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

Detalhes bibliográficos
Autor(a) principal: Sakamoto, A. P. [UNIFESP]
Data de Publicação: 2017
Outros Autores: Silva, C. A., Saad-Magalhaes, C., Alencar, A. N. [UNIFESP], Pereira, R. M. R., Kozu, K., Barbosa, C. M. P. L., Terreri, M. T. [UNIFESP]
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Institucional da UNIFESP
Texto Completo: http://www.actareumatologica.pt/onlinefirst_download.php?id=1170
https://repositorio.unifesp.br/handle/11600/53476
Resumo: Objective: 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
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spelling Sakamoto, A. P. [UNIFESP]Silva, C. A.Saad-Magalhaes, C.Alencar, A. N. [UNIFESP]Pereira, R. M. R.Kozu, K.Barbosa, C. M. P. L.Terreri, M. T. [UNIFESP]2020-06-26T16:30:19Z2020-06-26T16:30:19Z2017http://www.actareumatologica.pt/onlinefirst_download.php?id=1170Acta Reumatologica Portuguesa. Alges, v. 42, n. 3, p. 250-255, 2017.0303-464Xhttps://repositorio.unifesp.br/handle/11600/53476WOS000418501200006.pdfWOS:000418501200006Objective: 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-TENTEN 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.Conselho Nacional de Desenvolvimento Cientifico e TecnologicoFederico FoundationNucleo de Apoio a Pesquisa "Saude da Crianca e do Adolescente" of USP (NAP-CriAd)Univ Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo, SP, BrazilUniv Sao Paulo, Pediat Rheumatol Unit, Fac Med, Sao Paulo, BrazilUniv Sao Paulo, Div Rheumatol, Fac Med, Sao Paulo, BrazilSao Paulo State Univ UNESP, Fac Med Botucatu, Pediat Rheumatol Div, Botucatu, SP, BrazilHosp Infantil Darcy Vargas, Pediat Rheumatol Unit, Sao Paulo, BrazilUniv Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo, SP, BrazilCNPq: 303422/2015-7CNPq: 301805/2013-0CNPq: 305068/2014-8CNPq: 301479/2015CNPq: 303752/2015-7Federico FoundationNAP-CriAd-USPWeb of Science250-255engPublisaude-Edicoes Medicas LdaActa Reumatologica PortuguesaStevens-Johnson syndromeToxic epidermal necrolysisChildhood-onset systemic lupus erythematosusSystemic lupus erythematosusChildhoodStevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter studyinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleAlges423info:eu-repo/semantics/openAccessreponame:Repositório Institucional da UNIFESPinstname:Universidade Federal de São Paulo (UNIFESP)instacron:UNIFESPORIGINALWOS000418501200006.pdfapplication/pdf134190${dspace.ui.url}/bitstream/11600/53476/1/WOS000418501200006.pdf822e4c56ac15d2c03ac28c21e4868c1fMD51open accessTEXTWOS000418501200006.pdf.txtWOS000418501200006.pdf.txtExtracted texttext/plain24880${dspace.ui.url}/bitstream/11600/53476/2/WOS000418501200006.pdf.txted9df0cdcb80fe749c0d7b7db840bea4MD52open accessTHUMBNAILWOS000418501200006.pdf.jpgWOS000418501200006.pdf.jpgIM Thumbnailimage/jpeg6452${dspace.ui.url}/bitstream/11600/53476/4/WOS000418501200006.pdf.jpgdfefcd2069403ce1e28df84e7dab56e9MD54open access11600/534762022-08-01 10:24:56.746open accessoai:repositorio.unifesp.br:11600/53476Repositório InstitucionalPUBhttp://www.repositorio.unifesp.br/oai/requestopendoar:34652023-05-25T12:25:12.822467Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP)false
dc.title.en.fl_str_mv Stevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter study
title Stevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter study
spellingShingle Stevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter study
Sakamoto, A. P. [UNIFESP]
Stevens-Johnson syndrome
Toxic epidermal necrolysis
Childhood-onset systemic lupus erythematosus
Systemic lupus erythematosus
Childhood
title_short Stevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter study
title_full Stevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter study
title_fullStr Stevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter study
title_full_unstemmed Stevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter study
title_sort Stevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter study
author Sakamoto, A. P. [UNIFESP]
author_facet Sakamoto, A. P. [UNIFESP]
Silva, C. A.
Saad-Magalhaes, C.
Alencar, A. N. [UNIFESP]
Pereira, R. M. R.
Kozu, K.
Barbosa, C. M. P. L.
Terreri, M. T. [UNIFESP]
author_role author
author2 Silva, C. A.
Saad-Magalhaes, C.
Alencar, A. N. [UNIFESP]
Pereira, R. M. R.
Kozu, K.
Barbosa, C. M. P. L.
Terreri, M. T. [UNIFESP]
author2_role author
author
author
author
author
author
author
dc.contributor.author.fl_str_mv Sakamoto, A. P. [UNIFESP]
Silva, C. A.
Saad-Magalhaes, C.
Alencar, A. N. [UNIFESP]
Pereira, R. M. R.
Kozu, K.
Barbosa, C. M. P. L.
Terreri, M. T. [UNIFESP]
dc.subject.eng.fl_str_mv Stevens-Johnson syndrome
Toxic epidermal necrolysis
Childhood-onset systemic lupus erythematosus
Systemic lupus erythematosus
Childhood
topic Stevens-Johnson syndrome
Toxic epidermal necrolysis
Childhood-onset systemic lupus erythematosus
Systemic lupus erythematosus
Childhood
description Objective: 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
publishDate 2017
dc.date.issued.fl_str_mv 2017
dc.date.accessioned.fl_str_mv 2020-06-26T16:30:19Z
dc.date.available.fl_str_mv 2020-06-26T16:30:19Z
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dc.identifier.citation.fl_str_mv Acta Reumatologica Portuguesa. Alges, v. 42, n. 3, p. 250-255, 2017.
dc.identifier.uri.fl_str_mv https://repositorio.unifesp.br/handle/11600/53476
dc.identifier.issn.none.fl_str_mv 0303-464X
dc.identifier.file.none.fl_str_mv WOS000418501200006.pdf
dc.identifier.wos.none.fl_str_mv WOS:000418501200006
url http://www.actareumatologica.pt/onlinefirst_download.php?id=1170
https://repositorio.unifesp.br/handle/11600/53476
identifier_str_mv Acta Reumatologica Portuguesa. Alges, v. 42, n. 3, p. 250-255, 2017.
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WOS000418501200006.pdf
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dc.relation.ispartof.none.fl_str_mv Acta Reumatologica Portuguesa
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dc.coverage.none.fl_str_mv Alges
dc.publisher.none.fl_str_mv Publisaude-Edicoes Medicas Lda
publisher.none.fl_str_mv Publisaude-Edicoes Medicas Lda
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