Autor(es):
Blay, Gabriela ; Rodrigues, Joaquim C. ; Ferreira, Juliana C O ; Leal, Gabriela N. ; Gormezano, Natali W. ; Novak, Glaucia V. ; Pereira, Rosa M R ; Terreri, Maria T. ; Magalhães, Claudia S. [UNESP] ; Molinari, Beatriz C. ; Sakamoto, Ana P. ; Aikawa, Nadia E. ; Campos, Lucia M A ; Fernandes, Taciana A P [UNESP] ; Clemente, Gleice ; Peracchi, Octavio A B ; Bugni, Vanessa ; Marini, Roberto [UNESP] ; Sacchetti, Silvana B. ; Carvalho, Luciana M. ; Fraga, Melissa M. ; Castro, Tânia C M ; Ramos, Valéria C ; Bonfá, Eloisa ; Silva, Clovis A.
Data: 2019
Identificador Persistente: http://hdl.handle.net/11449/187274
Origem: Oasisbr
Assunto(s): Childhood; Diffuse alveolar hemorrhage; Multicenter study; Systemic lupus erythematosus
Descrição
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Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)
OBJECTIVE: To evaluate prevalence, clinical manifestations, laboratory abnormalities and treatment in a multicenter cohort study including 847 childhood-onset systemic lupus erythematosus (cSLE) patients with and without diffuse alveolar hemorrhage (DAH), as well as concomitant parameters of severity. METHODS: DAH was defined as the presence of at least three respiratory symptoms/signs associated with diffuse interstitial/alveolar infiltrates on chest x-ray or high-resolution computer tomography and sudden drop in hemoglobin levels. Statistical analysis was performed using Bonferroni correction (p < 0.0022). RESULTS: DAH was observed in 19/847 (2.2%) cSLE patients. Cough/dyspnea/tachycardia/hypoxemia occurred in all cSLE patients with DAH. Concomitant parameters of severity observed were: mechanical ventilation in 14/19 (74%), hemoptysis 12/19 (63%), macrophage activation syndrome 2/19 (10%) and death 9/19 (47%). Further analysis of cSLE patients at DAH diagnosis compared to 76 cSLE control patients without DAH with same disease duration [3 (1-151) vs. 4 (1-151) months, p = 0.335], showed higher frequencies of constitutional involvement (74% vs. 10%, p < 0.0001), serositis (63% vs. 6%, p < 0.0001) and sepsis (53% vs. 9%, p < 0.0001) in the DAH group. The median of disease activity score(SLEDAI-2 K) was significantly higher in cSLE patients with DAH [18 (5-40) vs. 6 (0-44), p < 0.0001]. The frequencies of thrombocytopenia (53% vs. 12%, p < 0.0001), intravenous methylprednisolone (95% vs. 16%, p < 0.0001) and intravenous cyclophosphamide (47% vs. 8%, p < 0.0001) were also significantly higher in DAH patients. CONCLUSIONS: This was the first study to demonstrate that DAH, although not a disease activity score descriptor, occurred in the context of significant moderate/severe cSLE flare. Importantly, we identified that this condition was associated with serious disease flare complicated by sepsis with high mortality rate.
Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP), Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César, São Paulo, SP, 05403-000, Brazil
Pediatric Pulmonology Unit, Children's Institute, FMUSP, Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César, São Paulo, SP, 05403-000, Brazil
Division of Rheumatology FMUSP
Pediatric Rheumatology Unit Universidade Federal de São Paulo
São Paulo State University (UNESP) Faculdade de Medicina de Botucatu
São Paulo State University of Campinas (UNICAMP)
Irmandade da Santa Casa de Misericórdia de São Paulo
Ribeirão Preto Medical School - University of São Paulo
Hospital Darcy Vargas
Hospital Menino Jesus
Pontifical Catholic University of Sorocaba
São Paulo State University (UNESP) Faculdade de Medicina de Botucatu
São Paulo State University of Campinas (UNICAMP)
CNPq: 303422/2015-7
