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(Reference retrieved automatically from Web of Science through information on FAPESP grant and its corresponding number as mentioned in the publication by the authors.)

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

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Author(s):
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Islaba, Aline G. [1, 2] ; Mota, Licia M. H. [2, 3] ; Ribeiro, Maria Custodia M. [1] ; Arabi, Tamima M. [4] ; Cividatti, Georgiana N. [4] ; Queiroz, Ligia B. [4] ; Andrade, Danieli C. [5] ; Sakamoto, Ana P. [6] ; Trindade, Vitor C. [4] ; Novak, Glaucia V. [4] ; Molinari, Beatriz C. [4] ; Campos, Lucia M. [4] ; Aikawa, Nadia E. [5] ; Pereira, Rosa M. R. [5] ; Terreri, Maria T. [6] ; Magalha, Claudia S. [7] ; Marini, Roberto [8] ; Gomes, Hugo R. [9] ; Silva, Marco F. [10] ; Oliveira, Sheila K. [11] ; Sztajnbok, Flavio R. [12] ; Sacchetti, Silvana B. [13] ; Bica, Blanca E. [14] ; Sena, Evaldo G. [15] ; Moraes, Ana P. [16] ; Santos, Maria C. [17] ; Robazzi, Teresa C. [18] ; Spelling, Paulo F. [19] ; Scheibel, Iloite M. [20] ; Cavalcanti, Andre S. [21] ; Naka, Erica N. [22] ; Guimaraes, Luciano J. [23] ; Santos, Flavia P. [24] ; Sampaio, Magda C. [25] ; Bonfa, Eloisa [5] ; Silva, Clovis A. [4] ; Syst, Brazilian Childhood Onset
Total Authors: 37
Affiliation:
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[1] Hosp Crianca Brasilia Jose Alencar, Pediat Rheumatol Unit, Brasilia, DF - Brazil
[2] Univ Brasilia, Postgrad Program Med Sci, Brasilia, DF - Brazil
[3] Univ Brasilia, Rheumatol Unit, Brasilia, DF - Brazil
[4] Univ Sao Paulo, Fac Med, Hosp Clin HCFMUSP, Pediat Rheumatol Unit, Childrens Inst, Sao Paulo - Brazil
[5] Univ Sao Paulo, Fac Med, Hosp Clin HCFMUSP, Div Rheumatol, Sao Paulo - Brazil
[6] Univ Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo - Brazil
[7] Sao Paulo State Univ UNESP, Div Pediat Rheumatol, Botucatu, SP - Brazil
[8] Univ Campinas UNICAMP, Pediat Rheumatol Unit, Campinas - Brazil
[9] Univ Sao Paulo, Ribeirao Preto Med Sch, Pediat Rheumatol Unit, Ribeirao Preto - Brazil
[10] Hosp Geral Fortaleza, Pediat Rheumatol Unit, Fortaleza, Ceara - Brazil
[11] Rio de Janeiro Fed Univ IPPMG UFRJ, Pediat Rheumatol Unit, Rio De Janeiro - Brazil
[12] Pedro Ernesto Univ Hosp, Pediat Rheumatol Unit, Rio De Janeiro - Brazil
[13] Irmandade Santa Casa Misericordia Sao Paulo, Pediat Rheumatol Unit, Sao Paulo - Brazil
[14] Univ Fed Rio de Janeiro, Hosp Univ Clementino Fraga Filho, Div Rheumatol, Rio De Janeiro - Brazil
[15] Lauro Vanderley Univ Hosp, Pediat Rheumatol Unit, Joao Pessoa, Paraiba - Brazil
[16] Fed Univ Para, Pediat Rheumatol Unit, Belem, Para - Brazil
[17] Hosp Darcy Vargas, Pediat Rheumatol Unit, Sao Paulo - Brazil
[18] Univ Fed Bahia, Pediat Rheumatol Unit, Salvador, BA - Brazil
[19] Hosp Evangel Curitiba, Pediat Rheumatol Unit, Curitiba, Parana - Brazil
[20] Hosp Crianca Conceicao, Pediat Rheumatol Unit, Porto Alegre, RS - Brazil
[21] Univ Fed Pernambuco, Pediat Rheumatol Unit, Recife, PE - Brazil
[22] Univ Fed Mato Grosso do Sul, Pediat Rheumatol Unit, Campo Grande, MS - Brazil
[23] Univ Brasilia, Pediat Rheumatol Unit, Brasilia, DF - Brazil
[24] Univ Fed Minas Gerais, Pediat Rheumatol Unit, Belo Horizonte, MG - Brazil
[25] Univ Sao Paulo, Fac Med, Hosp Clin HCFMUSP, Pediat Immunol Unit, Childrens Inst, Sao Paulo - Brazil
Total Affiliations: 25
Document type: Review article
Source: AUTOIMMUNITY REVIEWS; v. 19, n. 12 DEC 2020.
Web of Science Citations: 0
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. (AU)

FAPESP's process: 15/03756-4 - Assessment of relevance of blood levels of drugs in the monitoring rheumatic autoimmune diseases: safety, effectiveness and adherence to therapy
Grantee:Eloisa Silva Dutra de Oliveira Bonfá
Support Opportunities: Research Projects - Thematic Grants