Primary Biliary Cirrhosis

Primary biliary cirrhosis (PBC) is one of the most common chronic cholestatic liver diseases affecting the adult population. The clinical presentation of PBC can be diverse, ranging from the presymptomatic individual to the patient with advanced liver disease. The initial evaluation to establish the diagnosis, and the appropriate followup, are very important in the lifelong management of these patients. The primary medical treatment in PBC should focus on reducing the rate of disease progression. To this extent, ursodeoxycholic acid has been extensively evaluated and proven to improve liver biochemistries and survival in patients with PBC. The secondary medical management in PBC should address the treatment of complications of chronic cholestasis, hepatic cirrhosis, and failure. Liver transplantation remains the only established therapeutic approach in treating patients with end-stage PBC and its associated complications.