Michal R. Piják, Štefan Hrušovský, Boris Rychlý
Primary biliary cirrhosis is a slowly progressive autoimmune liver disease of unknown etiology, most often diagnosed in middle-aged women. It is characterized by inflammation and destruction of the intrahepatic biliary ducts, resulting in chronic cholestasis and eventually cirrhosis. The presence of serum antimitochondrial antibody (AMA) is considered a diagnostic hallmark based on its occurrence in approximately 90 % of patients. Treatment of PBC is addressed at preventing or relieving the symptoms and clinical consequences of chronic cholestasis, and also at correcting the bile duct abnormalities by specific treatments. Ursodeoxycholic acid (13 - 15 mg/kg/day), has become the standard specific treatment, resulting in marked relieving of cholestasis and prolonging survival. The disease is an indication for liver transplantation.