Aline G Islabão1, Licia M H Mota2, Maria Custodia M Ribeiro3, Tamima M Arabi4, Georgiana N Cividatti4, Ligia B Queiroz4, Danieli C Andrade5, Ana P Sakamoto6, Vitor C Trindade4, Glaucia V Novak4, Beatriz C Molinari4, Lucia M Campos4, Nádia E Aikawa5, Rosa M R Pereira5, Maria T Terreri6, Claudia S Magalhães7, Roberto Marini8, Hugo R Gomes9, Marco F Silva10, Sheila K Oliveira11, Flavio R Sztajnbok12, Silvana B Sacchetti13, Blanca E Bica14, Evaldo G Sena15, Ana P Moraes16, Maria C Santos17, Teresa C Robazzi18, Paulo F Spelling19, Iloite M Scheibel20, Andre S Cavalcanti21, Erica N Naka22, Luciano J Guimarães23, Flavia P Santos24, Magda C Sampaio25, Eloisa Bonfá5, Clovis A Silva4. 1. Pediatric Rheumatology Unit, Hospital da Criança de Brasília Jose Alencar, Brasília, BR, Brazil; Post-graduation Program in Medical Science, University of Brasilia, Brasília, BR, Brazil. Electronic address: aline.reumato@hotmail.com. 2. Post-graduation Program in Medical Science, University of Brasilia, Brasília, BR, Brazil; Rheumatology Unit, University of Brasilia, Brasília, BR, Brazil. 3. Pediatric Rheumatology Unit, Hospital da Criança de Brasília Jose Alencar, Brasília, BR, Brazil. 4. Pediatric Rheumatology Unit, Children's Institute, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, BR, Brazil. 5. Division of Rheumatology Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, BR, Brazil. 6. Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil. 7. Pediatric Rheumatology Division, Sao Paulo State University (UNESP), Botucatu, BR, Brazil. 8. Pediatric Rheumatology Unit, University of Campinas (UNICAMP), Campinas, BR, Brazil. 9. Pediatric Rheumatology Unit, Ribeirão Preto Medical School - University of São Paulo, Ribeirão Preto, BR, Brazil. 10. Pediatric Rheumatology Unit, Hospital Geral de Fortaleza, Fortaleza, BR, Brazil. 11. Pediatric Rheumatology Unit, Rio de Janeiro Federal University (IPPMG-UFRJ), Rio de Janeiro, BR, Brazil. 12. Pediatric Rheumatology Unit, Pedro Ernesto University Hospital, Rio de Janeiro, BR, Brazil. 13. Pediatric Rheumatology Unit, Irmandade da Santa Casa de Misericórdia de São Paulo, São Paulo, BR, Brazil. 14. Rheumatology Division - Universidade Federal do Rio de Janeiro, Hospital Universitário Clementino Fraga Filho, Rio de Janeiro, BR, Brazil. 15. Pediatric Rheumatology Unit, Lauro Vanderley University Hospital, João Pessoa, BR, Brazil. 16. Pediatric Rheumatology Unit, Federal University of Pará, Belém, BR, Brazil. 17. Pediatric Rheumatology Unit, Hospital Darcy Vargas, São Paulo, BR, Brazil. 18. Pediatric Rheumatology Unit, Federal University of Bahia, Salvador, BR, Brazil. 19. Pediatric Rheumatology Unit, Hospital Evangélico de Curitiba, Curitiba, BR, Brazil. 20. Pediatric Rheumatology Unit, Hospital Criança Conceição, Porto Alegre, BR, Brazil. 21. Pediatric Rheumatology Unit, Federal University of Pernambuco, Recife, BR, Brazil. 22. Pediatric Rheumatology Unit, Federal University of Mato Grosso do Sul, Campo Grande, BR, Brazil. 23. Pediatric Rheumatology Unit, University of Brasília, Brasília, BR, Brazil. 24. Pediatric Rheumatology Unit, Federal University of Minas Gerais, Belo Horizonte, BR, Brazil. 25. Pediatric Immunology Unit, Children's Institute, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, BR, Brazil.
Abstract
OBJECTIVE: To assess childhood-onset systemic lupus erythematosus-related antiphospholipid syndrome(cSLE-APS) in a large Brazilian population. METHODS: A retrospective observational cohort study was carried-out in 27 Pediatric Rheumatology university centers, including 1519 cSLE patients. RESULTS: cSLE-APS was observed in 67/1519 (4%) and was diagnosed at disease onset in 39/67 (58%). The median disease duration was 4.9 (0-17) years. Thrombosis recurrences were evidenced in 18/67 (27%) cSLE-APS patients. The most frequent thrombosis sites in cSLE-APS patients were: venous thrombosis in 40/67 (60%), especially deep vein thrombosis in 29/40 (72%); arterial thrombosis in 35/67 (52%), particularly stroke; small vessels thrombosis in 9/67 (13%) and mixed thrombosis in 3/67 (4%). Pregnancy morbidity was observed in 1/67 (1%). Non-thrombotic manifestation associated to cSLE-APS occurred in 21/67 (31%), mainly livedo reticularis in 14/67 (21%), valvar thickening in 4/67 (6%) and valvar vegetations not related to infections in 2/67 (3%). None of them had catastrophic APS. Further analysis demonstrated that the median of SLICC/ACR-DI [1(0-5) vs. 0(0-7),p < 0.0001] was significantly higher in cSLE-APS patients compared to cSLE without APS. The frequencies of cerebrovascular disease (40% vs. 1%,p < 0.0001), polyneuropathy (9% vs. 1%,p < 0.0001), SLICC/ACR-DI ≥1 (57% vs. 27%, p < 0.0001) and intravenous cyclophosphamide use (59% vs. 37%, p < 0.0001) were significantly higher in the former group. CONCLUSIONS: Our large multicenter study demonstrated that cSLE-APS was a rare condition, occurring during disease course with a high accrual damage. Central and peripheral neuropsychiatric involvements were distinctive features of this autoimmune thrombosis.
OBJECTIVE: To assess childhood-onset systemic lupus erythematosus-related antiphospholipid syndrome(cSLE-APS) in a large Brazilian population. METHODS: A retrospective observational cohort study was carried-out in 27 Pediatric Rheumatology university centers, including 1519 cSLE patients. RESULTS:cSLE-APS was observed in 67/1519 (4%) and was diagnosed at disease onset in 39/67 (58%). The median disease duration was 4.9 (0-17) years. Thrombosis recurrences were evidenced in 18/67 (27%) cSLE-APSpatients. The most frequent thrombosis sites in cSLE-APSpatients were: venous thrombosis in 40/67 (60%), especially deep vein thrombosis in 29/40 (72%); arterial thrombosis in 35/67 (52%), particularly stroke; small vessels thrombosis in 9/67 (13%) and mixed thrombosis in 3/67 (4%). Pregnancy morbidity was observed in 1/67 (1%). Non-thrombotic manifestation associated to cSLE-APS occurred in 21/67 (31%), mainly livedo reticularis in 14/67 (21%), valvar thickening in 4/67 (6%) and valvar vegetations not related to infections in 2/67 (3%). None of them had catastrophic APS. Further analysis demonstrated that the median of SLICC/ACR-DI [1(0-5) vs. 0(0-7),p < 0.0001] was significantly higher in cSLE-APSpatients compared to cSLE without APS. The frequencies of cerebrovascular disease (40% vs. 1%,p < 0.0001), polyneuropathy (9% vs. 1%,p < 0.0001), SLICC/ACR-DI ≥1 (57% vs. 27%, p < 0.0001) and intravenous cyclophosphamide use (59% vs. 37%, p < 0.0001) were significantly higher in the former group. CONCLUSIONS: Our large multicenter study demonstrated that cSLE-APS was a rare condition, occurring during disease course with a high accrual damage. Central and peripheral neuropsychiatric involvements were distinctive features of this autoimmune thrombosis.