Idiopathic inflammatory myopathies

Neurol Clin. 2014 Aug;32(3):595-628, vii. doi: 10.1016/j.ncl.2014.04.007.

Abstract

The idiopathic inflammatory myopathies (IIM) consist of rare heterogeneous autoimmune disorders that present with marked proximal and symmetric muscle weakness, except for distal and asymmetric weakness in inclusion body myositis. Despite many similarities, the IIM are fairly heterogeneous from the histopathologic and pathogenetic standpoints, and also show some clinical and treatment-response differences. The field has witnessed significant advances in our understanding of the pathophysiology and treatment of these rare disorders. This review focuses on dermatomyositis, polymyositis, and necrotizing myopathy, and examines current and promising therapies.

Keywords: Clinical presentation; Dermatomyositis; Diagnosis; Inclusion body myositis; Necrotizing myopathy; Pathology; Polymyositis; Treatment.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Autoimmune Diseases / diagnosis
  • Dermatomyositis / diagnosis*
  • Dermatomyositis / immunology
  • Dermatomyositis / therapy
  • Female
  • Humans
  • Male
  • Muscle Weakness / complications
  • Myositis / diagnosis*
  • Myositis / immunology
  • Myositis / therapy
  • Myositis, Inclusion Body / diagnosis