Management

Definitive treatment is liver transplantation for those with such severe cirrhosis as to cause liver failure. Endoscopic dilation of strictures to improve bile flow and relieve cholangitis is transiently palliative. Surgical excision of stenotic extrahepatic ducts with hepaticoenterostomy and transhepatic stenting in noncirrhotic patients is effective in alleviating jaundice, but requires frequent stent changes and makes later transplantation more difficult. Some studies suggest that ursodeoxycholic acid and methotrexate may be of some value. Cholangiocarcinoma develops or is present in 5-10% of patients with PSC; this is a contraindication to liver transplantation.

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