vitalwiki

Імуноопосередкована некротизуюча міопатія

ORPHA:206569· ICD-10 G72.4· Immune-mediated necrotizing myopathy

Визначення(English summary)

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.

Поширеність
1-9 / 100 000
Успадкування
Not applicable
Вік початку
Adolescent, Adult, Childhood, Elderly