Childhood-onset systemic lupus erythematosus-related antiphospholipid syndrome: A multicenter study with 1519 patients.

Autor: Islabão AG; 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., Mota LMH; Post-graduation Program in Medical Science, University of Brasilia, Brasília, BR, Brazil; Rheumatology Unit, University of Brasilia, Brasília, BR, Brazil., Ribeiro MCM; Pediatric Rheumatology Unit, Hospital da Criança de Brasília Jose Alencar, Brasília, BR, Brazil., Arabi TM; Pediatric Rheumatology Unit, Children's Institute, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, BR, Brazil., Cividatti GN; Pediatric Rheumatology Unit, Children's Institute, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, BR, Brazil., Queiroz LB; Pediatric Rheumatology Unit, Children's Institute, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, BR, Brazil., Andrade DC; Division of Rheumatology Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, BR, Brazil., Sakamoto AP; Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil., Trindade VC; Pediatric Rheumatology Unit, Children's Institute, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, BR, Brazil., Novak GV; Pediatric Rheumatology Unit, Children's Institute, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, BR, Brazil., Molinari BC; Pediatric Rheumatology Unit, Children's Institute, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, BR, Brazil., Campos LM; Pediatric Rheumatology Unit, Children's Institute, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, BR, Brazil., Aikawa NE; Division of Rheumatology Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, BR, Brazil., Pereira RMR; Division of Rheumatology Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, BR, Brazil., Terreri MT; Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil., Magalhães CS; Pediatric Rheumatology Division, Sao Paulo State University (UNESP), Botucatu, BR, Brazil., Marini R; Pediatric Rheumatology Unit, University of Campinas (UNICAMP), Campinas, BR, Brazil., Gomes HR; Pediatric Rheumatology Unit, Ribeirão Preto Medical School - University of São Paulo, Ribeirão Preto, BR, Brazil., Silva MF; Pediatric Rheumatology Unit, Hospital Geral de Fortaleza, Fortaleza, BR, Brazil., Oliveira SK; Pediatric Rheumatology Unit, Rio de Janeiro Federal University (IPPMG-UFRJ), Rio de Janeiro, BR, Brazil., Sztajnbok FR; Pediatric Rheumatology Unit, Pedro Ernesto University Hospital, Rio de Janeiro, BR, Brazil., Sacchetti SB; Pediatric Rheumatology Unit, Irmandade da Santa Casa de Misericórdia de São Paulo, São Paulo, BR, Brazil., Bica BE; Rheumatology Division - Universidade Federal do Rio de Janeiro, Hospital Universitário Clementino Fraga Filho, Rio de Janeiro, BR, Brazil., Sena EG; Pediatric Rheumatology Unit, Lauro Vanderley University Hospital, João Pessoa, BR, Brazil., Moraes AP; Pediatric Rheumatology Unit, Federal University of Pará, Belém, BR, Brazil., Santos MC; Pediatric Rheumatology Unit, Hospital Darcy Vargas, São Paulo, BR, Brazil., Robazzi TC; Pediatric Rheumatology Unit, Federal University of Bahia, Salvador, BR, Brazil., Spelling PF; Pediatric Rheumatology Unit, Hospital Evangélico de Curitiba, Curitiba, BR, Brazil., Scheibel IM; Pediatric Rheumatology Unit, Hospital Criança Conceição, Porto Alegre, BR, Brazil., Cavalcanti AS; Pediatric Rheumatology Unit, Federal University of Pernambuco, Recife, BR, Brazil., Naka EN; Pediatric Rheumatology Unit, Federal University of Mato Grosso do Sul, Campo Grande, BR, Brazil., Guimarães LJ; Pediatric Rheumatology Unit, University of Brasília, Brasília, BR, Brazil., Santos FP; Pediatric Rheumatology Unit, Federal University of Minas Gerais, Belo Horizonte, BR, Brazil., Sampaio MC; Pediatric Immunology Unit, Children's Institute, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, BR, Brazil., Bonfá E; Division of Rheumatology Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, BR, Brazil., Silva CA; Pediatric Rheumatology Unit, Children's Institute, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, BR, Brazil.
Jazyk: angličtina
Zdroj: Autoimmunity reviews [Autoimmun Rev] 2020 Dec; Vol. 19 (12), pp. 102693. Date of Electronic Publication: 2020 Oct 22.
DOI: 10.1016/j.autrev.2020.102693
Abstrakt: 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.
(Copyright © 2020 Elsevier B.V. All rights reserved.)
Databáze: MEDLINE
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