ABSTRACT
Primary biliary cholangitis (PBC) is a chronic progressive cholestatic liver disease of unknown etiology characterized by highly specific anti-mitochondrial antibody in serum and immune-mediated non-pyogenic destructive infection in the small intrahepatic bile ducts,which can lead to portal inflammation and fibrosis and finally progress to liver cirrhosis and liver failure.At present,ursodeoxycholic acid (UDCA) is the only drug approved for the treatment of PBC with a recommended dose of 13-15 mg · kg-1 · d-1.There are significant improvements in the survival rate of patients achieving biochemical response after UDCA treatment.However,about 40％ of PBC patients do not respond to UDCA,and such patients have a risk of disease progression and are in urgent need of other drugs.With reference to recent clinical studies and guidelines,this article summarizes the basic concepts and latest advances in pharmacotherapy for PBC,as well as the perspectives of new drugs in clinical trials,in order to bring new hopes to PBC patients with poor response to UDCA.