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Antisynthetase syndrome

Disease 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.


Classification level: Disorder
  • Synonym(s):
    • AS syndrome
    • Anti-Jo1 syndrome
  • Prevalence: 1-9 / 100 000
  • Inheritance: Not applicable 
  • Age of onset: Adult, Elderly
  • ICD-10: M35.8
  • ICD-11: 4A43.Y
  • OMIM: -
  • UMLS: C2609059
  • MeSH: C537778
  • GARD: 735
  • MedDRA: 10068801

Detailed information

General public


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