A comprehensive evaluation of serum autoantibodies in primary biliary cirrhosis

J Autoimmun. 2010 Feb;34(1):55-8. doi: 10.1016/j.jaut.2009.08.009. Epub 2009 Nov 7.


In primary biliary cirrhosis (PBC) serum markers other than anti-mitochondrial antibodies (AMA) are promising in terms of disease severity and comorbidities, as well represented by anti-nuclear antibodies (ANA). The aim of the present study was thus to evaluate the prevalence and clinical significance of a large profile of serum autoantibodies in PBC sera. We utilized 69 sera from European patients with PBC (including 20 AMA-negative) and 297 sera from geographically and sex-matched healthy controls. All sera were tested for the presence of ANA and autoantibodies associated with thrombophilia, vasculitis, and gastrointestinal disease. Autoantibodies other than AMA were detected in 53/69 (76%) PBC sera vs. 105/297 (35%) among controls. The prevalence of ANA (targeting dsDNA, Sm, chromatin, ribosomal-P, RNP, SmRNP, SSA, SSB, and centromere) and thrombophilia-associated autoantibodies (i.e. anti-beta2GPI, phosphatydilserine, prothrombin) was common among patients with PBC. When clinical features were compared, the presence of anti-prothrombin IgM was associated with a worse prognosis as represented by a higher Mayo score. We demonstrate an increased prevalence of ANA and thrombophilia-associated autoantibodies in PBC sera and an association between the latter autoantibodies and PBC stage. The role of thrombophilia-associated antibodies will warrant further studies, based in particular on the incidence of portal hypertension at early stages of PBC.

MeSH terms

  • Aged
  • Antibodies, Antinuclear / blood*
  • Cross-Sectional Studies
  • Disease Progression
  • Female
  • Humans
  • Immunoglobulin M / blood
  • Liver Cirrhosis, Biliary / blood
  • Liver Cirrhosis, Biliary / diagnosis*
  • Liver Cirrhosis, Biliary / immunology*
  • Liver Cirrhosis, Biliary / physiopathology
  • Male
  • Middle Aged
  • Prognosis
  • Prothrombin / immunology
  • Risk Assessment
  • Thrombophilia
  • Vasculitis


  • Antibodies, Antinuclear
  • Immunoglobulin M
  • Prothrombin