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Immune-mediated necrotizing myopathy

Disease definition

A rare form of idiopathic inflammatory myopathy characterized by acute or subacute, severe, symmetrical, proximal muscle weakness usually associated with muscle-specific antibodies (anti-HMGCR or anti-SRP). Histopathological characteristics include myocyte necrosis and regeneration without significant inflammation, and C5b-9 deposition on non-necrotic myofibers.

ORPHA:206569

Classification level: Disorder
  • Synonym(s):
    • Anti-HMG-CoA myopathy
    • Anti-SRP myopathy
    • Autoimmune necrotizing myositis
    • IMNM
    • Immune myopathy with myocyte necrosis
    • NAM
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Adult, Elderly, Childhood, Adolescent
  • ICD-10: G72.4
  • OMIM: -
  • UMLS: C3267047
  • MeSH: -
  • GARD: -
  • MedDRA: -

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