Treatment of primary biliary cirrhosis

Although primary biliary cirrhosis (PBC) is generally a progressive disease , the rate of progression varies greatly from one patient to another. The t erminal phase is characterized by hyperbilirubinaemia (> 100 mu mol/l), a m ajor decrease in the number of intrahepatic bile ducts, and extensive fibro sis or cirrhosis. It is now well established that orthotopic liver transpla ntation is the treatment of choice for patients entering the terminal phase of the disease. A variety of therapeutic agents have been proposed for treatment of patient s with PBC. However, most have been found ineffective or coo toxic to be wi dely used. In contrast, there is accumulating evidence from large therapeut ic trials that long-term administration of ursodeoxycholic acid (UDCA) is s afe and prolongs survival free of liver transplantation. Treatment with UDC A slows the histological progression and delays the onset of cirrhosis. In patients who have a sub-optimal response to UDCA therapy alone, the comb ination of colchicine or methotrexate with UDCA has minimal or no additiona l benefit, whereas that with corticosteroids is more promising but not yet demonstrated. Among causes of non-response to UDCA therapy, the most common is the PBC-au toimmune hepatitis overlap syndrome. The benefit from the combination of co rticosteroids and UDCA in this setting is obvious. Further studies are needed to define the patients who are most likely to re spond to UDCA therapy and to assess the benefit of combined medical treatme nts.