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

被引：22

作者：

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
]}

机构：

[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

来源：

AUTOIMMUNITY REVIEWS | 2020年 / 19卷 / 12期

基金：

巴西圣保罗研究基金会;

关键词：

Childhood-onset systemic lupus erythematosus; Antiphospholipid syndrome; Neuropsychiatric; Thrombosis and cumulative damage; ANTIBODIES; CRITERIA; MANIFESTATIONS; CLASSIFICATION; CHILDREN; UPDATE; INDEX;

D O I：

10.1016/j.autrev.2020.102693

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

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.

引用

页数：5

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