TAMIMA MOHAMAD ABOU ARABI

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Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina

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Autor: AIKAWA, Nadia E. ×

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  • article 15 Citação(ões) na Scopus
    Childhood-onset systemic lupus erythematosus-related antiphospholipid syndrome: A multicenter study with 1519 patients
    (2020) ISLABA, Aline G.; MOTA, Licia M. H.; RIBEIRO, Maria Custodia M.; ARABI, Tamima M.; CIVIDATTI, Georgiana N.; QUEIROZ, Ligia B.; ANDRADE, Danieli C.; SAKAMOTO, Ana P.; TRINDADE, Vitor C.; NOVAK, Glaucia V.; MOLINARI, Beatriz C.; CAMPOS, Lucia M.; AIKAWA, Nadia E.; PEREIRA, Rosa M. R.; TERRERI, Maria T.; MAGALHA, Claudia S.; MARINI, Roberto; GOMES, Hugo R.; SILVA, Marco F.; OLIVEIRA, Sheila K.; SZTAJNBOK, Flavio R.; SACCHETTI, Silvana B.; BICA, Blanca E.; SENA, Evaldo G.; MORAES, Ana P.; SANTOS, Maria C.; ROBAZZI, Teresa C.; SPELLING, Paulo F.; SCHEIBEL, Iloite M.; CAVALCANTI, Andre S.; NAKA, Erica N.; GUIMARAES, Luciano J.; SANTOS, Flavia P.; SAMPAIO, Magda C.; BONFA, Eloisa; SILVA, Clovis A.
    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.