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

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dc.contributor Sistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.author BLAY, Gabriela FMUSP-HC
RODRIGUES, Joaquim C. FMUSP-HC
FERREIRA, Juliana C. O. FMUSP-HC
LEAL, Gabriela N. FMUSP-HC
GORMEZANO, Natali W. FMUSP-HC
V, Glaucia Novak FMUSP-HC
PEREIRA, Rosa M. R. FMUSP-HC
TERRERI, Maria T.
MAGALHAES, Claudia S.
MOLINARI, Beatriz C. FMUSP-HC
SAKAMOTO, Ana P.
AIKAWA, Nadia E. FMUSP-HC
CAMPOS, Lucia M. A. FMUSP-HC
FERNANDES, Taciana A. P.
CLEMENTE, Gleice
PERACCHI, Octavio A. B.
BUGNI, Vanessa
MARINI, Roberto
SACCHETTI, Silvana B.
CARVALHO, Luciana M.
FRAGA, Melissa M.
CASTRO, Tania C. M.
RAMOS, Valeria C.
BONFA, Eloisa FMUSP-HC
SILVA, Clovis A. FMUSP-HC
dc.date.issued 2018
dc.identifier.citation ADVANCES IN RHEUMATOLOGY, v.58, article ID 39, 6p, 2018
dc.identifier.issn 2523-3106
dc.identifier.uri http://observatorio.fm.usp.br/handle/OPI/29804
dc.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.
dc.description.sponsorship · Conselho Nacional de Desenvolvimento Cientifico e Tecnologico (CNPq) [301805/2013-0, 303752/2015-7, 301479/2015-1, 305068/2014-8, 303422/2015-7]
· Federico Foundation
· Nucleo de Apoio a Pesquisa ""Saude da Crianca e do Adolescente"" da USP (NAP-CriAd)
dc.language.iso eng
dc.publisher BMC
dc.relation.ispartof Advances in Rheumatology
dc.rights restrictedAccess
dc.subject Diffuse alveolar hemorrhage; Childhood; Systemic lupus erythematosus; Multicenter study
dc.subject.other macrophage activation syndrome; pulmonary hemorrhage; rheumatology; diagnosis; juvenile; pancreatitis; management; features; criteria
dc.title Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome
dc.type article
dc.rights.holder Copyright BMC
dc.contributor.groupauthor Brazilian Childhood Onset Syst Lup
dc.description.group LIM/17
dc.description.group LIM/36
dc.identifier.doi 10.1186/s42358-018-0038-4
dc.identifier.pmid 30657099
dc.type.category original article
dc.type.version publishedVersion
hcfmusp.author BLAY, Gabriela:FM:
hcfmusp.author RODRIGUES, Joaquim C.:HC:ICR
hcfmusp.author FERREIRA, Juliana C. O.:HC:ICR
hcfmusp.author LEAL, Gabriela N.:HC:ICR
hcfmusp.author GORMEZANO, Natali W.:FM:
hcfmusp.author V, Glaucia Novak:FM:
hcfmusp.author PEREIRA, Rosa M. R.:FM:MCM
hcfmusp.author MOLINARI, Beatriz C.:HC:ICR
hcfmusp.author AIKAWA, Nadia E.:HC:ICHC
hcfmusp.author CAMPOS, Lucia M. A.:HC:ICR
hcfmusp.author BONFA, Eloisa:FM:MCM
hcfmusp.author SILVA, Clovis A.:FM:MPE
hcfmusp.author.external · TERRERI, Maria T.:Univ Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo, Brazil
· MAGALHAES, Claudia S.:Sao Paulo State Univ UNESP, Fac Med Botucatu, Sao Paulo, Brazil
· SAKAMOTO, Ana P.:Univ Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo, Brazil
· FERNANDES, Taciana A. P.:Sao Paulo State Univ UNESP, Fac Med Botucatu, Sao Paulo, Brazil
· CLEMENTE, Gleice:Univ Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo, Brazil
· PERACCHI, Octavio A. B.:Univ Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo, Brazil
· BUGNI, Vanessa:Univ Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo, Brazil
· MARINI, Roberto:Sao Paulo State Univ Campinas, UNICAMP, Sao Paulo, Brazil
· SACCHETTI, Silvana B.:Irmandade Santa Casa de Misericordia Sao Paulo, Sao Paulo, Brazil
· CARVALHO, Luciana M.:Univ Sao Paulo, Ribeirao Preto Med Sch, Sao Paulo, Brazil
· FRAGA, Melissa M.:Hosp Darcy Vargas, Sao Paulo, Brazil
· CASTRO, Tania C. M.:Hosp Menino Jesus, Sao Paulo, Brazil
· RAMOS, Valeria C.:Pontifical Catholic Univ Sorocaba, Sao Paulo, Brazil
hcfmusp.origem.id WOS:000451048200001
hcfmusp.origem.id 2-s2.0-85060122863
hcfmusp.origem.id SCIELO:S2523-31062018000100228
hcfmusp.publisher.city LONDON
hcfmusp.publisher.country ENGLAND
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dc.description.index MEDLINE
hcfmusp.citation.scopus 1
hcfmusp.citation.wos 0
hcfmusp.affiliation.country Brasil


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