Primary biliary cirrhosis (PBC), PBC autoantibodies, and hepatic parameter abnormalities in a large population of systemic sclerosis patients

J Rheumatol. 2009 Oct;36(10):2250-6. doi: 10.3899/jrheum.090340. Epub 2009 Sep 1.

Abstract

Objective: To investigate the diagnostic accuracy of antimitochondrial antibodies (AMA), sp100, and gp210 antibodies for primary biliary cirrhosis (PBC) in a large population of patients with systemic sclerosis (SSc); to examine concordance of these antibodies with subsets of SSc. Further, to assess the association of SSc-related antibodies with hepatic parameter abnormalities.

Methods: We obtained medical records to verify the diagnoses of SSc and PBC. Sera from all participants were examined for the presence of SSc- and PBC-related antibodies, as well as for abnormalities in hepatic parameters.

Results: We examined 817 patients with SSc, of whom 16 (2%) had confirmed PBC. The sensitivity and specificity of AMA by a MIT3 ELISA for PBC were 81.3% and 94.6%, respectively. Sp100 had a sensitivity and specificity of 31.3% and 97.4%, respectively, while gp210 had an even lower sensitivity. We were able to detect all PBC cases using AMA(MIT3) and sp100 as a combined marker, resulting in a significantly improved sensitivity of 100% (p = 0.042) with an incremental decrease in specificity to 92.6%. Independent of AMA or sp100 status, there was an association of anticentromere B (CENP-B) and anti-topoisomerase antibodies (ATA) with higher alkaline phosphatase levels (p = 0.051 and p = 0.003, respectively) while anti-RNA polymerase III (anti-RNAP) was associated with lower alkaline phosphatase levels (p = 0.019) among the patients with SSc.

Conclusion: Utilization of AMA(MIT3) and sp100 antibodies as a combined diagnostic marker leads to an improved detection of PBC in patients with SSc. CENP-B and ATA are associated with alkaline phosphatase elevation.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Alkaline Phosphatase / blood
  • Antigens, Nuclear / immunology
  • Autoantibodies / blood*
  • Autoantigens / immunology
  • Biomarkers / blood
  • Female
  • Humans
  • Liver / pathology*
  • Liver Cirrhosis, Biliary / blood*
  • Liver Cirrhosis, Biliary / diagnosis*
  • Liver Cirrhosis, Biliary / etiology
  • Male
  • Middle Aged
  • Mitochondria, Liver / immunology*
  • Platelet Membrane Glycoproteins / immunology
  • Registries
  • Retrospective Studies
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / immunology*
  • Scleroderma, Systemic / pathology*
  • Sensitivity and Specificity

Substances

  • Antigens, Nuclear
  • Autoantibodies
  • Autoantigens
  • Biomarkers
  • GP 210 protein, human
  • Platelet Membrane Glycoproteins
  • SP100 protein, human
  • Alkaline Phosphatase