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Dermatomyositis and Polymyositis.

Hannah R. Briemberg1, Anthony A. Amato

  • 1Department of Neurology, Brigham and Women's Hospital, 75 Francis Street, Boston, MA 02115, USA. aamato@partners.org

Current Treatment Options in Neurology
|August 5, 2003
PubMed
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Dermatomyositis and polymyositis are inflammatory muscle diseases. Polymyositis is now considered rare due to refined diagnostic criteria, with incidence of inflammatory myopathies around 1 in 100,000.

Area of Science:

  • Neurology
  • Rheumatology
  • Immunology

Background:

  • Dermatomyositis (DM) and polymyositis (PM) are idiopathic inflammatory myopathies.
  • Characterized by muscle weakness, elevated creatine kinase, and muscle biopsy inflammation.
  • Clinical and electrophysiologic similarities exist, with muscle biopsy as the gold standard.

Purpose of the Study:

  • To clarify the diagnostic criteria and incidence of DM and PM.
  • To review current understanding of these inflammatory myopathies.
  • To discuss therapeutic approaches for DM and PM.

Main Methods:

  • Review of literature on diagnostic criteria for DM and PM.
  • Analysis of incidence data for inflammatory myopathies.
  • Discussion of treatment strategies based on clinical experience and evidence.

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Main Results:

  • Refined diagnostic criteria suggest PM is rarer than previously thought.
  • The clinical entity of PM is poorly defined.
  • Overall annual incidence of inflammatory myopathies is approximately 1 in 100,000.

Conclusions:

  • DM and PM are treatable with immunomodulating therapies.
  • First-line therapy includes high-dose oral prednisone.
  • Methotrexate or azathioprine are second-line options, with IV immunoglobulin for refractory cases.