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Research ArticleArticle

Primary Biliary Cirrhosis (PBC), PBC Autoantibodies, and Hepatic Parameter Abnormalities in a Large Population of Systemic Sclerosis Patients

SHERVIN ASSASSI, MARVIN J. FRITZLER, FRANK C. ARNETT, GARY L. NORMAN, KAIRAV R. SHAH, PRAVITT GOURH, NEIL MANEK, MARILYN PERRY, DEVI GANESH, MOHAMMAD H. RAHBAR and MAUREEN D. MAYES
The Journal of Rheumatology October 2009, 36 (10) 2250-2256; DOI: https://doi.org/10.3899/jrheum.090340
SHERVIN ASSASSI
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MARVIN J. FRITZLER
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FRANK C. ARNETT
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GARY L. NORMAN
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KAIRAV R. SHAH
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PRAVITT GOURH
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NEIL MANEK
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MARILYN PERRY
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DEVI GANESH
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MOHAMMAD H. RAHBAR
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MAUREEN D. MAYES
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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.

  • LIVER CIRRHOSIS
  • BILIARY
  • SCLERODERMA
  • SYSTEMIC SCLEROSIS

Footnotes

  • Supported by the National Institute of Arthritis/Musculoskeletal and Skin Diseases (N01-AR-02251 to Dr. Mayes, P50-AR054144 to Dr. Arnett); the National Institute of Health Clinical and Translational Sciences Award (1U54 RR 23417-01 to Dr. Arnett); and American College of Rheumatology Clinical Investigator Fellowship Award (to Dr. Assassi).

    • Accepted for publication May 7, 2009.
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The Journal of Rheumatology: 36 (10)
The Journal of Rheumatology
Vol. 36, Issue 10
1 Oct 2009
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Primary Biliary Cirrhosis (PBC), PBC Autoantibodies, and Hepatic Parameter Abnormalities in a Large Population of Systemic Sclerosis Patients
SHERVIN ASSASSI, MARVIN J. FRITZLER, FRANK C. ARNETT, GARY L. NORMAN, KAIRAV R. SHAH, PRAVITT GOURH, NEIL MANEK, MARILYN PERRY, DEVI GANESH, MOHAMMAD H. RAHBAR, MAUREEN D. MAYES
The Journal of Rheumatology Oct 2009, 36 (10) 2250-2256; DOI: 10.3899/jrheum.090340

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Primary Biliary Cirrhosis (PBC), PBC Autoantibodies, and Hepatic Parameter Abnormalities in a Large Population of Systemic Sclerosis Patients
SHERVIN ASSASSI, MARVIN J. FRITZLER, FRANK C. ARNETT, GARY L. NORMAN, KAIRAV R. SHAH, PRAVITT GOURH, NEIL MANEK, MARILYN PERRY, DEVI GANESH, MOHAMMAD H. RAHBAR, MAUREEN D. MAYES
The Journal of Rheumatology Oct 2009, 36 (10) 2250-2256; DOI: 10.3899/jrheum.090340
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