Global InformationAntisynthetase syndrome information
| 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] |
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]
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]
- ^ a b Sobarun P. "Antisynthetase syndrome". DermNet. Retrieved December 1, 2023.
- ^ a b Cai C (May 26, 2021). "Antisynthetase syndrome associated myositis". Pathology Outlines. Retrieved December 1, 2023.
- ^ Cavagna L, Nuño L, Scirè CA, Govoni M, Longo FJ, Franceschini F, et al. (February 2017). "Serum Jo-1 Autoantibody and Isolated Arthritis in the Antisynthetase Syndrome: Review of the Literature and Report of the Experience of AENEAS Collaborative Group". Clinical Reviews in Allergy & Immunology. 52 (1). Springer Science and Business Media LLC: 71–80. doi:10.1007/s12016-016-8528-9. PMID 26782036. S2CID 34446814. Retrieved December 4, 2023.
- ^ Sreevilasan SK, Devarasetti P, Narahari NK, Desai A, Rajasekhar L (November 2021). "Clinical profile and treatment outcomes in antisynthetase syndrome: a tertiary centre experience". Rheumatology Advances in Practice. 5 (Suppl 2). Oxford University Press (OUP): ii10–ii18. doi:10.1093/rap/rkab054. PMC 8570161. PMID 34755025.
- ^ Zhao, Na; Jiang, Wei; Wu, Hongliang; Wang, Ping; Wang, Xiaoni; Bai, Yu; Li, Yao; Tang, Yanchun; Liu, Ying (August 10, 2022). "Clinical features, prognostic factors, and survival of patients with antisynthetase syndrome and interstitial lung disease". Frontiers in Immunology. 13. Frontiers Media SA. doi:10.3389/fimmu.2022.872615. ISSN 1664-3224. PMC 9399497. PMID 36032132.
- ^ Marguerie C, Bunn CC, Beynon HL, Bernstein RM, Hughes JM, So AK, Walport MJ (October 1990). "Polymyositis, pulmonary fibrosis and autoantibodies to aminoacyl-tRNA synthetase enzymes". The Quarterly Journal of Medicine. 77 (282). Oxford University Press (OUP): 1019–1038. doi:10.1093/qjmed/77.1.1019. PMID 2267280. Retrieved December 4, 2023.
- ^ Stanciu R, Guiguet M, Musset L, Touitou D, Beigelman C, Rigolet A, et al. (September 2012). "Antisynthetase syndrome with anti-Jo1 antibodies in 48 patients: pulmonary involvement predicts disease-modifying antirheumatic drug use". The Journal of Rheumatology. 39 (9): 1835–1839. doi:10.3899/jrheum.111604. PMID 22859355.
- ^ Wells M, Alawi S, Thin KY, Gunawardena H, Brown AR, Edey A, et al. (September 14, 2022). "A multidisciplinary approach to the diagnosis of antisynthetase syndrome". Frontiers in Medicine. 9. Frontiers Media SA: 959653. doi:10.3389/fmed.2022.959653. PMC 9515890. PMID 36186825.
- ^ Witt LJ, Curran JJ, Strek ME (September 2016). "The Diagnosis and Treatment of Antisynthetase Syndrome". Clinical Pulmonary Medicine. 23 (5). Ovid Technologies (Wolters Kluwer Health): 218–226. doi:10.1097/cpm.0000000000000171. PMC 5006392. PMID 27594777.
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