| [ Summary ] |
Primary biliary cirrhosis is a chronic progressive cholestatic liver disease, mainly affecting middle aged women. Histological findings include chronic nonsuppurative destructive cholangitis. Although the pathogenesis of PBC is an enigma, immunological disturbance is most often suspected because of the presence of autoantibodies, such as anti-mitochondrial antibodies, and complications with other autoimmune diseases. There have been major advances made regarding autoantibody responses as well as autoreactive T cell responses against PDC-E2, a major mitochondrial autoantigen. Some distinct atypical cases, such as autoimmune cholangitis, have been observed. UDCA and various other drugs are used for the treatment of PBC. Liver transplantation should be taken into consideration of the advanced stages. Hepatocellular carcinoma may also be found in association with PBC. |