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

Author(s)

Mendes, Cristiane ^{[1, 2]} ; Viana, Vilma S. T. ^[1] ; Pasoto, Sandra G. ^[1] ; Leon, Elaine P. ^[1] ; Bonfa, Eloisa ^[1] ; Sampaio-Barros, Percival D. ^{[1, 3]}

Affiliation

^[1] Universidade Sao Paulo, Hosp Clin HCFMUSP, Faculdade Medicina, Div Rheumatol, Sao Paulo, SP - Brazil

^[2] Universidade Fed Alfenas, Alfenas - Brazil

^[3] Universidade Sao Paulo, Disciplina Reumatol, Ave . Arnaldo 455, sala 3142, Cerqueira Cesar, BR-01246-903 Sao Paulo, SP - Brazil

• Document type: Journal article
• Source: CLINICAL RHEUMATOLOGY; v. 39, n. 1, p. 9-17, JAN 2020.
• Web of Science Citations: 0

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. (AU)

• FAPESP's process: 15/03756-4 - Assessment of relevance of blood levels of drugs in the monitoring rheumatic autoimmune diseases: safety, effectiveness and adherence to therapy
• Grantee: Eloisa Silva Dutra de Oliveira Bonfá
• Support Opportunities: Research Projects - Thematic Grants