Damage extent and predictors in adult and juvenile dermatomyositis and polymyositis as determined with the myositis damage index

Abstract

Objective. We undertook this study to validate the Myositis Damage Index (MDI) in juvenile and adult myositis, to describe the degree and types of damage and to develop predictors of damage.Methods. Retrospective MDI evaluations and prospective assessment of disease activity and illness features were conducted. Patients with juvenile-onset disease (n ‫؍‬ 143) were evaluated a median of 18 months after diagnosis; 135 patients were assessed 7-9 months later, and 121 were last assessed a median of 82 months after diagnosis. Ninety-six patients with adult-onset dermatomyositis or polymyositis had a baseline assessment a median of 30 months after diagnosis; 77 patients had a 6-month followup evaluation, and 55 had a final assessment a median of 60 months after diagnosis.Results. Damage was present in 79% of juvenile patients and in 97% of adult patients. In juveniles, scarring, contractures, persistent weakness, muscle dysfunction, and calcinosis were most frequent (23-30%) at the last evaluation. In adults, muscle atrophy, muscle dysfunction, and muscle weakness were most frequent (74-84%). MDI severity correlated with physicianassessed global damage, serum creatinine, and muscle atrophy on magnetic resonance imaging, and in juveniles also with functional disability and weakness. 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, duration of active disease, disease severity at diagnosis, physician-assessed global disease activity, and illness features, including ulcerations in children and pericarditis in adults.Conclusion. Damage is common in myositis after a median duration of 5 years in patients with adultonset disease and 6.8 years in patients with juvenileonset disease. The MDI has good content, construct, and predictive validity in juvenile and adult myositis.The idiopathic inflammatory myopathies (IIMs) constitute a common cause of acquired myopathy in adults and children. These systemic autoimmune diseases, although treatable with immunosuppressive medications, often result in a chronic illness course, func-

Treatment of Juvenile Dermatomyositis: An Update

“…The overall prognosis of JDM has improved significantly over recent years, but the long term outcome differs substantially from patient to patient [4,18] suggestive of distinct clinical phenotypes. Predicting these different disease trajectories has been challenging.…”

The role of interleukin-17 in immune-mediated inflammatory myopathies and possible therapeutic implications

“…antibodies and both disease activity [42] and muscle damage [41], suggesting that the antibody has a causative role. In addition, histidyl-tRNA synthetase and Mi-2 are overexpressed in muscle tissue in PM and DM [43] and expression of Mi-2 has been shown to beincreased in regenerating fibres in DM muscles and in human myoblasts in culture [31,44].…”

Improving outcome assessment in pediatric vasculitis

“…Among the disease-specific global damage instruments, the Myositis Damage Index has been validated in JDM and pediatric modification of the Systemic Lupus International Collaborating Clinics/ACR Damage Index has been proposed [35,36].…”