A comprehensive evaluation of serum autoantibodies in primary biliary cirrhosis

Nancy Agmon-Levin, Yinon Shapira, Carlo Selmi, Ori Barzilai, Maya Ram, Martine Szyper-Kravitz, Sara Sella, Bat sheva Porat Katz, Pierre Youinou, Yves Renaudineau, Bruno Larida, Pietro Invernizzi, M. Eric Gershwin, Yehuda Shoenfeld

Research output: Contribution to journalArticlepeer-review


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-β2GPI, 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.

Original languageEnglish
Pages (from-to)55-58
Number of pages4
JournalJournal of Autoimmunity
Issue number1
StatePublished - Feb 2010


  • Anti-nuclear antibodies
  • Anti-phospholipid antibodies
  • Anti-prothrombin antibodies
  • Autoimmune cholangitis
  • Primary biliary cirrhosis


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