4A41.10
category
Juvenile polymyositis
Definition
Juvenile polymyositis is a rare childhood idiopathic inflammatory myopathy. It is frequently misdiagnosed, as it lacks a unique clinical phenotype. Traditionally, it presents with weakness of the proximal muscles that evolves over weeks to months. The primary histologic features are fibre size variability, scattered necrotic and regenerating fibres, and perivascular and endomysial cellular infiltrates.
Exclusions
- Antiphospholipid syndrome
(4A45) - Lupus erythematosus
(4A40) - Overlap or undifferentiated nonorgan specific systemic autoimmune disease
(4A43) - Systemic sclerosis
(4A42) - Vasculitis
(4A44)
Also indexed as
Juvenile polymyositisJuvenile polymyositis with PM-Scl auto-antibodyJuvenile polymyositis with anti-synthetase auto-antibodyJuvenile polymyositis with interstitial lung diseaseJuvenile polymyositis with overlap to non-organ specific systemic autoimmune disorderJuvenile polymyositis, necrotisingJuvenile polymyositis, paraneoplastic