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Aggressive management of juvenile dermatomyositis results in improved outcome and decreased incidence of calcinosis

2002; Elsevier BV; Volume: 47; Issue: 4 Linguagem: Inglês

10.1067/mjd.2002.122196

1097-6787

Rita E. Fisler, Marilyn G. Liang, Robert C. Fuhlbrigge, Ali Yalçindağ, Robert P. Sundel,

Dermatological and Skeletal Disorders

Long-term consequences of juvenile dermatomyositis (JDM) include onset of calcinosis and subsequent functional impairment. Historic incidence of calcinosis has been reported between approximately 23% and 70%. Recent reports note improved outcome with high-dose steroids, yet the incidence of calcinosis has remained above 30%.We attempted to determine whether rapid, aggressive disease management can prevent calcinosis and improve functional outcome.Medical records of children with JDM managed at a pediatric medical center during a 10-year period were reviewed to determine (1) interval between onset of symptoms and diagnosis, (2) treatment modality, and (3) functional outcome and presence of calcinosis.A total of 21 female and 14 male subjects diagnosed with JDM met inclusion criteria, with a mean age of diagnosis of 7.6 +/- 3.9 years. Mean time from onset of symptoms to treatment was 6.6 +/- 8.2 months. Pulse intravenous methylprednisone (30 mg/kg daily) or high-dose prednisone was used in 31 of 35 patients. Patients who failed to respond within 6 weeks were started on a regimen of methotrexate (23/35). At follow-up, 5 patients had mild calcinosis (14%). Onset of calcinosis was associated with a longer time to diagnosis and treatment (30.6 vs 6 months, P =.003), a longer duration of elevated muscle enzymes (34 vs 12.6 months, P =.03), and longer disease duration (42.8 vs 22.2 months, P =.05).Stepwise, aggressive treatment directed at achieving rapid and complete control of muscle inflammation is highly successful in minimizing the long range sequelae of JDM, including calcinosis.