Factors affecting survival in juvenile systemic sclerosis
Autor(a) principal: | |
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Data de Publicação: | 2009 |
Outros Autores: | , , , , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Institucional da UNIFESP |
Texto Completo: | http://repositorio.unifesp.br/handle/11600/31266 http://dx.doi.org/10.1093/rheumatology/ken388 |
Resumo: | Objectives. To determine whether demographic, clinical and immunological features may predict the outcome in juvenile SSc (JSSc).Methods. Clinical and laboratory characteristics of patients with JSSc collected from paediatric rheumatology centres worldwide were analysed. First, univariate tests identified those features significantly related with fatal outcome, and then multivariate logistic regression analysis was applied to determine the predictors of mortality.Results. One hundred and thirty-four patients from 40 centres were eligible for the analysis. Sixteen patients died and a rapidly fatal course was observed in most of them: 4/16 died within 1 yr after diagnosis and 10/16 within 5 yrs. At the moment of diagnosis, patients with poor outcome showed a significantly higher frequency of internal organ involvement, particularly cardiac, respiratory and gastrointestinal systems. No significant difference emerged for entity of skin, vascular and musculo-skeletal involvement, nor for auto-antibodies profile and laboratory tests. Multivariate analysis showed the following factors to be significant predictors of mortality: fibrosis on chest X-rays [odds ratio (OR) 11.2], raised creatinine levels (OR 22.7) and pericarditis (OR 41.3), while a short disease duration at diagnosis conferred protection (OR 0.3).Conclusions. All patients with JSSc and fatal outcome were affected by the diffuse form of the disease, and most of them showed a very rapid progression and early signs of internal organ involvement. This suggests that, in children, SSc may have two possible courses: a rapid development of internal organ failure leading to severe disability and eventually to death, or a slow course of the disease with lower mortality. |
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Martini, G.Vittadello, F.Kasapcopur, OeMagni Manzoni, S.Corona, F.Duarte-Salazar, C.Nemcova, D.Len, C. A. [UNIFESP]Garay, S. M.Ullman, S.Zulian, F.Juvenile Scleroderma Working Grp PUniv PaduaCerrahpasa Tip FakFdn IRCCS Policlin S MatteoClin Pediat II Marchi MilanInst Nacl Rehabil Serv ReumatolFirst Fac MedGen Fac HospUniversidade Federal de São Paulo (UNIFESP)Unidad Reumatol Hosp Sor Maria LudovUniv Copenhagen Hosp2016-01-24T13:52:12Z2016-01-24T13:52:12Z2009-02-01Rheumatology. Oxford: Oxford Univ Press, v. 48, n. 2, p. 119-122, 2009.1462-0324http://repositorio.unifesp.br/handle/11600/31266http://dx.doi.org/10.1093/rheumatology/ken38810.1093/rheumatology/ken388WOS:000262518500006Objectives. To determine whether demographic, clinical and immunological features may predict the outcome in juvenile SSc (JSSc).Methods. Clinical and laboratory characteristics of patients with JSSc collected from paediatric rheumatology centres worldwide were analysed. First, univariate tests identified those features significantly related with fatal outcome, and then multivariate logistic regression analysis was applied to determine the predictors of mortality.Results. One hundred and thirty-four patients from 40 centres were eligible for the analysis. Sixteen patients died and a rapidly fatal course was observed in most of them: 4/16 died within 1 yr after diagnosis and 10/16 within 5 yrs. At the moment of diagnosis, patients with poor outcome showed a significantly higher frequency of internal organ involvement, particularly cardiac, respiratory and gastrointestinal systems. No significant difference emerged for entity of skin, vascular and musculo-skeletal involvement, nor for auto-antibodies profile and laboratory tests. Multivariate analysis showed the following factors to be significant predictors of mortality: fibrosis on chest X-rays [odds ratio (OR) 11.2], raised creatinine levels (OR 22.7) and pericarditis (OR 41.3), while a short disease duration at diagnosis conferred protection (OR 0.3).Conclusions. All patients with JSSc and fatal outcome were affected by the diffuse form of the disease, and most of them showed a very rapid progression and early signs of internal organ involvement. This suggests that, in children, SSc may have two possible courses: a rapid development of internal organ failure leading to severe disability and eventually to death, or a slow course of the disease with lower mortality.Univ Padua, Dept Paediat, I-35128 Padua, ItalyCerrahpasa Tip Fak, Dept Pediat, Istanbul, TurkeyFdn IRCCS Policlin S Matteo, Dept Pediat, Pavia, ItalyClin Pediat II Marchi Milan, Milan, ItalyInst Nacl Rehabil Serv Reumatol, Mexico City, DF, MexicoFirst Fac Med, Prague, Czech RepublicGen Fac Hosp, Prague, Czech RepublicUniversidade Federal de São Paulo, Dept Pediat, São Paulo, BrazilUnidad Reumatol Hosp Sor Maria Ludov, La Plata, ArgentinaUniv Copenhagen Hosp, DK-2100 Copenhagen, DenmarkUniversidade Federal de São Paulo, Dept Pediat, São Paulo, BrazilWeb of Science119-122engOxford Univ PressRheumatologyhttp://www.oxfordjournals.org/access_purchase/self-archiving_policyb.htmlinfo:eu-repo/semantics/openAccessFactors affecting survival in juvenile systemic sclerosisinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articlereponame:Repositório Institucional da UNIFESPinstname:Universidade Federal de São Paulo (UNIFESP)instacron:UNIFESP11600/312662022-06-02 09:12:52.966metadata only accessoai:repositorio.unifesp.br:11600/31266Repositório InstitucionalPUBhttp://www.repositorio.unifesp.br/oai/requestopendoar:34652022-06-02T12:12:52Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP)false |
dc.title.en.fl_str_mv |
Factors affecting survival in juvenile systemic sclerosis |
title |
Factors affecting survival in juvenile systemic sclerosis |
spellingShingle |
Factors affecting survival in juvenile systemic sclerosis Martini, G. |
title_short |
Factors affecting survival in juvenile systemic sclerosis |
title_full |
Factors affecting survival in juvenile systemic sclerosis |
title_fullStr |
Factors affecting survival in juvenile systemic sclerosis |
title_full_unstemmed |
Factors affecting survival in juvenile systemic sclerosis |
title_sort |
Factors affecting survival in juvenile systemic sclerosis |
author |
Martini, G. |
author_facet |
Martini, G. Vittadello, F. Kasapcopur, Oe Magni Manzoni, S. Corona, F. Duarte-Salazar, C. Nemcova, D. Len, C. A. [UNIFESP] Garay, S. M. Ullman, S. Zulian, F. Juvenile Scleroderma Working Grp P |
author_role |
author |
author2 |
Vittadello, F. Kasapcopur, Oe Magni Manzoni, S. Corona, F. Duarte-Salazar, C. Nemcova, D. Len, C. A. [UNIFESP] Garay, S. M. Ullman, S. Zulian, F. Juvenile Scleroderma Working Grp P |
author2_role |
author author author author author author author author author author author |
dc.contributor.institution.none.fl_str_mv |
Univ Padua Cerrahpasa Tip Fak Fdn IRCCS Policlin S Matteo Clin Pediat II Marchi Milan Inst Nacl Rehabil Serv Reumatol First Fac Med Gen Fac Hosp Universidade Federal de São Paulo (UNIFESP) Unidad Reumatol Hosp Sor Maria Ludov Univ Copenhagen Hosp |
dc.contributor.author.fl_str_mv |
Martini, G. Vittadello, F. Kasapcopur, Oe Magni Manzoni, S. Corona, F. Duarte-Salazar, C. Nemcova, D. Len, C. A. [UNIFESP] Garay, S. M. Ullman, S. Zulian, F. Juvenile Scleroderma Working Grp P |
description |
Objectives. To determine whether demographic, clinical and immunological features may predict the outcome in juvenile SSc (JSSc).Methods. Clinical and laboratory characteristics of patients with JSSc collected from paediatric rheumatology centres worldwide were analysed. First, univariate tests identified those features significantly related with fatal outcome, and then multivariate logistic regression analysis was applied to determine the predictors of mortality.Results. One hundred and thirty-four patients from 40 centres were eligible for the analysis. Sixteen patients died and a rapidly fatal course was observed in most of them: 4/16 died within 1 yr after diagnosis and 10/16 within 5 yrs. At the moment of diagnosis, patients with poor outcome showed a significantly higher frequency of internal organ involvement, particularly cardiac, respiratory and gastrointestinal systems. No significant difference emerged for entity of skin, vascular and musculo-skeletal involvement, nor for auto-antibodies profile and laboratory tests. Multivariate analysis showed the following factors to be significant predictors of mortality: fibrosis on chest X-rays [odds ratio (OR) 11.2], raised creatinine levels (OR 22.7) and pericarditis (OR 41.3), while a short disease duration at diagnosis conferred protection (OR 0.3).Conclusions. All patients with JSSc and fatal outcome were affected by the diffuse form of the disease, and most of them showed a very rapid progression and early signs of internal organ involvement. This suggests that, in children, SSc may have two possible courses: a rapid development of internal organ failure leading to severe disability and eventually to death, or a slow course of the disease with lower mortality. |
publishDate |
2009 |
dc.date.issued.fl_str_mv |
2009-02-01 |
dc.date.accessioned.fl_str_mv |
2016-01-24T13:52:12Z |
dc.date.available.fl_str_mv |
2016-01-24T13:52:12Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.citation.fl_str_mv |
Rheumatology. Oxford: Oxford Univ Press, v. 48, n. 2, p. 119-122, 2009. |
dc.identifier.uri.fl_str_mv |
http://repositorio.unifesp.br/handle/11600/31266 http://dx.doi.org/10.1093/rheumatology/ken388 |
dc.identifier.issn.none.fl_str_mv |
1462-0324 |
dc.identifier.doi.none.fl_str_mv |
10.1093/rheumatology/ken388 |
dc.identifier.wos.none.fl_str_mv |
WOS:000262518500006 |
identifier_str_mv |
Rheumatology. Oxford: Oxford Univ Press, v. 48, n. 2, p. 119-122, 2009. 1462-0324 10.1093/rheumatology/ken388 WOS:000262518500006 |
url |
http://repositorio.unifesp.br/handle/11600/31266 http://dx.doi.org/10.1093/rheumatology/ken388 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.ispartof.none.fl_str_mv |
Rheumatology |
dc.rights.driver.fl_str_mv |
http://www.oxfordjournals.org/access_purchase/self-archiving_policyb.html info:eu-repo/semantics/openAccess |
rights_invalid_str_mv |
http://www.oxfordjournals.org/access_purchase/self-archiving_policyb.html |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
119-122 |
dc.publisher.none.fl_str_mv |
Oxford Univ Press |
publisher.none.fl_str_mv |
Oxford Univ Press |
dc.source.none.fl_str_mv |
reponame:Repositório Institucional da UNIFESP instname:Universidade Federal de São Paulo (UNIFESP) instacron:UNIFESP |
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Universidade Federal de São Paulo (UNIFESP) |
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UNIFESP |
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UNIFESP |
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Repositório Institucional da UNIFESP |
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Repositório Institucional da UNIFESP |
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Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP) |
repository.mail.fl_str_mv |
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1802764130842574848 |