Immune-mediated necrotizing myopathy
ORPHA:206569· ICD-10 G72.4
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.
- Prevalence
- 1-9 / 100 000
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly