title: Long-term outcome and prognostic factors of juvenile dermatomyositis: a multinational, multicenter study of 490 patients.
abstract: 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%.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Istituto di Ricovero e Cura a Carattere Scientifico G. Gaslini and Università degli Studi di Genoa, Genoa, Italy. angeloravelli@ospedale-gaslini.ge.it
authors.forename: Angelo
authors.initials: A
authors.lastname: Ravelli
authors.forename: Lucia
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authors.lastname: Trail
authors.forename: Cristina
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authors.lastname: Ferrari
authors.forename: Nicolino
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authors.lastname: Ruperto
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authors.lastname: Pistorio
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authors.forename: Sheila K
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authors.forename: Flavio
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authors.lastname: Cuttica
authors.forename: Matilde
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authors.forename: Fabrizia
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authors.lastname: Corona
authors.forename: Maria Martha
authors.initials: MM
authors.lastname: Katsicas
authors.forename: Ricardo
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authors.lastname: Russo
authors.forename: Virginia
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authors.lastname: Ferriani
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authors.lastname: Burgos-Vargas
authors.forename: Silvia
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authors.lastname: Magni-Manzoni
authors.forename: Eunice
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authors.lastname: Solis-Valleoj
authors.forename: Marcia
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authors.lastname: Bandeira
authors.forename: Francesco
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authors.lastname: Zulian
authors.forename: Vicente
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authors.forename: Elisabetta
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authors.lastname: Cortis
authors.forename: Fernanda
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authors.lastname: Falcini
authors.forename: Maria
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authors.lastname: Alessio
authors.forename: Maria Giannina
authors.initials: MG
authors.lastname: Alpigiani
authors.forename: Valeria
authors.initials: V
authors.lastname: Gerloni
authors.forename: Claudia
authors.initials: C
authors.lastname: Saad-Magalhaes
authors.forename: Rosanna
authors.initials: R
authors.lastname: Podda
authors.forename: Clovis A
authors.initials: CA
authors.lastname: Silva
authors.forename: Loredana
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authors.lastname: Lepore
authors.forename: Enrico
authors.initials: E
authors.lastname: Felici
authors.forename: Federica
authors.initials: F
authors.lastname: Rossi
authors.forename: Elena
authors.initials: E
authors.lastname: Sala
authors.forename: Alberto
authors.initials: A
authors.lastname: Martini
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D003882:Dermatomyositis / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D005260:Female; D006801:Humans; D007223:Infant; D038622:Internationality; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome