Autoantibodies of inflammatory myopathies: Update

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Inflammatory myopathies are a heterogeneous group of immune-mediated diseases that involve the skeletal muscle as well as many other organs. In addition to a histological diagnosis at muscle biopsy, the clinical phenotypes of inflammatory myopathies can be defined by the presence of various autoantibodies that are originally detected by RNA or protein immunoprecipitation. However, the correlation between histological features and autoantibodies has not been fully elucidated. Immune-mediated necrotizing myopathy (IMNM), which is characterized by significant necrotic and regeneration muscle fibers with minimal or no inflammatory cell infiltration, is associated with the presence of autoantibodies. IMNM is now classified as a distinct category of inflammatory myopathies, separate from polymyositis, dermatomyositis, and sporadic inclusion body myositis. Here, we divided the autoantibodies of inflammatory myopathies into the following categories: those associated with IMNM, those with activity against aminoacyl transfer RNA synthetase, those associated with dermatomyositis, and those related to other disorders, including overlap syndrome, inclusion body myositis, and primary biliary cirrhosis. The detection of autoantibodies against signal recognition particle or 3-hydroxy-3-methylglutaryl-coenzyme A reductase is useful for the diagnosis of IMNM. The screening of autoantibodies has clinical relevance for managing patients with inflammatory myopathies.

Original languageEnglish
Pages (from-to)1443-1451
Number of pages9
JournalBrain and Nerve
Issue number12
Publication statusPublished - 2016 Dec 1


  • 3-hydroxy-3-methylglutarycoenzyme A reductase (HMGCR)
  • Autoantibodies
  • Enzyme-linked immunosorbent assay (ELISA)
  • Immune-mediated necrotizing myopathy (IMNM)
  • Signal recognition particle (SRP)

ASJC Scopus subject areas

  • Clinical Neurology


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