Primary biliary cirrhosis( PBC) is a chronic cholestatic liver disease,characterized by progressive non- pyogenic inflammation in the small intrahepatic bile ducts,and can cause hepatic fibrosis and liver cirrhosis. The etiology and pathogenesis of PBC remain unknown. The development and progression of PBC are caused by various factors,and the major factor is environmental factors acting on patients with genetic susceptibility. Ursodesoxycholic acid is thought to be the only effective drug,but the complete response rate remains low.This article reviews the diagnosis and treatment of PBC with reference to the literature in the world.
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