Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome
Abstract 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...
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| Format: | Article |
| Language: | English |
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BMC
2018-11-01
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| Series: | Advances in Rheumatology |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s42358-018-0038-4 |
| _version_ | 1819208254810488832 |
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| author | Gabriela Blay Joaquim C. Rodrigues Juliana C. O. Ferreira Gabriela N. Leal Natali W. Gormezano Glaucia V. Novak Rosa M. R. Pereira Maria T. Terreri Claudia S. Magalhães Beatriz C. Molinari Ana P. Sakamoto Nadia E. Aikawa Lucia M. A. Campos Taciana A. P. Fernandes Gleice Clemente Octavio A. B. Peracchi Vanessa Bugni Roberto Marini Silvana B. Sacchetti Luciana M. Carvalho Melissa M. Fraga Tânia C. M. Castro Valéria C. Ramos Eloisa Bonfá Clovis A. Silva Brazilian Childhood-onset Systemic Lupus Erythematosus Group |
| author_facet | Gabriela Blay Joaquim C. Rodrigues Juliana C. O. Ferreira Gabriela N. Leal Natali W. Gormezano Glaucia V. Novak Rosa M. R. Pereira Maria T. Terreri Claudia S. Magalhães Beatriz C. Molinari Ana P. Sakamoto Nadia E. Aikawa Lucia M. A. Campos Taciana A. P. Fernandes Gleice Clemente Octavio A. B. Peracchi Vanessa Bugni Roberto Marini Silvana B. Sacchetti Luciana M. Carvalho Melissa M. Fraga Tânia C. M. Castro Valéria C. Ramos Eloisa Bonfá Clovis A. Silva Brazilian Childhood-onset Systemic Lupus Erythematosus Group |
| author_sort | Gabriela Blay |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-12-23T05:36:28Z |
| format | Article |
| id | doaj.art-7358897ce4dd4021b75dbd7625a8422d |
| institution | Directory Open Access Journal |
| issn | 2523-3106 |
| language | English |
| last_indexed | 2024-12-23T05:36:28Z |
| publishDate | 2018-11-01 |
| publisher | BMC |
| record_format | Article |
| series | Advances in Rheumatology |
| spelling | doaj.art-7358897ce4dd4021b75dbd7625a8422d2022-12-21T17:58:20ZengBMCAdvances in Rheumatology2523-31062018-11-015811610.1186/s42358-018-0038-4Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcomeGabriela Blay0Joaquim C. Rodrigues1Juliana C. O. Ferreira2Gabriela N. Leal3Natali W. Gormezano4Glaucia V. Novak5Rosa M. R. Pereira6Maria T. Terreri7Claudia S. Magalhães8Beatriz C. Molinari9Ana P. Sakamoto10Nadia E. Aikawa11Lucia M. A. Campos12Taciana A. P. Fernandes13Gleice Clemente14Octavio A. B. Peracchi15Vanessa Bugni16Roberto Marini17Silvana B. Sacchetti18Luciana M. Carvalho19Melissa M. Fraga20Tânia C. M. Castro21Valéria C. Ramos22Eloisa Bonfá23Clovis A. Silva24Brazilian Childhood-onset Systemic Lupus Erythematosus GroupPediatric Rheumatology Unit, Children’s Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP)Pediatric Pulmonology Unit, Children’s Institute, FMUSPPediatric Rheumatology Unit, Children’s Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP)Pediatric Rheumatology Unit, Children’s Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP)Division of Rheumatology, FMUSPPediatric Rheumatology Unit, Children’s Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP)Division of Rheumatology, FMUSPPediatric Rheumatology Unit, Universidade Federal de São PauloSão Paulo State University (UNESP), Faculdade de Medicina de BotucatuPediatric Rheumatology Unit, Children’s Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP)Pediatric Rheumatology Unit, Universidade Federal de São PauloPediatric Rheumatology Unit, Children’s Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP)Pediatric Rheumatology Unit, Children’s Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP)São Paulo State University (UNESP), Faculdade de Medicina de BotucatuPediatric Rheumatology Unit, Universidade Federal de São PauloPediatric Rheumatology Unit, Universidade Federal de São PauloPediatric Rheumatology Unit, Universidade Federal de São PauloSão Paulo State University of Campinas (UNICAMP)Irmandade da Santa Casa de Misericórdia de São PauloRibeirão Preto Medical School – University of São PauloHospital Darcy VargasHospital Menino JesusPontifical Catholic University of SorocabaPediatric Rheumatology Unit, Children’s Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP)Pediatric Rheumatology Unit, Children’s Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP)Abstract 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.http://link.springer.com/article/10.1186/s42358-018-0038-4Diffuse alveolar hemorrhageChildhoodSystemic lupus erythematosusMulticenter study |
| spellingShingle | Gabriela Blay Joaquim C. Rodrigues Juliana C. O. Ferreira Gabriela N. Leal Natali W. Gormezano Glaucia V. Novak Rosa M. R. Pereira Maria T. Terreri Claudia S. Magalhães Beatriz C. Molinari Ana P. Sakamoto Nadia E. Aikawa Lucia M. A. Campos Taciana A. P. Fernandes Gleice Clemente Octavio A. B. Peracchi Vanessa Bugni Roberto Marini Silvana B. Sacchetti Luciana M. Carvalho Melissa M. Fraga Tânia C. M. Castro Valéria C. Ramos Eloisa Bonfá Clovis A. Silva Brazilian Childhood-onset Systemic Lupus Erythematosus Group Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome Advances in Rheumatology Diffuse alveolar hemorrhage Childhood Systemic lupus erythematosus Multicenter study |
| title | Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome |
| title_full | Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome |
| title_fullStr | Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome |
| title_full_unstemmed | Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome |
| title_short | Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome |
| title_sort | diffuse alveolar hemorrhage in childhood onset systemic lupus erythematosus a severe disease flare with serious outcome |
| topic | Diffuse alveolar hemorrhage Childhood Systemic lupus erythematosus Multicenter study |
| url | http://link.springer.com/article/10.1186/s42358-018-0038-4 |
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