ABSTRACT
Primary biliary cirrhosis(PBC) is a chronic cholestatic liver disease of unknown origin. The small and medium sized intrahepatic bile ducts are destroyed by an inflammatory process, which, it has been suggested, is of the autoimmune type. It is strongly associated with the presence of antimitochondrial antibodies, predominantly IgM and IgG. The liver changes are classified into four stages, of which stage IV represents the development of cirrhosis, which required orthotropic liver transplantation in the longrun. The prevalence rates was reported 128 per millon in Sweden , but the disease is relatively rare in Oriental area. In medical treatment, long-term administration of ursodeoxycholic acid improves both clinical and biochemical signs, slows the progression of the disease and reduces the complication requiring liver transplantation. We report two cases of PBC, one with histologically proven cirrhosis, and the other with bile duct destruction consistent with stage III and hypothyroidism.