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Primary sclerosing cholangitis 

Primary sclerosing cholangitis

Primary sclerosing cholangitis

R.W. Chapman

and K.D. Williamson



The epidemiology of PSC has been updated to reflect further recent studies, including new data on an increased prevalence of Crohn’s disease in PSC than previously recognised. There is further data to support the use of ursodeoxycholic acid in PSC, though not in high doses, and the section on IgG4-associated cholangitis (IAC) has been expanded due to the publication of multiple trials in this area and the increasing recognition of the importance of IAC as a differential diagnosis in PSC. Additonal important references have been added to the “Further Reading” section, including a new Genome Wide Association Study in PSC.

A relevant case history from Oxford Case Histories in Gastroenterology and Hepatology has been added to this chapter.

Updated on 28 Nov 2012. The previous version of this content can be found here.
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date: 23 April 2017

Primary sclerosing cholangitis is a chronic cholestatic liver disease caused by diffuse inflammation and fibrosis that can involve the entire biliary tree. The cause is unknown, but presumed to be immune mediated, and there is a very close association with inflammatory bowel disease, particularly ulcerative colitis.

The disorder tends to affect men (male:female 2:1), some presenting with fatigue, intermittent jaundice, weight loss, right upper quadrant pain, and pruritus, but many are asymptomatic at diagnosis, which is made incidentally when a persistently raised serum alkaline phosphatase is discovered, usually in the setting of ulcerative colitis.

Serum biochemical tests usually indicate cholestasis, but diagnosis is based on three criteria: (1) generalized beading and stenosis of the biliary system on cholangiography; (2) absence of choledocholithiasis or a history of bile duct surgery; and (3) exclusion of bile duct cancer, usually by prolonged follow-up.

There is no curative treatment. Pruritus is initially managed with cholestyramine, with second-line treatments including rifampicin and naltrexone. Orthotopic liver transplantation is the only option available for young patients with advanced liver disease.

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