Diagnosis and management of primary biliary cirrhosis

Expert Rev Clin Immunol. 2014 Dec;10(12):1667-78. doi: 10.1586/1744666X.2014.979792. Epub 2014 Nov 10.


Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease characterized histologically by destruction of intrahepatic bile ducts and serologically by the presence of antimitochondrial antibodies. The incidence and prevalence of PBC are increasing. Fatigue and pruritus are common symptoms in PBC, although the proportion of asymptomatic PBC is increasing due to the widespread use of screening biochemical tests and antimitochondrial antibody assays. PBC may eventually lead to cirrhosis and its consequent complications. In the 1980s, PBC was the leading indication for liver transplantation. Ursodeoxycholic acid is the only US FDA-approved therapeutic agent for PBC. Clinical trials have shown that the use of ursodeoxycholic acid in PBC results in reduction of liver biochemistries, a delay in histological progression, a delay in the development of varices and improvement in survival without liver transplantation.

Keywords: antimitochondrial antibody; liver transplantation; primary biliary cirrhosis; ursodeoxycholic acid.

Publication types

  • Review

MeSH terms

  • Cholagogues and Choleretics / therapeutic use
  • Humans
  • Liver Cirrhosis, Biliary / diagnosis*
  • Liver Cirrhosis, Biliary / therapy*
  • Liver Transplantation
  • Ursodeoxycholic Acid


  • Cholagogues and Choleretics
  • Ursodeoxycholic Acid