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Antisynthetase (AS) syndrome is a clinically heterogeneous form of idiopathic inflammatory myopathy characterized by myositis, arthralgia, Raynaud phenomenon, mechanic hands, interstitial lung disease (ILD), and serum autoantibodies to aminoacyl transfer RNA synthetases (anti-ARS).
Features include very common findings: Muscle weakness, Difficulty breathing (respiratory insufficiency), Lung scarring (pulmonary fibrosis), and Autoimmunity and others; and common findings: Xerostomia, Edema, Keratoconjunctivitis sicca, and Low muscle tone (hypotonia) and others. 44 total HPO annotations.
Organ System | Phenotype Count | Example Features |
|---|---|---|
Lab test results | 12 | Elevated creatine kinase (muscle enzyme) (elevated circulating creatine kinase concentration), Abnormal electrical muscle activity (EMG) (emg abnormality), Anti-threonyl-tRNA synthetase antibody positivity |
Biomarker and diagnostic research for antisynthetase syndrome has been reported in the published literature.
Phenotype severity distribution: 9 very common features, 22 common features.
Estimated prevalence: 1-9 in 100,000 (Uncommon).
7 clinical trials registered, 2 recruiting. Interventions under study include drug therapy, biologic therapy, and other interventions. Pipeline includes 1 PHASE3, 3 PHASE2, 1 EARLY_PHASE1. Research is sponsored by a mix of industry and academic institutions.
NCT ID | Title | Phase | Sponsor | Status |
|---|---|---|---|---|
[NCT07406932](https://clinicaltrials.gov/study/NCT07406932) |
Data assembled from 5 of 12 sources · Last updated Sep 18, 2026, 8:08 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Lungs and breathing | 6 | Difficulty breathing (respiratory insufficiency), Lung scarring (pulmonary fibrosis), Abnormal lung tissue (abnormal pulmonary interstitial morphology) |
Skin | 4 | Lack of skin elasticity, Skin rash, Pruritus |
Muscles | 3 | Muscle weakness, Myalgia, Low muscle tone (hypotonia) |
Heart and blood vessels | 3 | Chest pain, Aortic regurgitation, High blood pressure in lung arteries (pulmonary arterial hypertension) |
Bones and joints | 3 | Joint inflammation (arthritis), Arthralgia, Joint dislocation |
Blood and immune system | 2 | Autoimmunity, Recurrent respiratory infections |
Arms and legs | 2 | Mechanic's hands, Hiker's feet |
Metabolism | 1 | Fever |
Voice | 1 | Abnormality of the voice |
Digestive system | 1 | Difficulty swallowing (dysphagia) |
Brain and nerves | 1 | Difficulty swallowing (dysphagia) |
Neoplasm | 1 | Neoplasm |
A Study on the Efficacy and Safety of JAK Inhibitors Versus Calcineurin Inhibitors as Initial Therapy for Interstitial Lung Disease Associated With Antisynthetase Syndrome |
NA |
China-Japan Friendship Hospital |
RECRUITING |
[NCT05984394](https://clinicaltrials.gov/study/NCT05984394) | Evaluation of Antigen-specific T Cells in Patients With Antisynthetase Syndrome and Interstitial Lung Disease | — | Central Hospital, Nancy, France | UNKNOWN |
[NCT06613490](https://clinicaltrials.gov/study/NCT06613490) | An Exploratory Clinical Study of CD19 CAR NK Cells for the Treatment of Refractory Antisynthetase Antibody Syndrome and Rheumatoid Arthritis | EARLY_PHASE1 | The First Affiliated Hospital with Nanjing Medical University | RECRUITING |
[NCT07391605](https://clinicaltrials.gov/study/NCT07391605) | Descartes-08 in Autoantibody Myositis | PHASE2 | Cartesian Therapeutics | UNKNOWN |
[NCT05832034](https://clinicaltrials.gov/study/NCT05832034) | Add-on Intravenous Immunoglobulins in Early Myositis | PHASE2 | Academisch Medisch Centrum - Universiteit van Amsterdam (AMC-UvA) | ACTIVE_NOT_RECRUITING |
202 publications have been identified in PubMed for antisynthetase syndrome. Kisho has analyzed 137 by research type. Research spans Review / Meta-Analysis (27%), Epidemiology / Natural History (25%), and Case Report / Case Series (15%).
Research Type | Count | % of Total |
|---|---|---|
Research summaries | 37 | 27% |
Disease patterns and progression | 34 | 25% |
Patient case studies | 21 | 15% |
Testing and diagnosis research | 20 | 15% |
Laboratory research | 15 | 11% |
Other research | 6 | 4% |
Clinical study results | 3 | 2% |
New treatment approaches | 1 | 1% |
Pinal-Fernandez I (2026). [PMID: 40335356](https://pubmed.ncbi.nlm.nih.gov/40335356/). *Ann Rheum Dis*. [Other]
Murphy A (2026). [PMID: 41364196](https://pubmed.ncbi.nlm.nih.gov/41364196/). *J Neuropathol Exp Neurol*. [Basic Science / Preclinical]
Sriranganathan A (2026). [PMID: 41642723](https://pubmed.ncbi.nlm.nih.gov/41642723/). *Ocul Immunol Inflamm*. [Case Report / Case Series]
Billotte M (2026). [PMID: 41376133](https://pubmed.ncbi.nlm.nih.gov/41376133/). *J Intern Med*. [Epidemiology / Natural History]
Düsing C (2026). [PMID: 41714747](https://pubmed.ncbi.nlm.nih.gov/41714747/). *Nat Med*. [Gene Therapy / Novel Therapeutics]
Wilfong EM (2026). [PMID: 42023216](https://pubmed.ncbi.nlm.nih.gov/42023216/). *Front Immunol*. [Basic Science / Preclinical]
Clark J (2026). [PMID: 41610110](https://pubmed.ncbi.nlm.nih.gov/41610110/). *ACR Open Rheumatol*. [Epidemiology / Natural History]
Leemans E (2026). [PMID: 41761500](https://pubmed.ncbi.nlm.nih.gov/41761500/). *Rheumatology (Oxford)*. [Review / Meta-Analysis]
Pais F (2026). [PMID: 41898191](https://pubmed.ncbi.nlm.nih.gov/41898191/). *Biomedicines*. [Epidemiology / Natural History]
Pillot V (2026). [PMID: 41825679](https://pubmed.ncbi.nlm.nih.gov/41825679/). *Autoimmun Rev*. [Review / Meta-Analysis]
AI-curated news mentioning antisynthetase syndrome
Updated Feb 24, 2026
A recent study highlights the diagnostic challenges of antisynthetase syndrome, which can mimic pulmonary infections. This research underscores the need for heightened awareness among healthcare providers to improve diagnosis and treatment.