期刊论文详细信息
Advances in Rheumatology
Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome
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 Bonfá1,11  Octavio A. B. Peracchi1,12  Melissa M. Fraga1,12  Nadia E. Aikawa1,12  Juliana C. O. Ferreira1,12  1,13  Vanessa Bugni1,15  es1,15  Lucia M. A. Campos1,15  Gabriela N. Leal1,15  Tâ1,15  Rosa M. R. Pereira1,15  ria C. Ramos1,16  Glaucia V. Novak1,19  Claudia S. Magalhã1,19  Luciana M. Carvalho2,20  Joaquim C. Rodrigues2,20  Ana P. Sakamoto2,20  Gleice Clemente2,20 
[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
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【 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.

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