a 400-1500 times higher risk of cholangiocarcinoma (CC) and other hepatopancreatobiliary malignancy than the general population.1 4–9 Treatment of PSC revolves around managing symptoms and complications as they arise. Endoscopic retrograde cholangiography (ERC) is a valuable tool that allows therapeutic interventions to optimise biliary drainage and facilitate biliary sampling.10 Despite the utility, controversies remain as to when ERC should be performed.11 12 This article aims to clarify some of the issues surrounding this and to provide practical guidance on the ERC-based assessment and management of biliary strictures in PSC.bile ducts culminating in progressive fibrosis and cirrhosis.1–3 The course of PSC is complicated by biliary strictures, recurrent cholangitis and a 400-1500 times higher risk of cholangiocarcinoma (CC) and other hepatopancreatobiliary malignancy than the general population.1 4–9 Treatment of PSC revolves around managing symptoms and complications as they arise. Endoscopic retrograde cholangiography (ERC) is a valuable tool that allows therapeutic interventions to optimise biliary drainage and facilitate biliary sampling.10 Despite the utility, controversies remain as to when ERC should be performed.11 12 This article aims to clarify some of the issues surrounding this and to provide practical guidance on the ERC-based assessment and management of biliary strictures in PSC.
Natural history of PSC
The aetiology of PSC is unclear but may involve cholangiocyte injury resulting from an abnormal immune response to environmental exposure in genetically susceptible individuals.3 13 PSC most commonly affects men between the ages of 30 and 40 and usually occurs in the presence of …
Footnotes
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Contributors WA wrote and prepared the manuscript. DJ, MTH, SME, MJ, SM, KWO, WO, BP, PT and GW reviewed and corrected the manuscript. VSH supervised, reviewed and edited the manuscript. All authors have read and agreed to the final version of the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.