Primary biliary cirrhosis

Abstract

Primary biliary cirrhosis (PBC) is a chronic, cholestat-ic liver disease that is characterized by progressive immune mediated destruction of the intrahepatic bile ducts. Over time, fibrosis extends out of the portal tracts and progresses to cirrhosis. Neither the etiology nor the pathogenesis are well understood; however, most of the current evidence suggests that it is an autoimmune condition that may be triggered by environmental stimuli in genetically predisposed individuals. Since the precise mechanisms involved in the pathogenesis of PBC have not been elucidated, curative therapy has not been identified and the focus has been on preventing disease progression. Ursodeoxycholic acid (UDCA), the only approved therapy for PBC, improves histology and retards disease progression. Future studies will likely combine UDCA with anti-inflammatory, immunosuppressive, immunomodulatory or antimicrobial agents. Once the etiology and pathogenesis of PBC are better delineated, more definitive therapy can be designed with curative intent.