Immune-mediert necrotiserende myopati


Engelsk navn: Immune-mediated necrotizing myopathy
Engelske synonym: Anti-HMG-CoA myopathy,Anti-SRP myopathy,Autoimmune necrotizing myositis,IMNM,Immune myopathy with myocyte necrosis,NAM


Definisjon

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.

Fra Orphanet

ORPHA: 206569
Klassifiseringsnivå: Disorder
ICD-10: G72.4

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