title: Development of validated disease activity and damage indices for the juvenile idiopathic inflammatory myopathies: I. Physician, parent, and patient global assessments. Juvenile Dermatomyositis Disease Activity Collaborative Study Group.
abstract: OBJECTIVE
  To determine the reliability, content validity, and responsiveness of physician global assessments of disease activity and damage in the juvenile idiopathic inflammatory myopathies (IIM), and to investigate concordance among physician, parent, and patient global ratings.
  
  
  METHODS
  Sixteen pediatric rheumatologists rated 10 juvenile IIM paper patient cases for global disease activity and damage, and assessed the importance of 51 clinical and laboratory parameters in formulating their global assessments. Then, 117 juvenile IIM patients were enrolled in a protocol to examine the relationship between Likert and visual analog scale global assessments, their sensitivity to change, and the comparability of physician, parent, and patient global ratings.
  
  
  RESULTS
  Pediatric rheumatologists demonstrated excellent interrater reliability in their global assessments of juvenile IIM disease activity and damage (97.7% and 94.7% agreement among raters, respectively), and agreed on a core set of clinical parameters in formulating their judgments. Likert scale ratings correlated with those on a visual analog scale, and both were comparable in responsiveness (standardized response means -0.56 for disease activity, 0.02 [Likert] and 0.14 [visual analog] for damage, measured over 8 months). Parent global ratings of disease activity correlated with physician assessments, but were not colinear (Spearman's correlation [r] = 0.41-0.45). Patient global disease activity assessments correlated with those done by parents (r = 0.57-0.84) and physicians (r = 0.37-0.63), but demonstrated less responsiveness (standardized response means -0.21 and -0.12, respectively, over 8 months).
  
  
  CONCLUSIONS
  Physician global assessments of juvenile IIM disease activity and damage demonstrated high interrater reliability and were shown to be comprehensive measures. Both physician and parent disease activity assessments should be considered valuable as quantitative measures for evaluating therapeutic responses in juvenile IIM patients.
authors.affiliation: Food and Drug Administration, and National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland 20892, USA.
authors.forename: L G
authors.initials: LG
authors.lastname: Rider
authors.forename: B M
authors.initials: BM
authors.lastname: Feldman
authors.forename: M D
authors.initials: MD
authors.lastname: Perez
authors.forename: R M
authors.initials: RM
authors.lastname: Rennebohm
authors.forename: C B
authors.initials: CB
authors.lastname: Lindsley
authors.forename: L S
authors.initials: LS
authors.lastname: Zemel
authors.forename: C A
authors.initials: CA
authors.lastname: Wallace
authors.forename: S H
authors.initials: SH
authors.lastname: Ballinger
authors.forename: S L
authors.initials: SL
authors.lastname: Bowyer
authors.forename: A M
authors.initials: AM
authors.lastname: Reed
authors.forename: M H
authors.initials: MH
authors.lastname: Passo
authors.forename: I M
authors.initials: IM
authors.lastname: Katona
authors.forename: F W
authors.initials: FW
authors.lastname: Miller
authors.forename: P A
authors.initials: PA
authors.lastname: Lachenbruch
mesh_terms: D001171:Arthritis, Juvenile / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D006801:Humans; D015588:Observer Variation; D010147:Pain Measurement; D010290:Parents; D010361:Patients; D010820:Physicians; D015203:Reproducibility of Results; D012720:Severity of Illness Index