Fernanda J Fiorot1, Aline G Islabão2, Rosa M Pereira3, Maria T Terreri4, Claudia Saad-Magalhães5, Glaucia V Novak1, Beatriz C Molinari1, Ana P Sakamoto4, Nadia E Aikawa1,3, Lucia M Campos1, Octavio A Peracchi4, Simone Appenzeller6, Virgínia P Ferriani7, Marco F Silva8, Adriana R Fonseca9, Flávio R Sztajnbok10, Luciana B Paim11, Melissa M Fraga12, Eunice M Okuda13, Blanca E Bica14, Evaldo G Sena15, Ana J Moraes16, Ana M Rolim17, Paulo F Spelling18, Iloite M Scheibel19, André S Cavalcanti20, Erica N Matos21, Teresa C Robazzi22, Luciano J Guimarães23, Flávia P Santos24, Valeria C Ramos25, Magda Carneiro-Sampaio1, Eloisa Bonfá3, Clovis A Silva26,27. 1. 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, Sao Paulo, SP, 05403-000, Brazil. 2. Pediatric Rheumatology Unit, Hospital Jose Alencar, Brasilia, Brazil. 3. Division of Rheumatology Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil. 4. Pediatric Rheumatology Unit, Universidade Federal de Sao Paulo, Sao Paulo, Brazil. 5. Pediatric Rheumatology Division, Sao Paulo State University (UNESP), Botucatu, Brazil. 6. Pediatric Rheumatology Unit, University of Campinas (UNICAMP), Campinas, Brazil. 7. Pediatric Rheumatology Unit, Ribeirao Preto Medical School - University of Sao Paulo, Ribeirão Preto, Brazil. 8. Pediatric Rheumatology Unit, Hospital Geral de Fortaleza, Fortaleza, Brazil. 9. Pediatric Rheumatology Unit, Rio de Janeiro Federal University (IPPMG-UFRJ), Rio de Janeiro, Brazil. 10. Pediatric Rheumatology Unit, Pedro Ernesto University Hospital, Rio de Janeiro, Brazil. 11. Pediatric Rheumatology Unit, Albert Sabin Children's Hospital, Fortaleza, Brazil. 12. Pediatric Rheumatology Unit, Hospital Darcy Vargas, Sao Paulo, Brazil. 13. Pediatric Rheumatology Unit, Irmandade da Santa Casa de Misericórdia de Sao Paulo, Sao Paulo, Brazil. 14. Rheumatology Division - Universidade Federal do Rio de Janeiro, Hospital Universitário Clementino Fraga Filho, Rio de Janeiro, Brazil. 15. Pediatric Rheumatology Unit, Lauro Vanderley University Hospital, João Pessoa, Brazil. 16. Pediatric Rheumatology Unit, Federal University of Pará, Belém, Brazil. 17. Pediatric Rheumatology Unit, Obras Sociais Irmã Dulce, Salvador, Brazil. 18. Pediatric Rheumatology Unit, Hospital Evangélico de Curitiba, Curitiba, Brazil. 19. Pediatric Rheumatology Unit, Hospital Criança Conceição, Porto Alegre, Brazil. 20. Pediatric Rheumatology Unit, Federal University of Pernambuco, Recife, Brazil. 21. Pediatric Rheumatology Unit, Federal University of Mato Grosso do Sul, Campo Grande, Brazil. 22. Pediatric Rheumatology Unit, Federal University of Bahia, Salvador, Brazil. 23. Pediatric Rheumatology Unit, University of Brasilia, Brasília, Brazil. 24. Pediatric Rheumatology Unit, Federal University of Minas Gerais, Belo Horizonte, Brazil. 25. Pediatric Rheumatology Unit, Pontifícia Catholic University of Sorocaba, São Paulo, Brazil. 26. 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, Sao Paulo, SP, 05403-000, Brazil. clovisaasilva@gmail.com. 27. Division of Rheumatology Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil. clovisaasilva@gmail.com.
Abstract
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.
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.
Authors: Glaucia V Novak; Mariana Marques; Verena Balbi; Natali W S Gormezano; Kátia Kozu; Ana P Sakamoto; Rosa M R Pereira; Maria T Terreri; Claudia S Magalhães; Andressa Guariento; Adriana M E Sallum; Roberto Marini; Virginia Paes Leme Ferriani; Cássia Maria Barbosa; Tânia Caroline Monteiro de Castro; Valéria C Ramos; Eloisa Bonfá; Clovis A Silva Journal: Autoimmun Rev Date: 2016-12-14 Impact factor: 9.754
Authors: G V Novak; B C Molinari; J C Ferreira; A P Sakamoto; M T Terreri; R M R Pereira; C Saad-Magalhães; N E Aikawa; L M Campos; C A Len; S Appenzeller; V P Ferriani; M F Silva; S K Oliveira; A G Islabão; F R Sztajnbok; L B Paim; C M Barbosa; M C Santos; B E Bica; E G Sena; A J Moraes; A M Rolim; P F Spelling; I M Scheibel; A S Cavalcanti; E N Matos; T C Robazzi; L J Guimarães; F P Santos; C T Silva; E Bonfá; C A Silva Journal: Lupus Date: 2018-07-18 Impact factor: 2.911
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Authors: Magda Carneiro-Sampaio; Bernadete Lourdes Liphaus; Adriana Almeida Jesus; Clovis Artur A Silva; João Bosco Oliveira; Maria Helena Kiss Journal: J Clin Immunol Date: 2008-04-11 Impact factor: 8.317
Authors: Helen E Foster; Christiaan Scott; Carl J Tiderius; Matthew B Dobbs Journal: Best Pract Res Clin Rheumatol Date: 2020-07-26 Impact factor: 4.098
Authors: D Nikolopoulos; M Kostopoulou; A Pieta; T Karageorgas; D Tseronis; K Chavatza; S Flouda; P Rapsomaniki; A Banos; E Kremasmenou; V Tzavara; P Katsimbri; A Fanouriakis; D T Boumpas Journal: Lupus Date: 2020-02-27 Impact factor: 2.911