Long-Term Outcome and Prognostic Factors of Juvenile Dermatomyositis: A Multinational, Multicenter Study of 490 Patients
Autor(a) principal: | |
---|---|
Data de Publicação: | 2010 |
Outros Autores: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Institucional da UNESP |
Texto Completo: | http://dx.doi.org/10.1002/acr.20015 http://hdl.handle.net/11449/41943 |
Resumo: | Objective. To investigate the long-term outcome and prognostic factors of juvenile dermatomyositis (DM) through a multinational, multicenter study.Methods. Patients consisted of inception cohorts seen between 1980 and 2004 in 27 centers in Europe and Latin America. Predictor variables were sex, continent, ethnicity, onset year, onset age, onset type, onset manifestations, course type, disease duration, and active disease duration. Outcomes were muscle strength/endurance, continued disease activity, cumulative damage, muscle damage, cutaneous damage, calcinosis, lipodystrophy, physical function, and health-related quality of life (HRQOL).Results. A total of 490 patients with a mean disease duration of 7.7 years were included. At the cross-sectional visit, 41.2-52.8% of patients, depending on the instrument used, had reduced muscle strength/endurance, but less than 10% had severe impairment. Persistently active disease was recorded in 41.2-60.5% of the patients, depending on the activity measure used. Sixty-nine percent of the patients had cumulative damage. The frequency of calcinosis and lipodystrophy was 23.6% and 9.7%, respectively. A total of 40.7% of the patients had decreased functional ability, but only 6.5% had major impairment. Only a small fraction had decreased HRQOL. A chronic course, either polycyclic or continuous, consistently predicted a poorer outcome. Mortality rate was 3.1%.Conclusion. This study confirms the marked improvement in functional outcome of juvenile DM when compared with earlier literature. However, many patients had continued disease activity and cumulative damage at followup. A chronic course was the strongest predictor of poor prognosis. These findings highlight the need for treatment strategies that enable a better control of disease activity over time and the reduction of nonreversible damage. |
id |
UNSP_6df794afeff0cdfdd5c21be4976a175f |
---|---|
oai_identifier_str |
oai:repositorio.unesp.br:11449/41943 |
network_acronym_str |
UNSP |
network_name_str |
Repositório Institucional da UNESP |
repository_id_str |
2946 |
spelling |
Long-Term Outcome and Prognostic Factors of Juvenile Dermatomyositis: A Multinational, Multicenter Study of 490 PatientsObjective. To investigate the long-term outcome and prognostic factors of juvenile dermatomyositis (DM) through a multinational, multicenter study.Methods. Patients consisted of inception cohorts seen between 1980 and 2004 in 27 centers in Europe and Latin America. Predictor variables were sex, continent, ethnicity, onset year, onset age, onset type, onset manifestations, course type, disease duration, and active disease duration. Outcomes were muscle strength/endurance, continued disease activity, cumulative damage, muscle damage, cutaneous damage, calcinosis, lipodystrophy, physical function, and health-related quality of life (HRQOL).Results. A total of 490 patients with a mean disease duration of 7.7 years were included. At the cross-sectional visit, 41.2-52.8% of patients, depending on the instrument used, had reduced muscle strength/endurance, but less than 10% had severe impairment. Persistently active disease was recorded in 41.2-60.5% of the patients, depending on the activity measure used. Sixty-nine percent of the patients had cumulative damage. The frequency of calcinosis and lipodystrophy was 23.6% and 9.7%, respectively. A total of 40.7% of the patients had decreased functional ability, but only 6.5% had major impairment. Only a small fraction had decreased HRQOL. A chronic course, either polycyclic or continuous, consistently predicted a poorer outcome. Mortality rate was 3.1%.Conclusion. This study confirms the marked improvement in functional outcome of juvenile DM when compared with earlier literature. However, many patients had continued disease activity and cumulative damage at followup. A chronic course was the strongest predictor of poor prognosis. These findings highlight the need for treatment strategies that enable a better control of disease activity over time and the reduction of nonreversible damage.Myositis AssociationEuropean UnionIst Ricovero & Cura Carattere Sci G Gaslini, Genoa, ItalyUniv Genoa, Genoa, ItalyGreat Ormond St Hosp Sick Children, London WC1N 3JH, EnglandUCL, Inst Child Hlth, London, EnglandUniv Fed Rio de Janeiro, Rio de Janeiro, BrazilUniversidade do Estado do Rio de Janeiro (UERJ), BR-20550011 Rio de Janeiro, BrazilHosp Gen Ninos Pedro de Elizalde, Buenos Aires, DF, ArgentinaFdn IRCCS Policlin, Milan, ItalyHosp Pediat Juan P Garrahan, Buenos Aires, DF, ArgentinaUniv São Paulo, Fac Med Ribeirao Preto, Hosp Clin, Ribeirao Preto, BrazilHosp Gen Mexico City, Mexico City, DF, MexicoFdn Ist Ricovero & Cura Carattere Sci Policlin S, Pavia, ItalyCtr Med Natl La Raza, Mexico City, DF, MexicoHosp Pequeno Principe, Curitiba, Parana, BrazilClin Pediat 1, Padua, ItalyCtr Med Nacl Siglo XXI, Mexico City, DF, MexicoOsped Pediat Bambino Gesu, Rome, ItalyOsped Villa Monna Tessa, Florence, ItalyUniv Naples Federico 2, Naples, ItalyIst Ortoped Gaetano Pini, Milan, ItalyUniv estadual Paulista, Botucatu, SP, BrazilII Clin Pediat, Cagliari, ItalyUniv São Paulo, São Paulo, BrazilIst Ricovero & Cura Carattere Sci Burlo Garofalo, Trieste, ItalyUniv estadual Paulista, Botucatu, SP, BrazilEU: AML/B7-311/970666/II-0246-FIWiley-lissIst Ricovero & Cura Carattere Sci G GasliniUniv GenoaGreat Ormond St Hosp Sick ChildrenUCLUniversidade Federal do Rio de Janeiro (UFRJ)Universidade do Estado do Rio de Janeiro (UERJ)Hosp Gen Ninos Pedro de ElizaldeFdn IRCCS PoliclinHosp Pediat Juan P GarrahanUniversidade de São Paulo (USP)Hosp Gen Mexico CityFdn Ist Ricovero & Cura Carattere Sci Policlin SCtr Med Natl La RazaHosp Pequeno PrincipeClin Pediat 1Ctr Med Nacl Siglo XXIOsped Pediat Bambino GesuOsped Villa Monna TessaUniv Naples Federico 2Ist Ortoped Gaetano PiniUniversidade Estadual Paulista (Unesp)II Clin PediatIst Ricovero & Cura Carattere Sci Burlo GarofaloRavelli, AngeloTrail, LuciaFerrari, CristinaRuperto, NicolinoPistorio, AngelaPilkington, ClarissaMaillard, SusanOliveira, Sheila K.Sztajnbok, FlavioCuttica, RubenBeltramelli, MatildeCorona, FabriziaMartha Katsicas, MariaRusso, RicardoFerriani, VirginiaBurgos-Vargas, RubenMagni-Manzoni, SilviaSolis-Vallejo, EuniceBandeira, MarciaZulian, FrancescoBaca, VicenteCortis, ElisabettaFalcini, FernandaAlessio, MariaAlpigiani, Maria GianninaGerloni, ValeriaSaad-Magalhaes, Claudia [UNESP]Podda, RosannaSilva, Clovis A.Lepore, LoredanaFelici, EnricoRossi, FedericaSala, ElenaMartini, Alberto2014-05-20T15:33:16Z2014-05-20T15:33:16Z2010-01-15info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/article63-72http://dx.doi.org/10.1002/acr.20015Arthritis Care & Research. Hoboken: Wiley-liss, v. 62, n. 1, p. 63-72, 2010.2151-464Xhttp://hdl.handle.net/11449/4194310.1002/acr.20015WOS:00028097850000970983100083716320000-0002-7631-7093Web of Sciencereponame:Repositório Institucional da UNESPinstname:Universidade Estadual Paulista (UNESP)instacron:UNESPengArthritis Care & Research4.149info:eu-repo/semantics/openAccess2021-12-06T17:54:08Zoai:repositorio.unesp.br:11449/41943Repositório InstitucionalPUBhttp://repositorio.unesp.br/oai/requestopendoar:29462021-12-06T17:54:08Repositório Institucional da UNESP - Universidade Estadual Paulista (UNESP)false |
dc.title.none.fl_str_mv |
Long-Term Outcome and Prognostic Factors of Juvenile Dermatomyositis: A Multinational, Multicenter Study of 490 Patients |
title |
Long-Term Outcome and Prognostic Factors of Juvenile Dermatomyositis: A Multinational, Multicenter Study of 490 Patients |
spellingShingle |
Long-Term Outcome and Prognostic Factors of Juvenile Dermatomyositis: A Multinational, Multicenter Study of 490 Patients Ravelli, Angelo |
title_short |
Long-Term Outcome and Prognostic Factors of Juvenile Dermatomyositis: A Multinational, Multicenter Study of 490 Patients |
title_full |
Long-Term Outcome and Prognostic Factors of Juvenile Dermatomyositis: A Multinational, Multicenter Study of 490 Patients |
title_fullStr |
Long-Term Outcome and Prognostic Factors of Juvenile Dermatomyositis: A Multinational, Multicenter Study of 490 Patients |
title_full_unstemmed |
Long-Term Outcome and Prognostic Factors of Juvenile Dermatomyositis: A Multinational, Multicenter Study of 490 Patients |
title_sort |
Long-Term Outcome and Prognostic Factors of Juvenile Dermatomyositis: A Multinational, Multicenter Study of 490 Patients |
author |
Ravelli, Angelo |
author_facet |
Ravelli, Angelo Trail, Lucia Ferrari, Cristina Ruperto, Nicolino Pistorio, Angela Pilkington, Clarissa Maillard, Susan Oliveira, Sheila K. Sztajnbok, Flavio Cuttica, Ruben Beltramelli, Matilde Corona, Fabrizia Martha Katsicas, Maria Russo, Ricardo Ferriani, Virginia Burgos-Vargas, Ruben Magni-Manzoni, Silvia Solis-Vallejo, Eunice Bandeira, Marcia Zulian, Francesco Baca, Vicente Cortis, Elisabetta Falcini, Fernanda Alessio, Maria Alpigiani, Maria Giannina Gerloni, Valeria Saad-Magalhaes, Claudia [UNESP] Podda, Rosanna Silva, Clovis A. Lepore, Loredana Felici, Enrico Rossi, Federica Sala, Elena Martini, Alberto |
author_role |
author |
author2 |
Trail, Lucia Ferrari, Cristina Ruperto, Nicolino Pistorio, Angela Pilkington, Clarissa Maillard, Susan Oliveira, Sheila K. Sztajnbok, Flavio Cuttica, Ruben Beltramelli, Matilde Corona, Fabrizia Martha Katsicas, Maria Russo, Ricardo Ferriani, Virginia Burgos-Vargas, Ruben Magni-Manzoni, Silvia Solis-Vallejo, Eunice Bandeira, Marcia Zulian, Francesco Baca, Vicente Cortis, Elisabetta Falcini, Fernanda Alessio, Maria Alpigiani, Maria Giannina Gerloni, Valeria Saad-Magalhaes, Claudia [UNESP] Podda, Rosanna Silva, Clovis A. Lepore, Loredana Felici, Enrico Rossi, Federica Sala, Elena Martini, Alberto |
author2_role |
author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author |
dc.contributor.none.fl_str_mv |
Ist Ricovero & Cura Carattere Sci G Gaslini Univ Genoa Great Ormond St Hosp Sick Children UCL Universidade Federal do Rio de Janeiro (UFRJ) Universidade do Estado do Rio de Janeiro (UERJ) Hosp Gen Ninos Pedro de Elizalde Fdn IRCCS Policlin Hosp Pediat Juan P Garrahan Universidade de São Paulo (USP) Hosp Gen Mexico City Fdn Ist Ricovero & Cura Carattere Sci Policlin S Ctr Med Natl La Raza Hosp Pequeno Principe Clin Pediat 1 Ctr Med Nacl Siglo XXI Osped Pediat Bambino Gesu Osped Villa Monna Tessa Univ Naples Federico 2 Ist Ortoped Gaetano Pini Universidade Estadual Paulista (Unesp) II Clin Pediat Ist Ricovero & Cura Carattere Sci Burlo Garofalo |
dc.contributor.author.fl_str_mv |
Ravelli, Angelo Trail, Lucia Ferrari, Cristina Ruperto, Nicolino Pistorio, Angela Pilkington, Clarissa Maillard, Susan Oliveira, Sheila K. Sztajnbok, Flavio Cuttica, Ruben Beltramelli, Matilde Corona, Fabrizia Martha Katsicas, Maria Russo, Ricardo Ferriani, Virginia Burgos-Vargas, Ruben Magni-Manzoni, Silvia Solis-Vallejo, Eunice Bandeira, Marcia Zulian, Francesco Baca, Vicente Cortis, Elisabetta Falcini, Fernanda Alessio, Maria Alpigiani, Maria Giannina Gerloni, Valeria Saad-Magalhaes, Claudia [UNESP] Podda, Rosanna Silva, Clovis A. Lepore, Loredana Felici, Enrico Rossi, Federica Sala, Elena Martini, Alberto |
description |
Objective. To investigate the long-term outcome and prognostic factors of juvenile dermatomyositis (DM) through a multinational, multicenter study.Methods. Patients consisted of inception cohorts seen between 1980 and 2004 in 27 centers in Europe and Latin America. Predictor variables were sex, continent, ethnicity, onset year, onset age, onset type, onset manifestations, course type, disease duration, and active disease duration. Outcomes were muscle strength/endurance, continued disease activity, cumulative damage, muscle damage, cutaneous damage, calcinosis, lipodystrophy, physical function, and health-related quality of life (HRQOL).Results. A total of 490 patients with a mean disease duration of 7.7 years were included. At the cross-sectional visit, 41.2-52.8% of patients, depending on the instrument used, had reduced muscle strength/endurance, but less than 10% had severe impairment. Persistently active disease was recorded in 41.2-60.5% of the patients, depending on the activity measure used. Sixty-nine percent of the patients had cumulative damage. The frequency of calcinosis and lipodystrophy was 23.6% and 9.7%, respectively. A total of 40.7% of the patients had decreased functional ability, but only 6.5% had major impairment. Only a small fraction had decreased HRQOL. A chronic course, either polycyclic or continuous, consistently predicted a poorer outcome. Mortality rate was 3.1%.Conclusion. This study confirms the marked improvement in functional outcome of juvenile DM when compared with earlier literature. However, many patients had continued disease activity and cumulative damage at followup. A chronic course was the strongest predictor of poor prognosis. These findings highlight the need for treatment strategies that enable a better control of disease activity over time and the reduction of nonreversible damage. |
publishDate |
2010 |
dc.date.none.fl_str_mv |
2010-01-15 2014-05-20T15:33:16Z 2014-05-20T15:33:16Z |
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.uri.fl_str_mv |
http://dx.doi.org/10.1002/acr.20015 Arthritis Care & Research. Hoboken: Wiley-liss, v. 62, n. 1, p. 63-72, 2010. 2151-464X http://hdl.handle.net/11449/41943 10.1002/acr.20015 WOS:000280978500009 7098310008371632 0000-0002-7631-7093 |
url |
http://dx.doi.org/10.1002/acr.20015 http://hdl.handle.net/11449/41943 |
identifier_str_mv |
Arthritis Care & Research. Hoboken: Wiley-liss, v. 62, n. 1, p. 63-72, 2010. 2151-464X 10.1002/acr.20015 WOS:000280978500009 7098310008371632 0000-0002-7631-7093 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
Arthritis Care & Research 4.149 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
63-72 |
dc.publisher.none.fl_str_mv |
Wiley-liss |
publisher.none.fl_str_mv |
Wiley-liss |
dc.source.none.fl_str_mv |
Web of Science reponame:Repositório Institucional da UNESP instname:Universidade Estadual Paulista (UNESP) instacron:UNESP |
instname_str |
Universidade Estadual Paulista (UNESP) |
instacron_str |
UNESP |
institution |
UNESP |
reponame_str |
Repositório Institucional da UNESP |
collection |
Repositório Institucional da UNESP |
repository.name.fl_str_mv |
Repositório Institucional da UNESP - Universidade Estadual Paulista (UNESP) |
repository.mail.fl_str_mv |
|
_version_ |
1792961676892438528 |