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Disease presentation of 1312 childhood-onset systemic lupus erythematosus: influence of ethnicity

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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.

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Acknowledgments

We thank the colleagues of all 27 Pediatric Rheumatology services. Brazilian Childhood-onset Systemic Lupus Erythematosus Group.

Funding

This study was financially supported by grants from Fundação de Amparo à Pesquisa do Estado de Sao Paulo (FAPESP 2015/03756-4 to EB and CAS), Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq 303752/2015-7 to MTT, 301805/2013-0 to RMRP, 305068/2014-8 to EB and 303422/2015-7 to CAS), Federico Foundation (to RMRP, EB and CAS), and by Núcleo de Apoio à Pesquisa “Saúde da Criança e do Adolescente” da USP (NAP-CriAd) to CAS.

Author information

Authors and Affiliations

  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

    Fernanda J. Fiorot, Glaucia V. Novak, Beatriz C. Molinari, Nadia E. Aikawa, Lucia M. Campos, Magda Carneiro-Sampaio & Clovis A. Silva

  2. Pediatric Rheumatology Unit, Hospital Jose Alencar, Brasilia, Brazil

    Aline G. Islabão

  3. Division of Rheumatology Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil

    Rosa M. Pereira, Nadia E. Aikawa, Eloisa Bonfá & Clovis A. Silva

  4. Pediatric Rheumatology Unit, Universidade Federal de Sao Paulo, Sao Paulo, Brazil

    Maria T. Terreri, Ana P. Sakamoto & Octavio A. Peracchi

  5. Pediatric Rheumatology Division, Sao Paulo State University (UNESP), Botucatu, Brazil

    Claudia Saad-Magalhães

  6. Pediatric Rheumatology Unit, University of Campinas (UNICAMP), Campinas, Brazil

    Simone Appenzeller

  7. Pediatric Rheumatology Unit, Ribeirao Preto Medical School – University of Sao Paulo, Ribeirão Preto, Brazil

    Virgínia P. Ferriani

  8. Pediatric Rheumatology Unit, Hospital Geral de Fortaleza, Fortaleza, Brazil

    Marco F. Silva

  9. Pediatric Rheumatology Unit, Rio de Janeiro Federal University (IPPMG-UFRJ), Rio de Janeiro, Brazil

    Adriana R. Fonseca

  10. Pediatric Rheumatology Unit, Pedro Ernesto University Hospital, Rio de Janeiro, Brazil

    Flávio R. Sztajnbok

  11. Pediatric Rheumatology Unit, Albert Sabin Children’s Hospital, Fortaleza, Brazil

    Luciana B. Paim

  12. Pediatric Rheumatology Unit, Hospital Darcy Vargas, Sao Paulo, Brazil

    Melissa M. Fraga

  13. Pediatric Rheumatology Unit, Irmandade da Santa Casa de Misericórdia de Sao Paulo, Sao Paulo, Brazil

    Eunice M. Okuda

  14. Rheumatology Division - Universidade Federal do Rio de Janeiro, Hospital Universitário Clementino Fraga Filho, Rio de Janeiro, Brazil

    Blanca E. Bica

  15. Pediatric Rheumatology Unit, Lauro Vanderley University Hospital, João Pessoa, Brazil

    Evaldo G. Sena

  16. Pediatric Rheumatology Unit, Federal University of Pará, Belém, Brazil

    Ana J. Moraes

  17. Pediatric Rheumatology Unit, Obras Sociais Irmã Dulce, Salvador, Brazil

    Ana M. Rolim

  18. Pediatric Rheumatology Unit, Hospital Evangélico de Curitiba, Curitiba, Brazil

    Paulo F. Spelling

  19. Pediatric Rheumatology Unit, Hospital Criança Conceição, Porto Alegre, Brazil

    Iloite M. Scheibel

  20. Pediatric Rheumatology Unit, Federal University of Pernambuco, Recife, Brazil

    André S. Cavalcanti

  21. Pediatric Rheumatology Unit, Federal University of Mato Grosso do Sul, Campo Grande, Brazil

    Erica N. Matos

  22. Pediatric Rheumatology Unit, Federal University of Bahia, Salvador, Brazil

    Teresa C. Robazzi

  23. Pediatric Rheumatology Unit, University of Brasilia, Brasília, Brazil

    Luciano J. Guimarães

  24. Pediatric Rheumatology Unit, Federal University of Minas Gerais, Belo Horizonte, Brazil

    Flávia P. Santos

  25. Pediatric Rheumatology Unit, Pontifícia Catholic University of Sorocaba, São Paulo, Brazil

    Valeria C. Ramos

Authors
  1. Fernanda J. Fiorot
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  2. Aline G. Islabão
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  3. Rosa M. Pereira
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  4. Maria T. Terreri
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  5. Claudia Saad-Magalhães
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  6. Glaucia V. Novak
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  7. Beatriz C. Molinari
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  8. Ana P. Sakamoto
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  9. Nadia E. Aikawa
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  10. Lucia M. Campos
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  11. Octavio A. Peracchi
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  12. Simone Appenzeller
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  13. Virgínia P. Ferriani
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  14. Marco F. Silva
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  15. Adriana R. Fonseca
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  16. Flávio R. Sztajnbok
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  17. Luciana B. Paim
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  18. Melissa M. Fraga
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  19. Eunice M. Okuda
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  20. Blanca E. Bica
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  21. Evaldo G. Sena
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  22. Ana J. Moraes
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  23. Ana M. Rolim
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  24. Paulo F. Spelling
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  25. Iloite M. Scheibel
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  26. André S. Cavalcanti
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  27. Erica N. Matos
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  28. Teresa C. Robazzi
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  29. Luciano J. Guimarães
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  30. Flávia P. Santos
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  31. Valeria C. Ramos
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  32. Magda Carneiro-Sampaio
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  33. Eloisa Bonfá
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  34. Clovis A. Silva
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Consortia

Brazilian Childhood-onset Systemic Lupus Erythematosus Group

Contributions

1. Substantial contributions to study conception and design: Rosa M. Pereira, Maria T. Terreri, Claudia Saad-Magalhães, Glaucia V. Novak, Eloisa Bonfá, Clovis A. Silva;

2. Substantial contributions to data acquisition: Fernanda J. Fiorot, Aline G. Islabão, Rosa M. Pereira, Maria T. Terreri, Claudia Saad-Magalhães, Glaucia V. Novak, Beatriz C. Molinari, Ana P. Sakamoto, Nadia E. Aikawa, Lucia M. Campos, Octavio A. Peracchi, Simone Appenzeller, Virgínia P. Ferriani, Marco F. Silva, Adriana R. Fonseca, Flávio R. Sztajnbok, Luciana B. Paim, Melissa M. Fraga, Eunice M. Okuda, Blanca E. Bica, Evaldo G. Sena, Ana J. Moraes, Ana M. Rolim, Paulo F. Spelling, Iloite M. Scheibel, André S. Cavalcanti, Erica N. Matos, Teresa C. Robazzi, Luciano J. Guimarães, Flávia P. Santos, Valeria C. Ramos, Magda Carneiro-Sampaio, Eloisa Bonfá, Clovis A. Silva;

3. Substantial contributions to analysis and interpretation of data: Fernanda J. Fiorot, Aline G. Islabão, Glaucia V. Novak, Beatriz C. Molinari, Eloisa Bonfá, Clovis A. Silva;

4. Final approval of the article: Fernanda J. Fiorot, Aline G. Islabão, Rosa M. Pereira, Maria T. Terreri, Claudia Saad-Magalhães, Glaucia V. Novak, Beatriz C. Molinari, Ana P. Sakamoto, Nadia E. Aikawa, Lucia M. Campos, Octavio A. Peracchi, Simone Appenzeller, Virgínia P. Ferriani, Marco F. Silva, Adriana R. Fonseca, Flávio R. Sztajnbok, Luciana B. Paim, Melissa M. Fraga, Eunice M. Okuda, Blanca E. Bica, Evaldo G. Sena, Ana J. Moraes, Ana M. Rolim, Paulo F. Spelling, Iloite M. Scheibel, André S. Cavalcanti, Erica N. Matos, Teresa C. Robazzi, Luciano J. Guimarães, Flávia P. Santos, Valeria C. Ramos, Magda Carneiro-Sampaio, Eloisa Bonfá, Clovis A. Silva

Corresponding author

Correspondence to Clovis A. Silva.

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Fiorot, F.J., Islabão, A.G., Pereira, R.M. et al. Disease presentation of 1312 childhood-onset systemic lupus erythematosus: influence of ethnicity. Clin Rheumatol 38, 2857–2863 (2019). https://doi.org/10.1007/s10067-019-04631-0

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