Primary sclerosing cholangitis

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Primary sclerosing cholangitis (PSC) is an idiopathic liver disease that is most likely immune mediated. Unlike primary biliary cirrhosis, there is a male predominance (2:1) with median onset at 30 years, though the age of onset ranges widely.

Patients with PSC exhibit a marked prevalence of HLA B8 and DR3, which are also associated with autoimmune diseases. Because 70% of PSC patients have ulcerative colitis, toxin release from inflamed gut has been suggested as a possible mechanism. An infective agent in a genetically and immunologically susceptible individual also remains a possibility.

Pathologically, extrahepatic and large intrahepatic ducts show segmental periductal chronic inflammation and fibrosis, resulting in multiple strictures. The ducts proximal to the stricture become dilated, and cirrhosis develops in most.

Treatment is frustrating in that there is really no medical management. If there are dominant strictures then endoscopically placed biliary stents can be useful. If there is evidence of cholangitis, then intermittent use of antibiotics is preferable. However, over the long term, this chronic progressive fibrotic disease will result in end stage secondary biliary cirrhosis and subsequently will need liver transplantation.