Antisynthetase syndrome
Antisynthetase syndrome (ASS) is a multisystematic autoimmune disease associated with inflammatory myositis, interstitial lung disease, and antibodies directed against various synthetases of aminoacyl-transfer RNA. Other common symptoms include mechanic's hands, Raynaud's phenomenon, arthritis, and fever.
| Anti-synthetase syndrome | |
|---|---|
| Other names | Anti-Jo1 syndrome, AS syndrome, ASS. |
| End-stage interstitial lung disease. | |
| Specialty | Immunology |
| Symptoms | Fever, myositis, polyarthritis, interstitial lung disease, mechanic's hands, and Raynaud phenomenon. |
| Differential diagnosis | Dermatomyositis, Immune-mediated necrotizing myopathy, and Polymyositis. |
| Treatment | Glucocorticosteroids, immunosuppressive medications. |
| Frequency | 1/25,000 - 33,000 worldwide. |
It is still unknown what causes interstitial lung disease associated with antisynthetase syndrome. Many antisynthetase antibodies have been reported with anti-Jo1 being the most prevalent. Pulmonary involvement is an important factor of morbidity and mortality with Antisynthetase syndrome, affecting 70-100% of patients.
Antisynthetase syndrome is diagnosed by a combination of radiologic features, clinical criteria, and identification of aminoacyl tRNA synthetase antibodies. Immunosuppressive medications such as mycophenolate mofetil, azathioprine, and tacrolimus are often used alongside corticosteroids to manage myositis and other pulmonary symptoms.
It is believed that the mortality rate for antisynthetase syndrome is significantly higher than that of the general population. The estimated cumulative ten-year survival rate for patients with different antisyntetase antibodies is 76.8%.
Antisynthetase syndrome is estimated by Orphanet to affect 1-3 people per 100,000 worldwide; however, precise data on the disease's prevalence is not available. Antisynthetase syndrome is more common in women.