Efficacy of early treatment of severe juvenile dermatomyositis with intravenous methylprednisolone and methotrexate

A pilot study was conducted to assess the efficacy of early treatment of se vere juvenile dermatomyositis (JDMS) patients with intravenous methylpredni solone (IVMP) and methotrexate (MTX). Twelve children diagnosed with severe JDMS were treated with IVMP and MTX. Six patients were treated early (with in 6 weeks of the diagnosis) while in the other six patients, MTX was start ed 5-72 months after the diagnosis was made. The clinical responses of the patients to treatment, including alterations in muscle strength, muscle enz yme levels and corticosteroid dosage as well as the development of side-eff ects, were recorded. The indications for starting the treatment were define d and documented. The primary measures of response were resolution of the c linical indications for treatment, decreased activity of the disease manife stations and tapering of the corticosteroids to the minimal dose or discont inuation without clinical or biochemical flare. The main indications for st arting IVMP and MTX were dysphagia and severe cutaneous vasculitis. All the patients received MTX orally for at least 8 months, as well as IVMP (30 mg /kg/dose), but none of the patients was on additional second-line treatment s. The six patients who were treated early with MTX showed a significant cl inical improvement. In five out of the six, the corticosteroid dosage was e ventually reduced to <5 mg/day. None of them developed calcinosis. In contr ast, two of the six patients who were treated late with MTX developed calci nosis. This study suggests that MTX and IVMP are a useful combination in th e early treatment of severe JDMS. Given the fact that our sample was small, further studies in a controlled trial are necessary to confirm these findi ngs.