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Vascular Occlusion Training for Inclusion Body Myositis: A Novel Therapeutic Approach
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Vascular Occlusion Training for Inclusion Body Myositis: A Novel Therapeutic Approach

Published on: June 5, 2010

How to diagnose and treat the inflammatory myopathies

M C Dalakas1

  • 1Neuromuscular Disease Section, NINDS, National Institutes of Health, Bethesda, Maryland 20892.

Seminars in Neurology
|June 1, 1994
PubMed

Insights

Polymyositis (PM), dermatomyositis (DM), and inclusion body myositis (IBM) are distinct inflammatory myopathies. Intravenous immunoglobulin (IVIG) shows promise as a safe and effective treatment for some patients unresponsive to other therapies.

Area of Science:

  • Rheumatology and Immunology
  • Neuromuscular Disorders

Background:

  • Inflammatory myopathies encompass polymyositis (PM), dermatomyositis (DM), and inclusion body myositis (IBM).
  • These conditions present with distinct clinical, immunopathological, and therapeutic response profiles.
  • PM is often a diagnosis of exclusion, while DM and IBM have more defined diagnostic criteria.

Purpose of the Study:

  • To review the characteristics and treatment responses of PM, DM, and IBM.
  • To evaluate the potential of high-dose intravenous immunoglobulin (IVIG) as a therapeutic option for these myopathies.

Main Methods:

  • Review of clinical, immunopathological, and therapeutic data for PM, DM, and IBM.
  • Analysis of treatment outcomes with conventional therapies (prednisone, immunosuppressants) and IVIG.
  • Inclusion of findings from controlled and uncontrolled studies.

Main Results:

  • PM and DM show some response to prednisone and immunosuppressants, while IBM is largely resistant.
  • High-dose IVIG is identified as a potentially safe and encouraging new treatment modality.
  • IVIG demonstrated efficacy in a controlled study for DM and in uncontrolled studies for some PM and IBM patients.

Conclusions:

  • Inflammatory myopathies have unique autoimmune underpinnings, including T-cell mediated cytotoxicity (PM, IBM) and complement-mediated microangiopathy (DM).
  • While conventional therapies offer limited success for some, IVIG presents a promising alternative for refractory cases of DM, PM, and IBM.
  • Further research into IVIG's role in managing inflammatory myopathies is warranted.

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