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Start free with EleplanAntisynthetase syndrome
ORPHA:81Disease
Also called AS syndrome · ASS · ASyS · Anti-ARS syndrome · Anti-Jo1 syndrome · Anti-aminoacyl-tRNA synthetase syndrome
What it is
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.
Key facts
- Prevalence
- 1-9 / 100 000
- Age of onset
- Adult, Elderly
- Inheritance
- Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
9Common30–79%
22- Anti-alanyl-tRNA synthetase antibody positivity
- Anti-aminoacyl-tRNA synthetase antibody positivity
- Anti-asparaginyl-tRNA synthetase antibody positivity
- Anti-cytosolic-5-nucleotidase-1A antibody positivity
- Anti-glycyl tRNA-synthetase antibody positivity
- Anti-histidyl tRNA synthetase antibody positivity
- Anti-isoleucyl tRNA-synthetase antibody positivity
- Anti-phenylalanyl tRNA synthetase antibody positivity
- Anti-threonyl-tRNA synthetase antibody positivity
- Anti-tyrosyl-tRNA synthetase antibody positivity
- Arthralgia
- Arthritis
- Dyspnea
- Edema
- Elevated circulating creatine kinase concentration
- EMG abnormality
- Fever
- Hypotonia
- Keratoconjunctivitis sicca
- Lack of skin elasticity
- Raynaud phenomenon
- Xerostomia
Sometimes5–29%
13- Abnormality of the voice
- Aortic regurgitation
- Dysphagia
- Hiker's feet
- Joint dislocation
- Mechanic's hands
- Myocarditis
- Neoplasm
and 5 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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