title: Health-related quality of life of patients with juvenile dermatomyositis: results from the Pediatric Rheumatology International Trials Organisation multinational quality of life cohort study.
abstract: OBJECTIVE
  To investigate the health-related quality of life (HRQOL) change over time, as measured by the Child Health Questionnaire (CHQ), and its determinants in patients with active juvenile dermatomyositis (DM).
  
  
  METHODS
  We assessed patients with juvenile DM at both baseline and 6 months of followup, and healthy children age < or =18 years. Potential determinants of poor HRQOL included demographic data, physician's and parent's global assessments, muscle strength, functional ability as measured by the Childhood Health Assessment Questionnaire (C-HAQ), global disease activity assessments, and laboratory markers.
  
  
  RESULTS
  A total of 272 children with juvenile DM and 2,288 healthy children were enrolled from 37 countries. The mean +/- SD CHQ physical and psychosocial summary scores were significantly lower in children with juvenile DM (33.7 +/- 11.7 versus 54.6 +/- 4.1) than in healthy children (45.1 +/- 9.0 versus 52 +/- 7.2), with physical well-being domains being the most impaired. HRQOL improved over time in responders to treatment and remained unchanged or worsened in nonresponders. Both physical and psychosocial summary scores decreased with increasing levels of disease activity, muscle strength, and parent's evaluation of the child's overall well-being. A C-HAQ score >1.6 (odds ratio [OR] 5.06, 95% confidence interval [95% CI] 2.03-12.59), child's overall well-being score >6.2 (OR 5.24, 95% CI 2.27-12.10), and to a lesser extent muscle strength and alanine aminotransferase level were the strongest determinants of poor physical well-being at baseline. Baseline disability and longer disease duration were the major determinants for poor physical well-being at followup.
  
  
  CONCLUSIONS
  We found that patients with juvenile DM have a significant impairment in their HRQOL compared with healthy peers, particularly in the physical domain. Physical well-being was mostly affected by the level of functional impairment.
authors.affiliation: Paediatric Rheumatology International Trials Organisation, Genoa, Italy.
authors.forename: Maria Teresa
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authors.lastname: Apaz
authors.forename: Claudia
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authors.lastname: Saad-Magalhães
authors.forename: Angela
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authors.lastname: Pistorio
authors.forename: Angelo
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authors.lastname: Ravelli
authors.forename: Juliana
authors.initials: J
authors.lastname: de Oliveira Sato
authors.forename: Maria Beatriz
authors.initials: MB
authors.lastname: Marcantoni
authors.forename: Silvia
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authors.lastname: Meiorin
authors.forename: Giovanni
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authors.lastname: Filocamo
authors.forename: Clarissa
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authors.lastname: Pilkington
authors.forename: Susan
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authors.lastname: Maillard
authors.forename: Sulaiman
authors.initials: S
authors.lastname: Al-Mayouf
authors.forename: Sampath
authors.initials: S
authors.lastname: Prahalad
authors.forename: Anders
authors.initials: A
authors.lastname: Fasth
authors.forename: Rik
authors.initials: R
authors.lastname: Joos
authors.forename: Kenneth
authors.initials: K
authors.lastname: Schikler
authors.forename: Dagmar
authors.initials: D
authors.lastname: Mozolova
authors.forename: Jeanne M
authors.initials: JM
authors.lastname: Landgraf
authors.forename: Alberto
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authors.lastname: Martini
authors.forename: Nicolino
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authors.lastname: Ruperto