Clinical correlates of CENP-A and CENP-B antibodies in a large cohort of patients with systemic sclerosis

J Rheumatol. 2012 Apr;39(4):787-94. doi: 10.3899/rheum.111133.


Objective: To study the clinical phenotypes of centromeric proteins (CENP)-A- and CENP-B-positive patients with systemic sclerosis (SSc) and to compare them to anticentromere antibody (ACA)-positive and negative SSc patients.

Methods: Sera samples were collected from 802 patients with SSc enrolled in a multicenter cohort study. Antibodies to CENP-A and B were detected by ELISA, and ACA by indirect immunofluorescence. Associations with clinical and other serological manifestations of SSc were investigated.

Results: CENP-A antibodies were detected in 276 (34%), CENP-B in 286 (36%), and ACA in 279 (35%) patients. Patients having ACA, CENP-A, and/or CENP-B resembled each other and differed from the remainder of the cohort in the following respects: older chronologically and at disease onset; more commonly women; more likely to have limited disease and lower skin scores; less likely to have finger ulcers, digital tuft resorption, or finger contractures; more likely to have pulmonary hypertension; less likely to have interstitial lung disease, scleroderma renal crisis, inflammatory arthritis, and inflammatory myositis; and having lower overall disease severity. CENP-A and/or B status was predictive of the extent of skin involvement over time. Patients with limited disease who were CENP-A-negative at baseline were more likely to progress to diffuse disease compared to CENP-A-positive patients (OR 2.55, 95% CI 1.37, 4.85, p = 0.004).

Conclusion: Clinical immunology laboratories are increasingly using high-throughput ELISA tests for CENP antibodies, with or without ACA detected by indirect immunofluorescence. The phenotype of CENP-A and/or B-positive patients is generally similar to that associated with ACA.

Publication types

  • Comparative Study
  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Antibodies, Antinuclear / biosynthesis
  • Antibodies, Antinuclear / blood
  • Autoantibodies / biosynthesis
  • Autoantibodies / blood*
  • Autoantigens / immunology*
  • Centromere Protein A
  • Centromere Protein B / immunology*
  • Chromosomal Proteins, Non-Histone / immunology*
  • Cohort Studies
  • Female
  • Humans
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Scleroderma, Systemic / blood
  • Scleroderma, Systemic / epidemiology*
  • Scleroderma, Systemic / immunology*
  • Skin Diseases / epidemiology
  • Skin Diseases / immunology
  • Skin Diseases / pathology


  • Antibodies, Antinuclear
  • Autoantibodies
  • Autoantigens
  • CENPA protein, human
  • CENPB protein, human
  • Centromere Protein A
  • Centromere Protein B
  • Chromosomal Proteins, Non-Histone
  • anticentromere antibody