Antisynthetase syndrome
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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.[3] Other common symptoms include mechanic's hands, Raynaud's phenomenon, arthritis, and fever.[4]
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.[1] |
Differential diagnosis | Dermatomyositis, Immune-mediated necrotizing myopathy, and Polymyositis.[2] |
Treatment | Glucocorticosteroids, immunosuppressive medications.[1] |
Frequency | 1/25,000 - 33,000 worldwide.[2] |
It is still unknown what causes interstitial lung disease associated with antisynthetase syndrome.[5] Many antisynthetase antibodies have been reported with anti-Jo1 being the most prevalent.[6] Pulmonary involvement is an important factor of morbidity and mortality with Antisynthetase syndrome, affecting 70-100% of patients.[7]
Antisynthetase syndrome is diagnosed by a combination of radiologic features, clinical criteria, and identification of aminoacyl tRNA synthetase antibodies.[8] Immunosuppressive medications such as mycophenolate mofetil, azathioprine, and tacrolimus are often used alongside corticosteroids to manage myositis and other pulmonary symptoms.[9]
It is believed that the mortality rate for antisynthetase syndrome is significantly higher than that of the general population.[10] The estimated cumulative ten-year survival rate for patients with different antisyntetase antibodies is 76.8%.[11]
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.[12] Antisynthetase syndrome is more common in women.[13]