Characteristics of 1555 childhood-onset lupus in three groups based on distinct time intervals to disease diagnosis: a Brazilian multicenter study

Author(s):Show less -

Novak, V, G. ; Molinari, B. C. ^[1] ; Ferreira, J. C. ^[1] ; Sakamoto, A. P. ^[2] ; Terreri, M. T. ^[2] ; Pereira, R. M. R. ^[3] ; Saad-Magalhaes, C. ^[4] ; Aikawa, N. E. ^{[1, 3]} ; Campos, L. M. ^[1] ; Len, C. A. ^[2] ; Appenzeller, S. ^[5] ; Ferriani, V. P. ^[6] ; Silva, M. F. ^[7] ; Oliveira, S. K. ^[8] ; Islabdo, A. G. ^[9] ; Sztajnbok, F. R. ^[10] ; Paim, L. B. ^[11] ; Barbosa, C. M. ^[12] ; Santos, M. C. ^[13] ; Bica, B. E. ^[14] ; Sena, E. G. ^[15] ; Moraes, A. J. ^[16] ; Rolim, A. M. ^[17] ; Spelling, P. F. ^[18] ; Scheibel, I. M. ^[19] ; Cavalcanti, A. S. ^[20] ; Matos, E. N. ^[21] ; Robazzi, T. C. ^[22] ; Guimaracs, L. J. ^[23] ; Santos, F. P. ^[24] ; Silva, C. T. ^[25] ; Bonfa, E. ^[3] ; Silva, C. A. ^{[1, 3]} ; Systemic, Brazilian Childhood-onset

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^[1] Novak, G., V, Univ Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Pediat Rheumatol Unit, Sao Paulo - Brazil

^[2] Univ Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo - Brazil

^[3] Univ Sao Paulo, Div Rheumatol, Hosp Clin HCFMUSP, Fac Med, Sao Paulo - Brazil

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

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

^[6] Univ Sao Paulo, 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] Albert Sabin Childrens Hosp, Pediat Rheumatol Unit, Fortaleza, Ceara - Brazil

^[12] Hosp Darcy Vargas, Pediat Rheumatol Unit, Sao Paulo - Brazil

^[13] Irmandade Santa Casa Misericordia Sao Paulo, Pediat Rheumatol Unit, Sao Paulo - Brazil

^[14] Hosp Univ Clementino Fraga Filho, Rheumatol Div, 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] Obras Sociais Irma Dulce, Pediat Rheumatol Unit, Salvador, BA - Brazil

^[18] Hosp Evangelico Curitiba, Pediat Rheumatol Unit, Curitiba, Parana - Brazil

^[19] Hosp Crianca Conceicao, Pediat Rheumatol Unit, Porto Alegre, RS - Brazil

^[20] Univ Fed Pernambuco, Pediat Rheumatol Unit, Recife, PE - Brazil

^[21] Univ Fed Mato Grosso do Sul, Pediat Rheumatol Unit, Campo Grande - Brazil

^[22] Univ Fed Bahia, Pediat Rheumatol Unit, Salvador, BA - Brazil

^[23] Univ Brasilia, Pediat Rheumatol Unit, Brasilia, DF - Brazil

^[24] Univ Fed Minas Gerais, Pediat Rheumatol Unit, Belo Horizonte, MG - Brazil

^[25] Hosp Municipal Piedade, Pediat Rheumatol Unit, Rio De Janeiro - Brazil

• Document type: Journal article
• Source: Lupus; v. 27, n. 10, p. 1712-1717, SEP 2018.
• Web of Science Citations: 2

Abstract

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 (<1 month); B: intermediate time interval (1 and <3 months); and C: long time interval (3 months). An investigator meeting was held to define the protocol. Demographic data, SLICC classification criteria and SLEDAI-2K were evaluated. Results The number of patients in each group was: A=60 (4%); B=522 (33.5%); and C=973 (62.5%). The median age at diagnosis (11.1 (4.2-17) vs. 12 (1.9-17.7) vs. 12.5 (3-18) years, P=0.025) was significantly lower in group A compared with groups B and C. The median number of diagnostic criteria according to SLICC (7 (4-12) vs. 6 (4-13) vs. 6 (4-12), P<0.0001) and SLEDAI-2K (18 (6-57) vs. 16 (2-63) vs. 13 (1-49), P<0.0001) were significantly higher in group A than the other two groups. The frequency of oral ulcers in the palate (25% vs. 15% vs. 11%, P=0.003), pleuritis (25% vs. 24% vs. 14%, P<0.0001), nephritis (52% vs. 47% vs. 40%, P=0.009), neuropsychiatric manifestations (22% vs. 13% vs. 10%, P=0.008), thrombocytopenia (32% vs. 18% vs. 19%, P=0.037), leucopenia/lymphopenia (65% vs. 46% vs. 40%, P<0.0001) and anti-dsDNA antibodies (79% vs. 66% vs. 61%, P=0.01) were significantly higher in group A compared with the other groups. In contrast, group C had a less severe disease characterized by higher frequencies of synovitis (61% vs. 66% vs. 71%, P=0.032) and lower frequencies of serositis (37% vs. 33% vs. 25%, P=0.002), proteinuria >500mg/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. (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