Failure of pulse intravenous methylprednisolone treatment in juvenile dermatomyositis

Objective. To review the experience with high-dose intravenous pulse methylprednisolone (IVMP) therapy in patients with juvenile dermatomyositis (JDM) in our institution. Study design. We reviewed the charts of seven consecutive patients (four female subjects; three male subjects; age 3 to 18 years [mean age 8 years]) treated for JDM between 1989 and 1992. Results. All patients met the criteria of Bahan and Peter for JDM and were treated within 3 months of onset of weakness. All received initial treatment with IVMP, 30 mg/kg per day for 3 days, administered weekly for between 1 and 4 weeks. Treatment with orally administered prednisone was required because of deteriorating muscle strength and persistent elevation of creatine kinase activity despite IVMP in five patients, increased skin vasculitis in one patient, and elevated creatine kinase activity with no improvement in strength in one patient. Conclusion. We conclude that muscle strength in patients with JDM may deteriorate during pulse IVMP therapy when this is used alone as initial treatment, even early in the course of the illness. We recommend either conventional high doses of orally administered corticosteroids or IVMP with oral corticosteroid therapy as initial treatment of JDM.