The Autoimmune Myopathies

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

The autoimmune myopathies (also known as myositis) comprise an important group of disorders in which skeletal muscle is one of the primary targets of the autoimmune response. The immune responses in these disorders target a limited set of ubiquitously expressed autoantigens, and there is a striking association of specific autoantibodies with distinct clinical phenotypes. Several features of these diseases, including the association with cancer, the induction of disease in response to statins, and the preferential expression of myositis-specific autoantigens in regenerating but not resting muscle cells, have provided important insights into the initiating and propagating mechanisms. Emerging data suggest a model of disease in which initiation and propagation may be separated in time and, potentially, space. In this model, increased expression of myositis-specific autoantigens in regenerating cells focuses immune injury on cells healing muscle injury, establishing a self-sustaining loop of immune-mediated muscle injury and repair. Such loops may be amenable to novel therapeutic interventions.

Original languageEnglish (US)
Title of host publicationThe Autoimmune Diseases
Subtitle of host publicationFifth Edition
PublisherElsevier Inc.
Pages547-554
Number of pages8
ISBN (Print)9780123849298
DOIs
StatePublished - Dec 1 2013

Keywords

  • Aminoacyl tRNA synthetases
  • Autoimmune myopathy
  • Cancer
  • Feed-forward cycle
  • Immune-mediated necrotizing myopathy
  • Myositis
  • Myositis-specific autoantibodies
  • Paraneoplastic
  • Regeneration
  • Statins

ASJC Scopus subject areas

  • Immunology and Microbiology(all)

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