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Made available in DSpace on 2019-10-06T16:49:50Z (GMT). No. of bitstreams: 0 Previous issue date: 2019-01-01 Objective: To evaluate the influence of ethnicity in presentation of childhood-onset systemic lupus erythematosus (cSLE) patients. Methods: This multicenter study included cSLE patients (American College of Rheumatology criteria) followed in 27 Pediatric Rheumatology services of Brazil. Ethnicities were classified in four groups according to the parents’ and all four grandparents’ self-reported ethnicity. The statistical analysis was performed using the Bonferroni’s correction (p < 0.0027). Results: According to ethnic groups, 1537 cSLE patients were classified in Caucasian (n = 786), African-Latin American (n = 526), Asian (n = 8), and others/unknown (n = 217). Comparisons between 1312 African-Latin American and Caucasian revealed similar median age at cSLE diagnosis [12.2(2.6–18) vs. 12.1(0.3–18) years, p = 0.234], time interval to diagnosis [0.25(0–12) vs. 0.3(0–10) years, p = 0.034], and SLEDAI-2K score [14(0–55) vs. 14(0–63), p = 0.781] in both groups. The mean number of diagnostic criteria according to SLICC (6.47 ± 1.911 vs. 5.81 ± 1.631, p < 0.0001) and frequencies of maculopapular lupus rash (8% vs. 3%, p < 0.0001), palate oral ulcers (17% vs. 11%, p = 0.001), tongue oral ulcers (4% vs. 1%, p = 0.001), and nonscarring alopecia (29% vs. 16%, p < 0.0001) were significantly higher in African-Latin American, whereas malar rash (45% vs. 58%, p < 0.0001) was more frequent in Caucasian. The presence of anti-phospholipid antibody (23% vs. 12%, p < 0.0001), low complement levels (58% vs. 41%, p < 0.0001), and isolated direct Coombs test (10% vs. 5%, p = 0.001) was also significantly higher in the former group. Conclusions: Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable with Caucasian. Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of the former group. The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients.Key Points• Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable with Caucasian.• Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of African-Latin American cSLE patients.• African-Latin American cSLE patients had more often anti-phospholipid antibodies and hypocomplementemia.• The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients. Pediatric Rheumatology Unit Children’s Institute Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo, Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César Pediatric Rheumatology Unit Hospital Jose Alencar Division of Rheumatology Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo Pediatric Rheumatology Unit Universidade Federal de Sao Paulo Pediatric Rheumatology Division Sao Paulo State University (UNESP) Pediatric Rheumatology Unit University of Campinas (UNICAMP) Pediatric Rheumatology Unit Ribeirao Preto Medical School – University of Sao Paulo Pediatric Rheumatology Unit Hospital Geral de Fortaleza Pediatric Rheumatology Unit Rio de Janeiro Federal University (IPPMG-UFRJ) Pediatric Rheumatology Unit Pedro Ernesto University Hospital Pediatric Rheumatology Unit Albert Sabin Children’s Hospital Pediatric Rheumatology Unit Hospital Darcy Vargas Pediatric Rheumatology Unit Irmandade da Santa Casa de Misericórdia de Sao Paulo Rheumatology Division - Universidade Federal do Rio de Janeiro Hospital Universitário Clementino Fraga Filho Pediatric Rheumatology Unit Lauro Vanderley University Hospital Pediatric Rheumatology Unit Federal University of Pará Pediatric Rheumatology Unit Obras Sociais Irmã Dulce Pediatric Rheumatology Unit Hospital Evangélico de Curitiba Pediatric Rheumatology Unit Hospital Criança Conceição Pediatric Rheumatology Unit Federal University of Pernambuco Pediatric Rheumatology Unit Federal University of Mato Grosso do Sul Pediatric Rheumatology Unit Federal University of Bahia Pediatric Rheumatology Unit University of Brasilia Pediatric Rheumatology Unit Federal University of Minas Gerais Pediatric Rheumatology Unit Pontifícia Catholic University of Sorocaba Pediatric Rheumatology Division Sao Paulo State University (UNESP)

Made available in DSpace on 2018-12-11T17:24:18Z (GMT). No. of bitstreams: 0 Previous issue date: 2018-09-01 Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP) Objective: The objective of this study was to compare demographic data, clinical/laboratorial features and disease activity at diagnosis in three different groups with distinct time intervals between onset of signs/symptoms and disease diagnosis. Methods: A multicenter study was performed in 1555 childhood-onset systemic lupus erythematosus (American College of Rheumatology criteria) patients from 27 pediatric rheumatology services. Patients were divided into three childhood-onset systemic lupus erythematosus groups: A: short time interval to diagnosis (500 mg/day (48% vs. 45% vs. 36%, P = 0.002) and low complement levels (81% vs. 81% vs. 71%, P < 0.0001) compared with groups A or B. Conclusions: Our large Brazilian multicenter study demonstrated that for most childhood-onset systemic lupus erythematosus patients, diagnosis is delayed probably due to mild disease onset. Conversely, the minority has a very short time interval to diagnosis and a presentation with a more severe and active multisystemic condition. Pediatric Rheumatology Unit Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo Pediatric Rheumatology Unit Universidade Federal de Sao Paulo Division of Rheumatology Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo Pediatric Rheumatology Division Sao Paulo State University (UNESP) Pediatric Rheumatology Unit University of Campinas (UNICAMP) Pediatric Rheumatology Unit University of Sao Paulo Pediatric Rheumatology Unit Hospital Geral de Fortaleza Pediatric Rheumatology Unit Rio de Janeiro Federal University (IPPMG-UFRJ) Pediatric Rheumatology Unit Hospital Jose Alencar Pediatric Rheumatology Unit Pedro Ernesto University Hospital Pediatric Rheumatology Unit Albert Sabin Children’s Hospital Pediatric Rheumatology Unit Hospital Darcy Vargas Pediatric Rheumatology Unit Irmandade da Santa Casa de Misericórdia de Sao Paulo Rheumatology Division Hospital Universitário Clementino Fraga Filho Pediatric Rheumatology Unit Lauro Vanderley University Hospital Pediatric Rheumatology Unit Federal University of Pará Pediatric Rheumatology Unit Obras Sociais Irmã Dulce Pediatric Rheumatology Unit Hospital Evangélico de Curitiba Pediatric Rheumatology Unit Hospital Criança Conceição Pediatric Rheumatology Unit Federal University of Pernambuco Pediatric Rheumatology Unit Federal University of Mato Grosso do Sul Pediatric Rheumatology Unit Federal University of Bahia Pediatric Rheumatology Unit University of Brasilia Pediatric Rheumatology Unit Federal University of Minas Gerais Pediatric Rheumatology Unit Hospital Municipal Piedade Pediatric Rheumatology Division Sao Paulo State University (UNESP) FAPESP: 03756-4

Objetivo Analisar as características clínicas e epidemiológicas das espondiloartrites (EpA) de início juvenil (< 16 anos) e compará-las com um grupo de pacientes com EspA de início na vida adulta (≥ 16 anos). Pacientes e métodos Coorte prospectiva, observacional e multicêntrica com 1.424 pacientes com diagnóstico de EspA de acordo com o European Spondyloarthropathy Study Group (ESSG) submetidos a um protocolo comum de investigação e recrutados em 29 centros de referência participantes do Registro Brasileiro de Espondiloartrites (RBE). Os pacientes foram divididos em dois grupos: idade no início

OBJETIVO: Descrever as manifestações extra-articulares (cardíacas, renais, pulmonares e neurológicas) geralmente não relacionadas às espondiloartrites (EpA) em uma grande coorte de pacientes brasileiros. MÉTODOS: Este estudo retrospectivo analisou 1.472 pacientes com o diagnóstico de EpA atendidos em 29 centros distribuídos pelas cinco principais regiões geográficas do Brasil, integrantes do Registro Brasileiro de Espondiloartrites. Todos os pacientes foram avaliados para a prevalência das principais manifestações extra-articulares (cardíacas, renais, pulmonares e neurológicas), divididas por diagnóstico [espondilite anquilosante (EA), artrite psoriásica (AP), artrite reativa (ARe), artrite associada a doença inflamatória intestinal (DII), EpA indiferenciada (EI) e EpA juvenil] e por forma clínica (axial, periférica, mista e entesítica). RESULTADOS: Dentre os pacientes avaliados com EpA, 963 apresentavam EA, 271 AP, 49 ARe, 48 artrite associada a DII, 98 EI e 43 EpA juvenil. Acometimento cardíaco foi observado em 44 pacientes (3,0%), seguido por acometimento pulmonar em 19 (1,3%), renal em 17 (1,2%) e neurológico em 13 pacientes (0,9%). A maioria dos casos de acometimento visceral ocorreu nos pacientes com EA ou AP e naqueles com forma clínica mista (axial e periférica) e/ou predominantemente axial. CONCLUSÃO: As manifestações extra-articulares cardíacas, renais, pulmonares e neurológicas são muito pouco frequentes nas EpA, variando de 0,9%-3% nesta grande coorte brasileira, estando mais associadas a EA e AP. OBJECTIVE: To describe the extra-articular manifestations (cardiac, renal, pulmonary, and neurological), usually not related to spondyloarthritis (SpA), in a large cohort of Brazilian patients. MATERIALS AND METHODS: This retrospective study analyzed 1,472 patients diagnosed with SpA and cared for at 29 health care centers distributed in the five major geographic regions in the country, participating in the Brazilian Registry of Spondyloarthritis (BRS). All patients were assessed for the prevalence of major extra-articular manifestations (cardiac, renal, pulmonary, and neurological), classified according to the diagnosis [ankylosing spondylitis (AS), psoriatic arthritis (PsA), reactive arthritis (ReA), arthritis associated with inflammatory bowel disease (IBD), undifferentiated spondyloarthritis (uSpA), and juvenile SpA], and according to the clinical presentation (axial, peripheral, mixed, and enthesitis). RESULTS: Of the patients with SpA assessed, 963 had AS, 271 PsA, 49 ReA, 48 arthritis associated with IBD, 98 uSpA, and 43 juvenile SpA. Cardiac involvement was reported in 44 patients (3.0%), pulmonary involvement in 19 (1.3%), renal involvement in 17 (1.2%), and neurological involvement in 13 patients (0.9%). Most patients with visceral involvement had AS or PsA, and the mixed (axial + peripheral) and/or predominantly axial clinical form. CONCLUSION: Cardiac, renal, pulmonary, and neurological extra-articular manifestations are quite infrequent in SpA, ranging from 0.9% to 3% in this large Brazilian cohort, and affected predominantly patients with AS and PsA. Universidade de Fortaleza Hospital Geral de Fortaleza Hospital Geral de Fortaleza Serviço de Reumatologia Instituto de Ensino e Pesquisa FMUSP Hospital das Clínicas Universidade de Brasília Hospital Geral de Goiânia Universidade Estadual de Campinas Universidade Federal do Amazonas Pontifícia Universidade Católica de Porto Alegre Faculdade de Medicina de São José do Rio Preto Hospital Evangélico de Curitiba Universidade Federal do Rio de Janeiro Universidade Federal do Paraná Universidade do Estado do Rio de Janeiro Santa Casa da Misericórdia do Rio de Janeiro Pontifícia Universidade Católica de Campinas Santa Casa de Misericórdia de São Paulo Hospital de Base de Brasília Universidade Federal do Mato Grosso do Sul Universidade Federal de Pernambuco Universidade Federal do Rio Grande do Sul Fundação Técnico-Educacional Souza Marques Hospital do Servidor Público Estadual de São Paulo FMUSP Universidade Federal de Santa Catarina Universidade Federal de São Paulo (UNIFESP) Santa Casa de Misericórdia de Belo Horizonte Universidade Federal de Minas Gerais Universidade Federal do Ceará Escola de Saúde Pública da Bahia Universidade Federal do Pará Universidade Federal do Espírito Santo UNIFESP, EPM, São Paulo SciELO