We validated the Myositis Damage Index (MDI) in juvenile and adult myositis, to describe the degree and types of damage and to develop predictors of damage.
Retrospective MDI evaluations and prospective assessment of disease activity and illness features were conducted. Juvenile-onset patients (n = 143) were evaluated a median of 18 months after diagnosis; 135 patients were assessed 7–9 months later, and 121 were last assessed 82 months after diagnosis. Adult-onset patients (n = 96) with dermatomyositis (DM) or polymyositis (PM) had a baseline assessment a median of 30 months after diagnosis; 77 had a 6-month follow-up evaluation, and 55 had a final assessment 60 months after diagnosis.
Damage was present in 79% of juvenile and 97% of adult patients. In juveniles, scar, contractures, persistent weakness, muscle dysfunction and calcinosis (23–30%) were most frequent on last evaluation. In adults, muscle atrophy, muscle dysfunction and weakness were most frequent (74–84%). MDI severity correlated with physician global damage, functional disability, weakness and muscle atrophy on MRI. MDI damage scores and frequency were highest in patients with a chronic illness course and in adult patients who died. Predictors of damage included functional disability, active disease duration, onset severity, global activity, and illness features, including ulcerations in children and pericarditis in adults.
Damage is common in myositis patients after a median of 5 years duration in adult-onset and 6.8 years in juvenile-onset patients. The MDI has good content, construct and predictive validity in juvenile and adult myositis.