Idiopathic Inflammatory Myopathies: A Review of the Classification and Impact of Pathogenesis

Int J Mol Sci. 2017 May 18;18(5):1084. doi: 10.3390/ijms18051084.


Idiopathic inflammatory myopathies (IIMs) are a group of autoimmune muscle diseases with significant morbidity and mortality. This review details and updates the pathogenesis and emerging importance of myositis-specific antibodies in the development of IIMs. An increase in the understanding of how these myositis-specific antibodies play a role in IIMs has led to the further categorization of IIMs from the traditional polymyositis versus dermatomyositis, to additional subcategories of IIMs such as necrotizing autoimmune myositis (NAM). The diagnosis of IIMs, including manual muscle testing, laboratory studies, and non-invasive imaging have become important in classifying IIM subtypes and for identifying disease severity. Treatment has evolved from an era where glucocorticoid therapy was the only option to a time now that includes traditional steroid-sparing agents along with immunoglobulin therapy and biologics, such as rituximab.

Keywords: idiopathic; inflammation; muscle diseases.

Publication types

  • Review

MeSH terms

  • Animals
  • Autoantibodies / immunology
  • Autoantibodies / metabolism
  • Autoimmune Diseases / immunology*
  • Autoimmune Diseases / metabolism*
  • Dermatomyositis / immunology
  • Dermatomyositis / metabolism
  • Humans
  • Myositis / immunology
  • Myositis / metabolism*
  • Polymyositis / immunology
  • Polymyositis / metabolism


  • Autoantibodies