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Clinical analysis of 50 children with juvenile dermatomyositis

Takayuki Kishi1, Takako Miyamae, Ryoki Hara

  • 1Department of Pediatrics, Tokyo Women's Medical University, School of Medicine, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, Japan. takayuki@ped.twmu.ac.jp

Modern Rheumatology
|April 25, 2012
PubMed

Insights

Juvenile dermatomyositis (JDM) in Japanese children presents with muscle weakness and characteristic rashes. Intravenous cyclophosphamide (IVCY) shows promise for refractory cases and interstitial pneumonia.

Area of Science:

  • Pediatrics
  • Rheumatology
  • Dermatology

Background:

  • Juvenile dermatomyositis (JDM) is a rare autoimmune disease affecting children.
  • Understanding the clinical spectrum and treatment outcomes in diverse populations is crucial.

Purpose of the Study:

  • To describe the clinical characteristics, treatment, and outcomes of 50 Japanese children diagnosed with JDM.
  • To evaluate the efficacy of different treatment regimens, including pulsed methylprednisolone and intravenous cyclophosphamide.

Main Methods:

  • Retrospective review of medical records of 50 Japanese children with JDM treated between 1983 and 2008.
  • Analysis of demographic data, clinical manifestations, laboratory findings, imaging, treatment strategies, and patient outcomes.

Main Results:

  • The mean age of disease onset was 6.9 years, with a female predominance (36 girls, 14 boys).
  • Common symptoms included muscle pain/weakness (90%), malar rash (90%), Gottron's papules (86%), and heliotrope rash (80%).
  • Pulsed methylprednisolone (mPSL) was more effective than oral prednisolone (PSL), and intravenous cyclophosphamide (IVCY) showed efficacy in refractory cases and interstitial pneumonia.

Conclusions:

  • JDM exhibits systemic inflammatory and vasculopathic features.
  • IVCY may be a valuable treatment option for refractory JDM and associated interstitial pneumonia.
Abstract

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