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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.

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date: 27 September 2021

Case History—A 60 yr old woman, known to have long standing colitis, now presenting with abdominal pain and weight loss.

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....

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