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Update on Inclusion Body Myositis

  • Duaa Jabari
  • V. V. Vedanarayanan
  • Richard J. Barohn
  • Mazen M. Dimachkie
Inflammatory Muscle Disease (R Aggarwal, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Inflammatory Muscle Disease

Abstract

Purpose of Review

While sporadic inclusion body myositis (sIBM) is the most common acquired muscle disease after age 50, the pathogenesis of this disease is still poorly understood. In this review, we discuss our current state of knowledge in sIBM and provide an update on our current understanding of its pathophysiology and management.

Recent Findings

Lines of evidence in support of an inflammatory pathogenesis include inflammatory infiltrates in the target organ, NFκB activation, cytokine response, MHC I upregulation, and cN1A antibody. Refractoriness to immunotherapies has led to suggestion of a degenerative pathophysiology. Evidence for impaired protein homeostasis with misfolding burden is coupled with findings of endoplasmic reticulum stress, proteasome dysfunction, and mitochondrial lesion. Recent treatment trials have focused more on correcting the degenerative process or muscle growth rather than controlling the inflammation.

Summary

There has been growing evidence toward degeneration as the primary process in sIBM. This is consistent with the refractory nature of this disease. Improving our understanding of this disease pathogenesis will propel efforts to find an effective therapy.

Keywords

Inclusion body myositis Clinical presentation Pathogenesis Diagnostic criteria Treatment 

Notes

Compliance With Ethical Standards

Conflict of Interest

Dr. Barohn reports personal fees from NuFactor, Plan 365 Inc., Novartis Pharmaceuticals, Option Care, and PlatformQ Health Education and grants from Eli Lilly and Company, PTC Therapeutics, Cytokinetics, Inc., Neuraltus Pharmaceuticals, Inc., Alexion Pharmaceuticals, Inc., ALSA, MDA-Myotonic Dystrophy Foundation, The Marigold Foundation, Sarepta Therapeutics, Ionis Pharmaceuticals, TEVA Pharmaceuticals, Biomarin, Sanofi/Genzyme, NIH, FDA/OOPD, and outside the submitted work.

Dr. Dimachkie is a consultant or on the speaker’s bureau for Alnylam, Audentes, Biomarin, Catalyst, CSL-Behring, Genzyme, Mallinckrodt, Novartis, NuFactor, Octapharma, Sanofi, Shire and Terumo. Dr. Dimachkie received grants from Alexion, Alnylam, Amicus, Biomarin, Bristol-Myers Squibb, Catalyst, CSL-Behring, FDA/OPD, GlaxoSmithKline, Genentech, Grifols, MDA, NIH, Novartis, Genzyme, Octapharma, UCB Biopharma, Viromed and TMA.

Drs. Jabari and Vedanarayanan have nothing to disclose.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Duaa Jabari
    • 1
  • V. V. Vedanarayanan
    • 2
  • Richard J. Barohn
    • 3
  • Mazen M. Dimachkie
    • 4
  1. 1.Neuromuscular Division, Department of NeurologyUniversity of Kansas Medical CenterKansas CityUSA
  2. 2.Neuromuscular MedicineMississippi Center for Advanced MedicineMadisonUSA
  3. 3.Frontiers: University of Kansas Clinical & Translational Science Institute, University of Kansas Medical Center Kansas CityUSA
  4. 4.Neuromuscular Division, Department of Neurology and the Institute for Neurological DiscoveriesThe University of Kansas Medical CenterKansas CityUSA

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