Diagnosis of primary biliary cirrhosis

Best Pract Res Clin Gastroenterol. 2011 Dec;25(6):701-12. doi: 10.1016/j.bpg.2011.10.005.


Primary biliary cirrhosis is the archetypal autoimmune liver disease, with the disease label describing a chronic granulomatous lymphocytic small bile duct cholangitis, which now most commonly presents asymptomatically and at an early pre-cirrhotic stage. Disease is more common than thought, with 1 in 1000 women over the age of 40 affected. Characteristic immunologic features of the disease assist clinicians in ready non-invasive diagnosis of patients, even if asymptomatic with only anicteric/cholestatic liver biochemical profiles. Over 90% of patients are anti-mitochondrial antibody positive, and for those negative, a significant proportion have highly specific anti-nuclear antibody profiles. Liver biopsy remains useful in certain settings where clarity is needed to confirm diagnosis, exclude alternative disease, and assess the relative contribution of PBC to other co-existent liver injury, and seeks to demonstrate in particular the classic bile duct lesions, as well as the degree of interface activity.

Publication types

  • Review

MeSH terms

  • Alkaline Phosphatase / blood
  • Antibodies, Antinuclear / blood
  • Autoimmune Diseases / blood
  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / immunology
  • Biopsy
  • Humans
  • Liver Cirrhosis, Biliary / blood
  • Liver Cirrhosis, Biliary / diagnosis*
  • Liver Cirrhosis, Biliary / immunology
  • Mitochondria / immunology
  • gamma-Glutamyltransferase / blood


  • Antibodies, Antinuclear
  • gamma-Glutamyltransferase
  • Alkaline Phosphatase