Anti-mitochondrial antibodies and other immunological tests in primary biliary cirrhosis

Eur J Gastroenterol Hepatol. 1999 Jun;11(6):595-601. doi: 10.1097/00042737-199906000-00003.


Primary biliary cirrhosis (PBC) has been classified as an autoimmune disease. It is characterized by a multitude of immune-mediated symptoms and phenomena. Humoral autoimmunity directed against mitochondrial auto-antigens or structures cross-reactive with these proteins represents the basis of one of the most specific tests in any autoimmune disease: the detection of auto-antibodies against pyruvate dehydrogenase. The molecular cloning and expression of antigens of the ketoacid dehydrogenase complex has led to the establishment of highly specific and reliable testing systems for anti-mitochondrial antibodies (AMA). The characterization of specific antinuclear auto-antibodies has contributed to the diagnosis of AMA-negative PBC and is an important marker in overlap syndromes of PBC and other autoimmune liver diseases.

Publication types

  • Review

MeSH terms

  • Antibodies, Anti-Idiotypic*
  • Antibodies, Antinuclear / immunology
  • Antibody Formation
  • Autoimmune Diseases / immunology*
  • Autoimmunity
  • Humans
  • Ketoglutarate Dehydrogenase Complex / immunology
  • Liver Cirrhosis, Biliary / immunology*
  • Mitochondria, Liver / immunology*


  • Antibodies, Anti-Idiotypic
  • Antibodies, Antinuclear
  • Ketoglutarate Dehydrogenase Complex