Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome

Advances in Rheumatology

nia C. M. Castro²  Roberto Marini²  Natali W. Gormezano⁶  Maria T. Terreri⁶  Taciana A. P. Fernandes⁷  Gabriela Blay⁷  Clovis A. Silva⁷  Silvana B. Sacchetti⁷  Beatriz C. Molinari⁷  Valé⁸  Eloisa Bonfá^1,11  Octavio A. B. Peracchi^1,12  Melissa M. Fraga^1,12  Nadia E. Aikawa^1,12  Juliana C. O. Ferreira^{1,12  1,13}  Vanessa Bugni^1,15  es^1,15  Lucia M. A. Campos^1,15  Gabriela N. Leal^1,15  Tâ^1,15  Rosa M. R. Pereira^1,15  ria C. Ramos^1,16  Glaucia V. Novak^1,19  Claudia S. Magalhã^1,19  Luciana M. Carvalho^2,20  Joaquim C. Rodrigues^2,20  Ana P. Sakamoto^2,20  Gleice Clemente^2,20 

[1] University of SãDivision of Rheumatology, FMUSP, Sao Paulo, Brazil;Hospital Darcy Vargas, Sao Paulo, Brazil;Hospital Menino Jesus, Sao Paulo, Brazil;Irmandade da Santa Casa de MisericóPediatric Pulmonology Unit, Children’Pediatric Rheumatology Unit, Children’Pediatric Rheumatology Unit, Universidade Federal de SãPontifical Catholic University of Sorocaba, Sao Paulo, Brazil;RibeirãSão Paulo (FMUSP), São Paulo State University (UNESP), Faculdade de Medicina de Botucatu, Sao Paulo, Brazil;o Paulo State University of Campinas (UNICAMP), Sao Paulo, Brazil;o Paulo, Brazil;o Paulo, Sao Paulo, Brazil;o Preto Medical School –rdia de Sãs Institute, FMUSP, Sãs Institute, Faculdade de Medicina da Universidade de Sã

关键词: Diffuse alveolar hemorrhage;    Childhood;    Systemic lupus erythematosus;    Multicenter study;   

DOI  :  10.1186/s42358-018-0038-4

学科分类：过敏症与临床免疫学

来源: Springer

【 摘 要 】

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. 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). 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. 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.

【 授权许可】

CC BY