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

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dc.contributor.authorBlay, Gabriela
dc.contributor.authorRodrigues, Joaquim C.
dc.contributor.authorFerreira, Juliana C O
dc.contributor.authorLeal, Gabriela N.
dc.contributor.authorGormezano, Natali W.
dc.contributor.authorNovak, Glaucia V.
dc.contributor.authorPereira, Rosa M R
dc.contributor.authorTerreri, Maria T.
dc.contributor.authorMagalhães, Claudia S. [UNESP]
dc.contributor.authorMolinari, Beatriz C.
dc.contributor.authorSakamoto, Ana P.
dc.contributor.authorAikawa, Nadia E.
dc.contributor.authorCampos, Lucia M A
dc.contributor.authorFernandes, Taciana A P [UNESP]
dc.contributor.authorClemente, Gleice
dc.contributor.authorPeracchi, Octavio A B
dc.contributor.authorBugni, Vanessa
dc.contributor.authorMarini, Roberto [UNESP]
dc.contributor.authorSacchetti, Silvana B.
dc.contributor.authorCarvalho, Luciana M.
dc.contributor.authorFraga, Melissa M.
dc.contributor.authorCastro, Tânia C M
dc.contributor.authorRamos, Valéria C
dc.contributor.authorBonfá, Eloisa
dc.contributor.authorSilva, Clovis A.
dc.contributor.institutionUniversidade de São Paulo (USP)
dc.contributor.institutionUniversidade Federal de São Paulo (UNIFESP)
dc.contributor.institutionUniversidade Estadual Paulista (Unesp)
dc.contributor.institutionIrmandade da Santa Casa de Misericórdia de São Paulo
dc.contributor.institutionHospital Darcy Vargas
dc.contributor.institutionHospital Menino Jesus
dc.contributor.institutionPontifical Catholic University of Sorocaba
dc.date.accessioned2019-10-06T15:31:10Z
dc.date.available2019-10-06T15:31:10Z
dc.date.issued2018-11-23
dc.description.abstractOBJECTIVE: 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.en
dc.description.affiliationPediatric 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
dc.description.affiliationPediatric Pulmonology Unit, Children's Institute, FMUSP, Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César, São Paulo, SP, 05403-000, Brazil
dc.description.affiliationDivision of Rheumatology FMUSP
dc.description.affiliationPediatric Rheumatology Unit Universidade Federal de São Paulo
dc.description.affiliationSão Paulo State University (UNESP) Faculdade de Medicina de Botucatu
dc.description.affiliationSão Paulo State University of Campinas (UNICAMP)
dc.description.affiliationIrmandade da Santa Casa de Misericórdia de São Paulo
dc.description.affiliationRibeirão Preto Medical School - University of São Paulo
dc.description.affiliationHospital Darcy Vargas
dc.description.affiliationHospital Menino Jesus
dc.description.affiliationPontifical Catholic University of Sorocaba
dc.description.affiliationUnespSão Paulo State University (UNESP) Faculdade de Medicina de Botucatu
dc.description.affiliationUnespSão Paulo State University of Campinas (UNICAMP)
dc.description.sponsorshipConselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)
dc.description.sponsorshipIdCNPq: 303422/2015-7
dc.format.extent39
dc.identifierhttp://dx.doi.org/10.1186/s42358-018-0038-4
dc.identifier.citationAdvances in rheumatology (London, England), v. 58, n. 1, p. 39-, 2018.
dc.identifier.doi10.1186/s42358-018-0038-4
dc.identifier.fileS2523-31062018000100228.pdf
dc.identifier.issn2523-3106
dc.identifier.scieloS2523-31062018000100228
dc.identifier.scopus2-s2.0-85060122863
dc.identifier.urihttp://hdl.handle.net/11449/187274
dc.language.isoeng
dc.relation.ispartofAdvances in rheumatology (London, England)
dc.rights.accessRightsAcesso aberto
dc.sourceScopus
dc.subjectChildhood
dc.subjectDiffuse alveolar hemorrhage
dc.subjectMulticenter study
dc.subjectSystemic lupus erythematosus
dc.titleDiffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcomeen
dc.typeArtigo
unesp.author.orcid0000-0001-9250-6508 0000-0001-9250-6508[25]

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