Juvenile dermatomyositis: clinical profile and disease course in 25 patients

Clin Exp Rheumatol. Jan-Feb 1999;17(1):115-8.

Abstract

A retrospective analysis of 25 Arab patients with juvenile dermatomyositis (JDMS) was conducted between 1988 and 1996. The mean age at disease onset was 8.25 years (range 1.5-15 yrs) with a male: female ratio of 1.5:1. The disease duration before diagnosis was 1-108 months. Two patients had a family history of JDMS. The clinical features included fever in 14 patients (56%), weight loss in 20 (80%), muscle weakness in all 25 (100%), and muscle pain in 14 (56%). Skin lesions included Gottron's papules in 15 patients (60%), heliotrope in 13 (52%), erythematous malar rash in 8 (32%), and pigmentary changes in 12 (48%). Seventeen of the 25 patients had arthralgia (68%) and 16 patients had arthritis (64%). Gastrointestinal symptoms were noted in 19 patients (76%). Myocarditis with cardiac failure was the initial presentation of 1 patient, while 2 had conduction defect. Twelve patients (48%) had respiratory symptoms. The course of the disease was complicated by calcinosis in 10 patients (40%). All of the patients were treated with prednisone; 15 were also treated with methotrexate. The duration of follow up ranged from 6-108 months (mean 54.5 months). Twenty-three patients improved, including those who had calcinosis at the time of presentation, with a current muscle power of 4/5 in 10 patients (40%) and 5/5 in 13 patients (52%). No deaths were reported in our series and no patients are currently bedridden.

MeSH terms

  • Adolescent
  • Calcinosis / complications
  • Calcinosis / pathology
  • Child
  • Child, Preschool
  • Dermatologic Agents / therapeutic use
  • Dermatomyositis / complications
  • Dermatomyositis / drug therapy
  • Dermatomyositis / pathology*
  • Drug Therapy, Combination
  • Female
  • Glucocorticoids / therapeutic use
  • Humans
  • Hydroxychloroquine / therapeutic use
  • Immunosuppressive Agents / therapeutic use
  • Infant
  • Male
  • Methotrexate / therapeutic use
  • Prednisolone / therapeutic use
  • Retrospective Studies
  • Vasculitis / complications
  • Vasculitis / pathology

Substances

  • Dermatologic Agents
  • Glucocorticoids
  • Immunosuppressive Agents
  • Hydroxychloroquine
  • Prednisolone
  • Methotrexate