Medical condition
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 in 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 .
^ Cite error: The named reference cohort of Spanish patients
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^ Cite error: The named reference Clinical Profiles
was invoked but never defined (see the help page ).
^ Cite error: The named reference Orphanet
was invoked but never defined (see the help page ).
^ Cite error: The named reference EuroMyositis
was invoked but never defined (see the help page ).