Stevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter study

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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.authorSAKAMOTO, A. P.
dc.contributor.authorSILVA, C. A.
dc.contributor.authorSAAD-MAGALHAES, C.
dc.contributor.authorALENCAR, A. N.
dc.contributor.authorPEREIRA, R. M. R.
dc.contributor.authorKOZU, K.
dc.contributor.authorBARBOSA, C. M. P. L.
dc.contributor.authorTERRERI, M. T.
dc.date.accessioned2018-04-20T17:24:43Z
dc.date.available2018-04-20T17:24:43Z
dc.date.issued2017
dc.description.abstractObjective: To assess Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in a large population of childhood-onset systemic lupus erythematosus (cSLE) patients. Methods: Multicenter study including 852 cSLE patients followed in Pediatric Rheumatology centers in Sao Paulo, Brazil. SJS was defined as epidermal detachment below 10% of body surface area (BSA), overlap SJS-TEN 10-30% and TEN greater than 30% of BSA. Results: SJS and TEN were observed in 5/852 (0.6%) cSLE female patients, three patients were classified as SJS and two patients were classified as overlap SJS-TEN; TEN was not observed. The mean duration of SJS and overlap SJS-TEN was 15 days (range 7-22) and antibio tics induced four cases. Regarding extra-cutaneous manifestations, hepatomegaly was observed in two cSLE patients, nephritis in two and neuropsychiatric involvement and conjunctivitis were observed respectively in one patient. Hematological involvement included lymphopenia in four, leucopenia in three and thrombocytopenia in two patients. The mean SLEDAI-2K score was 14.8 (range 6-30). Laboratory analysis showed low C3, C4 and/or CH50 in two patients and the presence of anti-dsDNA autoantibody in two patients. One patient had lupus anticoagulant and another one had anticardiolipin IgG. All patients were treated with steroids and four needed additional treatment such as intravenous immunoglobulin in two patients, hydroxychloroquine and azathioprine in two and intravenous cyclophosphamide in one patient. Sepsis was observed in three cSLE patients. Two patients required intensive care and death was observed in one patient. Conclusion: Our study identified SJS and overlap SJS-TEN as rare manifestations of active cSLE associated with severe multisystemic disease, with potentially lethal outcome.
dc.description.indexMEDLINE
dc.description.sponsorshipConselho Nacional de Desenvolvimento Cientifico e Tecnologico [CNPq 303422/2015-7, 301805/2013-0, 305068/2014-8, 301479/2015, 303752/2015-7]
dc.description.sponsorshipFederico Foundation
dc.description.sponsorshipNucleo de Apoio a Pesquisa ""Saude da Crianca e do Adolescente"" of USP (NAP-CriAd)
dc.identifier.citationACTA REUMATOLOGICA PORTUGUESA, v.42, n.3, p.250-255, 2017
dc.identifier.issn0303-464X
dc.identifier.urihttps://observatorio.fm.usp.br/handle/OPI/26198
dc.language.isoeng
dc.publisherPUBLISAUDE-EDICOES MEDICAS LDA
dc.relation.ispartofActa Reumatologica Portuguesa
dc.rightsopenAccess
dc.rights.holderCopyright PUBLISAUDE-EDICOES MEDICAS LDA
dc.subjectStevens-Johnson syndrome
dc.subjectToxic epidermal necrolysis
dc.subjectChildhood-onset systemic lupus erythematosus
dc.subjectSystemic lupus erythematosus
dc.subjectChildhood
dc.subject.otherof-the-literature
dc.subject.otherdiagnosis
dc.subject.othercriteria
dc.subject.othertherapy
dc.subject.wosRheumatology
dc.titleStevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter study
dc.typearticle
dc.type.categoryoriginal article
dc.type.versionpublishedVersion
dspace.entity.typePublication
hcfmusp.author.externalSAKAMOTO, A. P.:Univ Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo, SP, Brazil
hcfmusp.author.externalSAAD-MAGALHAES, C.:Sao Paulo State Univ UNESP, Fac Med Botucatu, Pediat Rheumatol Div, Botucatu, SP, Brazil
hcfmusp.author.externalALENCAR, A. N.:Univ Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo, SP, Brazil
hcfmusp.author.externalBARBOSA, C. M. P. L.:Hosp Infantil Darcy Vargas, Pediat Rheumatol Unit, Sao Paulo, Brazil
hcfmusp.author.externalTERRERI, M. T.:Univ Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo, SP, Brazil
hcfmusp.citation.scopus8
hcfmusp.contributor.author-fmusphcCLOVIS ARTUR ALMEIDA DA SILVA
hcfmusp.contributor.author-fmusphcROSA MARIA RODRIGUES PEREIRA
hcfmusp.contributor.author-fmusphcKATIA TOMIE KOZU
hcfmusp.description.beginpage250
hcfmusp.description.endpage255
hcfmusp.description.issue3
hcfmusp.description.volume42
hcfmusp.origemWOS
hcfmusp.origem.pubmed28375199
hcfmusp.origem.scopus2-s2.0-85030475654
hcfmusp.origem.wosWOS:000418501200006
hcfmusp.publisher.cityALGES
hcfmusp.publisher.countryPORTUGAL
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