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Clinical and laboratory features of African-Brazilian patients with systemic sclerosis

Abstract

Objective

African-Brazilians comprise a group of blacks and “pardos.” As racial differences can be associated with distinct presentations, we evaluated the clinical and serological associations of African-Brazilians with systemic sclerosis (SSc).

Methods

Sera from 260 adult SSc patients (203 whites and 57 African-Brazilians) were evaluated. Patients with overlap syndromes were excluded. Clinical and demographic data were obtained from an electronic register database. Laboratory analysis included the following: anti-CENP-A/CENP-B, Scl70, RNA polymerase III, Ku, fibrillarin, Th/To, PM-Scl75, and PM-Scl100 by line immunoassay and anti-nuclear antibodies (ANA) by indirect immunofluorescence (IIF) on HEp-2 cells.

Results

African-Brazilian SSc patients presented shorter disease duration (12.8 ± 6.5 vs. 15.9 ± 8.1 years, p = 0.009), higher frequency of nucleolar ANA pattern (28% vs. 13%, p = 0.008), and lower frequencies of centromeric ANA pattern (14% vs. 29%, p = 0.026) and CENP-B (18% vs. 34%, p = 0.017), as well as an association with severe interstitial lung disease (58% vs. 43%; p = 0.044). Further comparison of ethnic groups according to subsets revealed that diffuse SSc African-Brazilian patients presented higher frequency of pulmonary hypertension (p = 0.017), heart involvement (p = 0.037), nucleolar ANA pattern (p = 0.036), anti-fibrillarin antibodies (p = 0.037), and higher mortality (48% vs. 19%; p = 0.009). A different pattern was observed for the limited subset with solely a lower frequency of esophageal involvement (p = 0.050) and centromeric ANA pattern (p = 0.049). Survival analysis showed that African-Brazilians had a higher mortality, when adjusted for age, gender, and clinical subset (RR 2.06, CI 95% 1.10–3.83, p = 0.023).

Conclusion

African-Brazilians have distinct characteristics according to clinical subset and an overall more severe SSc than whites, similar to the blacks from other countries.

Key Points
African-Brazilian SSc patients were associated with severe interstitial lung disease and nucleolar ANA pattern when compared to white SSc patients.
When disease subsets were considered, African-Brazilian patients with diffuse SSc presented association with pulmonary hypertension, heart involvement, nucleolar ANA pattern, and anti-fibrillarin antibodies.
White SSc patients were associated with centromeric ANA pattern.
Survival analysis at 5, 10, 15, and 20 years, adjusted for age, gender, and disease subset, was significantly worse in African-Brazilian SSc patients.

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Author information

CM collected the data, performed the statistical analysis, analyzed the results, and wrote the manuscript; VST and EPL supervised the laboratory data and analysis; SGP contributed to the writing and critically reviewed the manuscript; EB and PDSB designed, analyzed the results, critically reviewed the manuscript, and wrote the final version. All authors read and approved the final manuscript.

Correspondence to Percival D. Sampaio-Barros.

Ethics declarations

This study was approved by the local research ethics committee (no. 1.627.446).

Disclosures

None.

Ethical approval

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Mendes, C., Viana, V.S.T., Pasoto, S.G. et al. Clinical and laboratory features of African-Brazilian patients with systemic sclerosis. Clin Rheumatol 39, 9–17 (2020) doi:10.1007/s10067-019-04575-5

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Keywords

  • African-Brazilians
  • Autoantibodies
  • Diffuse scleroderma
  • Ethnicity
  • Limited scleroderma
  • Systemic sclerosis