Primary sclerosing cholangitis.

Six patients with primary sclerosing cholangitis are described. It is an uncommon disease of uncertain aetiology following an unpredictable course but often progresses to secondary biliary cirrhosis, portal hypertension, and premature death from liver failure. Ulcerative colitis is commonly associated with primary sclerosing cholangitis. Endoscopic retrograde cholangiography is the investigation of choice. Liver biopsy may support the diagnosis but is not pathognomonic. There is no effective treatment but symptoms may be controlled.