Show Summary Details
Page of

Primary sclerosing cholangitis 

Primary sclerosing cholangitis

Chapter:
Primary sclerosing cholangitis
Author(s):

R.W. Chapman

and K.D. Williamson

DOI:
10.1093/med/9780199204854.003.152104_update_001

Update:

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.
Page of

PRINTED FROM OXFORD MEDICINE ONLINE (www.oxfordmedicine.com). © Oxford University Press, 2015. All Rights Reserved. Under the terms of the licence agreement, an individual user may print out a PDF of a single chapter of a title in Oxford Medicine Online for personal use (for details see Privacy Policy).

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.

Access to the complete content on Oxford Medicine Online requires a subscription or purchase. Public users are able to search the site and view the abstracts for each book and chapter without a subscription.

Please subscribe or login to access full text content.

If you have purchased a print title that contains an access token, please see the token for information about how to register your code.

For questions on access or troubleshooting, please check our FAQs, and if you can't find the answer there, please contact us.