RESUMO
Primary biliary cirrhosis (PBC) is a chronic progressive disease, more often affecting women, characterised by progressive destruction of the small intrahepatic bile ducts with portal inflammation leading to fibrosis and cirrhosis. There is a close association between PBC and antibodies to antimitochondrial antibodies (AMA). The pathogenesis of PBC remains uncertain. The disease has been considered as an example of autoimmunity. Estimates of disease prevalence vary between 20 and 240 cases per milion per year. PBC is a extremely rare liver disease in Asia. There is no definitive drug treatment, liver transplantation is indicated for patients with intractable symptoms and for end-stage disease. The authors have experienced a case of PBC in 66 year-old female who suffered from lethargy, pruritus and dry mouth since 2 years ago. We report a case of PBC with a review of literatures.