Diagnosis of primary biliary cirrhosis

Gideon M Hirschfield

Research output: Contribution to journalArticlepeer-review

29 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)701-12
Number of pages12
JournalBest Practice & Research: Clinical Gastroenterology
Issue number6
Publication statusPublished - Dec 2011


  • Alkaline Phosphatase
  • Antibodies, Antinuclear
  • Autoimmune Diseases
  • Biopsy
  • Humans
  • Liver Cirrhosis, Biliary
  • Mitochondria
  • gamma-Glutamyltransferase


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