Reference
Antisynthetase Antibodies
Eight autoantibodies are currently recognised in ASyS, each targeting a different aminoacyl-tRNA synthetase enzyme. Antibody subtype influences clinical phenotype, organ involvement, and prognosis.
Explore by body system
Select an antibody to see which regions are affected and how commonly.
Jo-1
Anti-histidyl-tRNA synthetase
60–75% of ASyS cases
Lungs / ChestInterstitial lung disease (ILD)
CommonProximal arm musclesShoulder & upper arm weakness
CommonHands & fingersMechanic's hands · Raynaud's · Arthritis
CommonProximal leg musclesThigh & hip weakness (myopathy)
CommonSystemicConstitutional fever & fatigue
FrequentThroat / OesophagusDysphagia — difficulty swallowing
OccasionalThe most common antisynthetase antibody and the most studied. Classical ASyS triad (myopathy, ILD, arthritis) is most consistently seen with Jo-1.
Jo-1
60–75% of cases
Inflammatory myopathyOften the presenting feature
CommonInterstitial lung disease70–80% of Jo-1 patients
CommonArthritis / arthralgia
CommonMechanic's hands
CommonRaynaud's phenomenon
FrequentFever
FrequentDysphagia
Occasional The most common antisynthetase antibody and the most studied. Classical ASyS triad (myopathy, ILD, arthritis) is most consistently seen with Jo-1.
PL-7ILD-predominant
5–10% of cases
Interstitial lung diseaseOften severe; may precede myopathy
CommonInflammatory myopathyTypically milder than Jo-1
FrequentArthritis / arthralgia
FrequentMechanic's hands
FrequentRaynaud's phenomenon
FrequentFever
Occasional ILD is more dominant than myopathy. Patients may present to respiratory physicians before a rheumatology diagnosis is made.
PL-12ILD-predominant
5–10% of cases
Interstitial lung diseaseOften severe and progressive
CommonRaynaud's phenomenon
CommonMechanic's hands
FrequentInflammatory myopathyLess prominent than other subtypes
OccasionalArthritis / arthralgia
OccasionalFever
Occasional Strongly ILD-predominant. Myopathy may be subclinical or absent. Often initially misdiagnosed as idiopathic pulmonary fibrosis.
EJ
3–5% of cases
Interstitial lung disease
CommonInflammatory myopathy
CommonArthritis / arthralgia
FrequentMechanic's hands
FrequentRaynaud's phenomenon
OccasionalFever
Occasional Phenotype similar to Jo-1 with both myopathy and ILD prominent. Limited data due to rarity.
OJILD-predominant
2–4% of cases
Interstitial lung diseaseFrequently severe
CommonInflammatory myopathyCan be severe
CommonMechanic's hands
FrequentRaynaud's phenomenon
OccasionalArthritis / arthralgia
Occasional Both ILD and myopathy can be severe. One of the rarer subtypes with limited prospective data.
KSILD-predominant
2–3% of cases
Interstitial lung diseasePredominant feature
CommonRaynaud's phenomenon
FrequentFever
FrequentInflammatory myopathyOften subclinical
OccasionalArthritis / arthralgia
OccasionalMechanic's hands
Occasional Strongly ILD-predominant with frequent Raynaud's and fever. Myopathy is often mild or absent entirely.
Ha / YRS
<1% of cases
Interstitial lung disease
CommonInflammatory myopathy
FrequentMechanic's hands
FrequentArthritis / arthralgia
FrequentRaynaud's phenomenon
Occasional Very rare. Clinical data are limited to case reports and small series. Phenotype appears to resemble Jo-1 ASyS.
Zo
<1% of cases
Interstitial lung disease
CommonInflammatory myopathy
FrequentMechanic's hands
OccasionalArthritis / arthralgia
Occasional Extremely rare. Fewer than 20 cases described in the literature. Clinical significance and full phenotypic spectrum remain under investigation.
Quick comparison
| Antibody | Cases | Myopathy | ILD | Arthritis | Mechanic's hands | Raynaud's |
|---|---|---|---|---|---|---|
| Jo-1 | 60–75% | |||||
| PL-7 | 5–10% | |||||
| PL-12 | 5–10% | |||||
| EJ | 3–5% | |||||
| OJ | 2–4% | |||||
| KS | 2–3% | |||||
| Ha / YRS | <1% | |||||
| Zo | <1% |
Prevalence estimates are approximations based on published literature. Individual patient presentations vary considerably.
Sources & further reading
- Connors GR et al. Interstitial lung disease associated with the idiopathic inflammatory myopathies. Eur Respir Rev. 2010.
- Lega JC et al. The clinical phenotype associated with myositis-specific and associated autoantibodies. Semin Arthritis Rheum. 2014.
- Hamaguchi Y et al. Clinical features of anti-aminoacyl-tRNA synthetase antibody-positive patients. Mod Rheumatol. 2013.
- Mahler M et al. Autoantibodies in antisynthetase syndrome. Autoimmun Rev. 2021.
- Gutsche M et al. Connective tissue disease-associated interstitial lung disease. Semin Respir Crit Care Med. 2012.