Abstract
BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease that involves progressive destruction of the bile ducts. Its prevalence is 4 to 16 cases per 100,000 persons. Its incidence has risen over the last 20 years, with a more than 35% increase in the last 10 years alone. PSC tends to arise in patients with chronic inflammatory bowel diseases. It is associated with an increased risk of various types of cancer (13%-14%), most prominently cholangiocellular carcinoma (CCC). METHOD: This review is based on a selective search in PubMed for original articles, meta-analyses, and review articles about PSC that appeared from January 1980 to May 2013. RESULTS: The diagnosis is generally established with a bile duct imaging study--typically, magnetic resonance cholangiopancreaticography (MRCP): this test is more than 80% sensitive and more than 90% specific for the diagnosis of PSC. The time from diagnosis to death or liver transplantation is 12 to 18 years, and the risk that a patient with PSC will die of cancer is 40% to 58%. Options for drug treatment are limited. Randomized, controlled trials have not shown any improvement of outcomes from the administration of ursodeoxycholic acid (UDCA). Interventional endoscopy is used to treat dominant stenoses and cholangitis, even though this method of treatment is supported only by low-level evidence. Liver transplantation results in a 10-year survival rate above 80%. CONCLUSION: There is no causally directed treatment for PSC. Early diagnosis, complication management, and the evaluation of an optimally timed liver transplantation are the main determinants of outcome.