Primary sclerosing cholangitis- a comprehensive review and update
Journal Title: International Journal of Research in Pharmacology & Pharmacotherapeutics (IJRPP) - Year 2015, Vol 4, Issue 2
Abstract
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology characterised by inflammation and fibrosis of the biliary tree. The aim of this review is to find out the cause, symptoms, and treatment of PSC. Remissions and relapses characterize the disease course. PSC may remain quiescent for long periods of time in some patients, however, it is progressive. The mean age at diagnosis is 40 years and men are affected twice as often as women. There is a reported annual incidence of PSC of 0.9–1.31/100,000 and point prevalence of 8.5–13.6/100,000. In most, the disease progresses to cirrhosis and liver failure. Cholangiocarcinoma develops in 8–30% of patients. PSC is thought to be immune mediated and is often associated with inflammatory bowel disease, especially ulcerative colitis. The disease is diagnosed on typical cholangiographic and histological findings and after exclusion of secondary sclerosing cholangitis. Median survival has been estimated to be 12 years from diagnosis in symptomatic patients. Patients who are asymptomatic at diagnosis, the majority of whom will develop progressive disease, have a survival rate greater than 70% at 16 years after diagnosis. Liver transplantation remains the only effective therapeutic option for patients with end-stage liver disease from PSC, although high dose ursodeoxycholic acid may have a beneficial effect.
Authors and Affiliations
K. Dilip Krishnan
Keywords
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