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Antisynthetase syndrome

ORPHA:81· ICD-10 M35.8

Definition

A rare idiopathic inflammatory myopathy (IIM) characterized principally by myositis, generally symmetrical arthritis and interstitial lung disease (ILD) in association with serum autoantibodies to aminoacyl-transfer RNA synthetases (anti-ARS). More variable features include arthralgia, Raynaud phenomenon, heliotrophic rash, distal esophageal dysmotility and mechanic's hands.

Prevalence
1-9 / 100 000
Inheritance
Not applicable
Age of onset
Adult, Elderly