Anti-fibrillarin antibody in African American patients with systemic sclerosis: Immunogenetics, clinical features, and survival analysis

Roozbeh Sharif, Marvin J. Fritzler, Maureen D. Mayes, Emilio B. Gonzalez, Terry A. McNearney, Hilda Draeger, Murray Baron, Daniel E. Furst, Dinesh K. Khanna, Deborah J. Del Junco, Jerry A. Molitor, Elena Schiopu, Kristine Phillips, James R. Seibold, Richard M. Silver, Robert W. Simms, Marilyn Perry, Carlos Rojo, Julio Charles, Xiaodong ZhouSandeep K. Agarwal, John D. Reveille, Shervin Assassi, Frank C. Arnett, Janet E. Pope, Janet Markland, David Robinson, Niall Jones, Nader Khalidi, Peter Docherty, Maysan Abu-Hakima, Sharon LeClercq, Evelyn Sutton, Douglas Smith, Jean Pierre Mathieu, Alejandra Masetto, Elzbieta Kaminska, Sophie Ligier

Research output: Contribution to journalArticlepeer-review

38 Scopus citations


Objective. Anti-U3-RNP, or anti-fibrillarin antibodies (AFA), are detected more frequently among African American (AA) patients with systemic sclerosis (SSc) compared to other ethnic groups and are associated with distinct clinical features. We examined the immunogenetic, clinical, and survival correlates of AFA in a large group of AA patients with SSc. Methods. Overall, 278 AA patients with SSc and 328 unaffected AA controls were enrolled from 3 North American cohorts. Clinical features, autoantibody profile, and HLA class II genotyping were determined. To compare clinical manifestations, relevant clinical features were adjusted for disease duration. Cox proportional hazards regression was used to determine the effect of AFA on survival. Results. Fifty (18.5%) AA patients had AFA. After Bonferroni correction, HLA-DRB1*08:04 was associated with AFA, compared to unaffected AA controls (OR 11.5, p < 0.0001) and AFA-negative SSc patients (OR 5.2, p = 0.0002). AFA-positive AA patients had younger age of disease onset, higher frequency of digital ulcers, diarrhea, pericarditis, higher Medsger perivascular and lower Medsger lung severity indices (p = 0.004, p = 0.014, p = 0.019, p = 0.092, p = 0.006, and p = 0.016, respectively). After adjustment for age at enrollment, AFA-positive patients did not have different survival compared to patients without AFA (p = 0.493). Conclusion. Our findings demonstrate strong association between AFA and HLA-DRB1*08:04 allele in AA patients with SSc. AA SSc patients with AFA had younger age of onset, higher frequency of digital ulcers, pericarditis and severe lower gastrointestinal involvement, but less severe lung involvement compared to AA patients without AFA. Presence of AFA did not change survival. The Journal of Rheumatology

Original languageEnglish (US)
Pages (from-to)1622-1630
Number of pages9
JournalJournal of Rheumatology
Issue number8
StatePublished - Aug 2011


  • Anti-U3-RNP
  • Digital ulcer
  • Genisos
  • HLA-DRB1
  • Scleroderma
  • Scleroderma family registry

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Immunology


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