Abstract
Primary sclerosing cholangitis is characterized by progressive fibrosing inflammation of the bile ducts leading to their obliteration, resulting in cholestasis, and finally, cirrhosis of the liver. The majority of patients with advanced disease with time develop dominant stenoses of major bile ducts. Such dominant stenoses may be recognized by magnetic resonance cholangiopancreatography with good specificity and sensitivity, but for definite diagnosis and especially for therapeutic decisions, endoscopic retrograde cholangiography still represents the gold standard. Ursodeoxycholic acid treatment does not prevent the development of such stenoses. Endoscopic measures allow the opening of short- and long-segment stenoses of the common bile duct. Short- but not long-segment stenoses of the hepatic ducts near to the bifurcation also may be treated endoscopically. Repeated balloon dilatations over years appear to be the treatment of choice. In patients treated by early endoscopic dilatation of dominant stenoses, survival may be significantly improved compared with the predicted survival.
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