Primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is a chronic intrahepatic cholestatic liver disease. Serum anti-mitochondrial antibody (AMA) is a specific marker for the diagnosis of PBC, and ursodeoxycholic acid (UDCA) is the only drug proven to be safe and effective for the treatment of PBC in randomized controlled clinical trials. Although the pathogenesis of PBC may be related to autoimmunity, the efficacy of immunosuppressive agents has not yet been confirmed, and drug-related adverse reactions limit their clinical application. The course of PBC is progressive, ultimately leading to cirrhosis and liver failure. Liver transplantation is the only effective treatment for patients with end-stage PBC.