Subclinical Pulmonary Hypertension in Childhood Systemic Lupus Erythematosus Associated with Minor Disease Manifestations

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dc.contributorSistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.authorANUARDO, Pedro
dc.contributor.authorVERDIER, Monica
dc.contributor.authorGORMEZANO, Natali W. S.
dc.contributor.authorFERREIRA, Gabriela R. V.
dc.contributor.authorLEAL, Gabriela N.
dc.contributor.authorLIANZA, Alessandro
dc.contributor.authorFERREIRA, Juliana C. O. A.
dc.contributor.authorPEREIRA, Rosa M. R.
dc.contributor.authorAIKAWA, Nadia E.
dc.contributor.authorTERRERI, Maria Teresa
dc.contributor.authorMAGALHES, Claudia S.
dc.contributor.authorAPPENZELLER, Simone
dc.contributor.authorSANTOS, Maria Carolina dos
dc.contributor.authorSACHETTI, Silvana B.
dc.contributor.authorLEN, Claudio A.
dc.contributor.authorPILLEGGI, Gecilmara S.
dc.contributor.authorLOTUFO, Simone
dc.contributor.authorBONFA, Eloisa
dc.contributor.authorSILVA, Clovis A.
dc.date.accessioned2017-06-09T15:18:21Z
dc.date.available2017-06-09T15:18:21Z
dc.date.issued2017
dc.description.abstractThe aim of this study was to evaluate pulmonary hypertension (PH) in 852 childhood-onset systemic lupus erythematosus (cSLE) patients. This was a large multicenter study conducted in 10 Pediatric Rheumatology Services of So Paulo state, Brazil. PH was defined as systolic pulmonary artery pressure > 35 mmHg and/or measurement of the mean pulmonary artery pressure > 25 mmHg and/or diastolic pressure > 15 mmHg by transthoracic echocardiogram. Demographic data, clinical manifestations, disease activity score (SLEDAI-2K), disease damage score (SLICC/ACR-DI) and treatments were also evaluated. Statistical analysis was performed using Bonferroni correction (p < 0.002). PH was observed in 17/852 (2%) cSLE patients. Effort dyspnea occurred in 3/17, chest pain in 1/17 and right ventricle dysfunction in 3/17 cSLE patients. None had pulmonary thromboembolism or antiphospholipid syndrome. Further comparison between 17 cSLE with PH and 85 cSLE control patients without PH with similar disease duration [15 (0-151) vs. 15 (0-153) months, p = 0.448], evaluated at the last visit, revealed higher frequencies of fever (47 vs. 9%, p < 0.001), reticuloendothelial manifestations (41 vs. 7%, p < 0.001) and serositis (35 vs. 5%, p = 0.001) in the former group. Frequencies of renal and neuropsychiatric involvements and antiphospholipid syndrome, as well as the median of SLEDAI-2K and SLICC/ACR-DI scores, were comparable in both groups (p > 0.002). Normal transthoracic echocardiography was evidenced in 9/17 (53%), with median cSLE duration of 17.5 months (1-40) after PH standard treatment. PH was a rare manifestation of cSLE occurring in the first two years of disease. The majority of patients were asymptomatic with mild lupus manifestations. The underlying mechanism seemed not to be related to pulmonary thromboembolism and/or antiphospholipid syndrome.
dc.description.indexMEDLINE
dc.description.sponsorshipConselho Nacional de Desenvolvimento Cientifico e Tecnologico (CNPq) [301805/2013-0, 303752/2015-7, 301479/2015-1, 305068/2014-8, 303422/2015-7]
dc.description.sponsorshipFederico Foundation
dc.description.sponsorshipNucleo de Apoio a Pesquisa ""Saude da Crianc, a e do Adolescente'' da USP (NAP-CriAd)
dc.identifier.citationPEDIATRIC CARDIOLOGY, v.38, n.2, p.234-239, 2017
dc.identifier.doi10.1007/s00246-016-1504-6
dc.identifier.eissn1432-1971
dc.identifier.issn0172-0643
dc.identifier.urihttps://observatorio.fm.usp.br/handle/OPI/19950
dc.language.isoeng
dc.publisherSPRINGER
dc.relation.ispartofPediatric Cardiology
dc.rightsrestrictedAccess
dc.rights.holderCopyright SPRINGER
dc.subjectPulmonary hypertension
dc.subjectChildhood systemic lupus erythematosus
dc.subjectLung
dc.subjectMulticenter cohort
dc.subject.otherarterial-hypertension
dc.subject.otherinvolvement
dc.subject.otherprevalence
dc.subject.otherdiagnosis
dc.subject.othercriteria
dc.subject.otherregistry
dc.subject.otherindex
dc.subject.wosCardiac & Cardiovascular Systems
dc.subject.wosPediatrics
dc.titleSubclinical Pulmonary Hypertension in Childhood Systemic Lupus Erythematosus Associated with Minor Disease Manifestations
dc.typearticle
dc.type.categoryoriginal article
dc.type.versionpublishedVersion
dspace.entity.typePublication
hcfmusp.author.externalFERREIRA, Gabriela R. V.:Univ Sao Paulo, Fac Med, Childrens Inst, Pediat Rheumatol Unit, Sao Paulo, Brazil
hcfmusp.author.externalTERRERI, Maria Teresa:Univ Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo, Brazil
hcfmusp.author.externalMAGALHES, Claudia S.:Sao Paulo State Univ UNESP, Fac Med Botucatu, Sao Paulo, Brazil
hcfmusp.author.externalAPPENZELLER, Simone:Univ Estadual Campinas, Campinas, SP, Brazil
hcfmusp.author.externalSANTOS, Maria Carolina dos:Irmandade Santa Casa Misericordia Sao Paulo, Sao Paulo, Brazil
hcfmusp.author.externalSACHETTI, Silvana B.:Irmandade Santa Casa Misericordia Sao Paulo, Sao Paulo, Brazil
hcfmusp.author.externalLEN, Claudio A.:Univ Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo, Brazil
hcfmusp.author.externalPILLEGGI, Gecilmara S.:Univ Sao Paulo, Ribeirao Preto Med Sch, Sao Paulo, Brazil
hcfmusp.author.externalLOTUFO, Simone:Hosp Menino Jesus, Sao Paulo, Brazil
hcfmusp.citation.scopus13
hcfmusp.contributor.author-fmusphcPEDRO ADDE ANUARDO
hcfmusp.contributor.author-fmusphcMONICA VERDIER
hcfmusp.contributor.author-fmusphcNATALI WENIGER SPELLING GORMEZANO
hcfmusp.contributor.author-fmusphcGABRIELA NUNES LEAL
hcfmusp.contributor.author-fmusphcALESSANDRO CAVALCANTI LIANZA
hcfmusp.contributor.author-fmusphcJULIANA CAIRES DE OLIVEIRA ACHILI FERREIRA
hcfmusp.contributor.author-fmusphcROSA MARIA RODRIGUES PEREIRA
hcfmusp.contributor.author-fmusphcNADIA EMI AIKAWA
hcfmusp.contributor.author-fmusphcELOISA SILVA DUTRA DE OLIVEIRA BONFA
hcfmusp.contributor.author-fmusphcCLOVIS ARTUR ALMEIDA DA SILVA
hcfmusp.description.beginpage234
hcfmusp.description.endpage239
hcfmusp.description.issue2
hcfmusp.description.volume38
hcfmusp.origemWOS
hcfmusp.origem.pubmed27826712
hcfmusp.origem.scopus2-s2.0-84994417490
hcfmusp.origem.wosWOS:000395096700004
hcfmusp.publisher.cityNEW YORK
hcfmusp.publisher.countryUSA
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hcfmusp.scopus.lastupdate2024-04-12
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