Symptomatic polyautoimmunity at diagnosis of 1463 childhood-onset lupus: A Brazilian multicenter study

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Setoue, Debora N. ^[1] ; Pitta, Ana C. ^[1] ; Fiorot, Fernanda J. ^[1] ; Nastri, Mariana M. ^[1] ; Novak, Glaucia V. ^[1] ; Molinari, Beatriz C. ^[1] ; Oliveira, Juliana C. ^[1] ; Gormezano, Natali W. ^{[1, 2]} ; Sakamoto, Ana P. ^[3] ; Terreri, Maria T. ^[3] ; Pereira, Rosa M. ^[2] ; Saad-Magalhaes, Claudia ^[4] ; Sallum, Adriana M. ^[1] ; Kozu, Katia ^[1] ; Fraga, Melissa M. ^[3] ; Piotto, Daniela P. ^[3] ; Clemente, Gleice ^[3] ; Marini, Roberto ^[5] ; Gomes, Hugo R. ^[6] ; Rabelo-Junior, Carlos N. ^[7] ; Felix, Marta M. ^[8] ; Ribeiro, Maria C. ^[9] ; Almeida, Rozana G. ^[10] ; Assad, Ana P. ^[2] ; Sacchetti, Silvana B. ^[11] ; Barros, Leandra C. ^[12] ; Bonfa, Eloisa ^[2] ; Silva, Clovis A. ^{[1, 2]} ; Systemic, Brazilian Childhood-Onset

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^[1] Univ Sao Paulo, Fac Med, Hosp Clin HCFMUSP, Pediat Rheumatol Unit, Childrens Inst, Sao Paulo - Brazil

^[2] Univ Sao Paulo, Fac Med, Hosp Clin HCFMUSP, Div Rheumatol, Sao Paulo - Brazil

^[3] Univ Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo - Brazil

^[4] Sao Paulo State Univ UNESP, Div Pediat Rheumatol, Botucatu, SP - Brazil

^[5] State Univ Campinas UNICAMP, Pediat Rheumatol Unit, Campinas, SP - Brazil

^[6] Univ Sao Paulo, Ribeirao Preto Med Sch, Pediat Rheumatol Unit, Ribeirao Preto - Brazil

^[7] Hosp Geral Fortaleza, Pediat Rheumatol Unit, Fortaleza, Ceara - Brazil

^[8] Rio de Janeiro Fed Univ IPPMG UFRJ, Pediat Rheumatol Unit, Rio De Janeiro - Brazil

^[9] Hosp Jose Alencar, Pediat Rheumatol Unit, Brasilia, DF - Brazil

^[10] Pedro Ernesto Univ Hosp, Pediat Rheumatol Unit, Rio De Janeiro - Brazil

^[11] Irmandade Santa Casa Misericordia Sao Paulo, Pediat Rheumatol Unit, Sao Paulo - Brazil

^[12] Univ Fed Bahia, Pediat Rheumatol Unit, Salvador, BA - Brazil

• Tipo de documento: Artigo de Revisão
• Fonte: AUTOIMMUNITY REVIEWS; v. 17, n. 8, p. 836-839, AUG 2018.
• Citações Web of Science: 3

Resumo

Objective: To evaluate symptomatic polyautoimmunity (PA) at childhood-onset systemic lupus erythematosus (cSLE) diagnosis, and its association with demographic data, disease activity, clinical manifestations and laboratorial abnormalities in a large Brazilian cSLE population. Methods: A multicenter retrospective study was performed in 1463 cSLE(ACR criteria) patients from 27 Pediatric Rheumatology services. Symptomatic PA was defined according to the presence of more than one concomitant autoimmune disease(AD) and symptomatic multiple autoimmune syndrome(MAS) was defined as three or more AD. An investigator meeting was held to define the protocol. Demographic data, SLICC classification criteria and SLEDAI-2K were evaluated. Results: At cSLE diagnosis symptomatic PA was observed in 144/1463(9.8%) and symptomatic MAS occurred in solely 10/1463(0.7%). In the former group the more frequently observed associated AD were Hashimoto thyroiditis n = 42/144(29%), antiphospholipid syndrome n = 42/144(29%), autoimmune hepatitis n = 26/144(18%) and type 1 diabetes mellitus n = 23/144(15.9%). Further comparisons between cSLE patients with and without PA showed a higher median age(p = 0.016) and lower mean SLICC criteria (p = 0.039) in those with PA. Additionally, these cSLE patients had less renal involvement(35% vs. 44%, p = 0.038) and red blood cell cast(6% vs. 12%, p = 0.042) and more antiphospholipid antibodies(29% vs. 15%, p < 0.0001). Conclusions: Approximately 10% of cSLE had symptomatic PA at diagnosis, particularly endocrine autoimmune disorders and antiphospholipid syndrome. Lupus was characterized by a mild disease onset and MAS was infrequently evidenced. Further studies are necessary to determine if this subgroup of cSLE patients have a distinct genetic background with a less severe disease and a better long-term outcome. (AU)

• Processo FAPESP: 15/03756-4 - Avaliação da relevância dos níveis sanguíneos de drogas utilizadas em doenças autoimunes reumatológicas no acompanhamento da segurança, eficácia e aderência à terapêutica
• Beneficiário: Eloisa Silva Dutra de Oliveira Bonfá
• Modalidade de apoio: Auxílio à Pesquisa - Temático