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Objective: To evaluate the influence of ethnicity in presentation of childhood-onset systemic lupus erythematosus (cSLE) patients. Methods: This multicenter study included cSLE patients (American College of Rheumatology criteria) followed in 27 Pediatric Rheumatology services of Brazil. Ethnicities were classified in four groups according to the parents’ and all four grandparents’ self-reported ethnicity. The statistical analysis was performed using the Bonferroni’s correction (p < 0.0027). Results: According to ethnic groups, 1537 cSLE patients were classified in Caucasian (n = 786), African-Latin American (n = 526), Asian (n = 8), and others/unknown (n = 217). Comparisons between 1312 African-Latin American and Caucasian revealed similar median age at cSLE diagnosis [12.2(2.6–18) vs. 12.1(0.3–18) years, p = 0.234], time interval to diagnosis [0.25(0–12) vs. 0.3(0–10) years, p = 0.034], and SLEDAI-2K score [14(0–55) vs. 14(0–63), p = 0.781] in both groups. The mean number of diagnostic criteria according to SLICC (6.47 ± 1.911 vs. 5.81 ± 1.631, p < 0.0001) and frequencies of maculopapular lupus rash (8% vs. 3%, p < 0.0001), palate oral ulcers (17% vs. 11%, p = 0.001), tongue oral ulcers (4% vs. 1%, p = 0.001), and nonscarring alopecia (29% vs. 16%, p < 0.0001) were significantly higher in African-Latin American, whereas malar rash (45% vs. 58%, p < 0.0001) was more frequent in Caucasian. The presence of anti-phospholipid antibody (23% vs. 12%, p < 0.0001), low complement levels (58% vs. 41%, p < 0.0001), and isolated direct Coombs test (10% vs. 5%, p = 0.001) was also significantly higher in the former group. Conclusions: Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable with Caucasian. Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of the former group. The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients.Key Points• Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable with Caucasian.• Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of African-Latin American cSLE patients.• African-Latin American cSLE patients had more often anti-phospholipid antibodies and hypocomplementemia.• The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients.
Pediatric Rheumatology Unit Children’s Institute Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo, Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César
Pediatric Rheumatology Unit Hospital Jose Alencar
Division of Rheumatology Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo
Pediatric Rheumatology Unit Universidade Federal de Sao Paulo
Pediatric Rheumatology Division Sao Paulo State University (UNESP)
Pediatric Rheumatology Unit University of Campinas (UNICAMP)
Pediatric Rheumatology Unit Ribeirao Preto Medical School – University of Sao Paulo
Pediatric Rheumatology Unit Hospital Geral de Fortaleza
Pediatric Rheumatology Unit Rio de Janeiro Federal University (IPPMG-UFRJ)
Pediatric Rheumatology Unit Pedro Ernesto University Hospital
Pediatric Rheumatology Unit Albert Sabin Children’s Hospital
Pediatric Rheumatology Unit Hospital Darcy Vargas
Pediatric Rheumatology Unit Irmandade da Santa Casa de Misericórdia de Sao Paulo
Rheumatology Division - Universidade Federal do Rio de Janeiro Hospital Universitário Clementino Fraga Filho
Pediatric Rheumatology Unit Lauro Vanderley University Hospital
Pediatric Rheumatology Unit Federal University of Pará
Pediatric Rheumatology Unit Obras Sociais Irmã Dulce
Pediatric Rheumatology Unit Hospital Evangélico de Curitiba
Pediatric Rheumatology Unit Hospital Criança Conceição
Pediatric Rheumatology Unit Federal University of Pernambuco
Pediatric Rheumatology Unit Federal University of Mato Grosso do Sul
Pediatric Rheumatology Unit Federal University of Bahia
Pediatric Rheumatology Unit University of Brasilia
Pediatric Rheumatology Unit Federal University of Minas Gerais
Pediatric Rheumatology Unit Pontifícia Catholic University of Sorocaba
Pediatric Rheumatology Division Sao Paulo State University (UNESP)
