Citation, DOI, disclosures and article data
Citation:
Weerakkody Y, Sharma R, Knipe H, et al. Anti-synthetase syndrome. Reference article, Radiopaedia.org (Accessed on 17 Jan 2025) https://doi.org/10.53347/rID-17810
Anti-synthetase syndrome is an idiopathic inflammatory myopathy that is characterized by inflammatory myositis, polyarthritis, interstitial lung disease, and anti-synthetase autoantibodies. It is considered a distinct entity to dermatomyositis.
Anti-synthetase syndrome can result from autoantibodies to eight of the aminoacyl-transfer RNA synthetases. These include:
anti-JO-1: most common anti-synthetase autoantibody
anti-PL12
anti-PL7 / anti-threonyl-tRNA synthetase
anti-OJ
anti-EJ
anti-KS
anti-ZO
anti-YRS
anti-Ro52 / anti-Ro52-positive antisynthetase syndrome (ASS)
Imaging findings vary depending on the organ involved.
CT
The associated interstitial lung disease of anti-synthetase syndrome usually gives either a NSIP pattern, an organizing pneumonia (OP) pattern or a combination of the two (NSIP-OP pattern) 6. A UIP pattern may also occasionally occur 8. The consolidative regions may decrease or resolve in many cases although the disease can at times progress to fibrosis in more than one third of patients.
Treatment and prognosis
Medications used for anti-synthetase syndrome include corticosteroids, azathioprine, mycophenolate mofetil (MMF), calcineurin inhibitors (tacrolimus and cyclosporine), rituximab, cyclophosphamide and intravenous immunoglobulin (IV Ig) 9.
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1. Hervier B, Wallaert B, Hachulla E et al. Clinical Manifestations of Anti-Synthetase Syndrome Positive for Anti-Alanyl-TRNA Synthetase (Anti-PL12) Antibodies: A Retrospective Study of 17 Cases. Rheumatology (Oxford). 2010;49(5):972-6. doi:10.1093/rheumatology/kep455 - Pubmed
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2. Patel H & Lauder N. The Antisynthetase Syndrome. Am J Med. 2011;124(9):e3-4. doi:10.1016/j.amjmed.2011.02.010 - Pubmed
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3. Mumm G, McKown K, Bell C. Antisynthetase Syndrome Presenting as Rheumatoid-Like Polyarthritis. J Clin Rheumatol. 2010;16(7):307-12. doi:10.1097/RHU.0b013e3181f3bf9c - Pubmed
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4. Plastiras S, Soliotis F, Vlachoyiannopoulos P, Tzelepis G. Interstitial Lung Disease in a Patient with Antisynthetase Syndrome and No Myositis. Clin Rheumatol. 2007;26(1):108-11. doi:10.1007/s10067-005-0111-3 - Pubmed
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5. Katzap E, Barilla-LaBarca M, Marder G. Antisynthetase Syndrome. Curr Rheumatol Rep. 2011;13(3):175-81. doi:10.1007/s11926-011-0176-8 - Pubmed
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6. Debray M, Borie R, Revel M et al. Interstitial Lung Disease in Anti-Synthetase Syndrome: Initial and Follow-Up CT Findings. Eur J Radiol. 2015;84(3):516-23. doi:10.1016/j.ejrad.2014.11.026 - Pubmed
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7. Hervier B, Uzunhan Y, Hachulla E et al. Antisynthetase Syndrome Positive for Anti-Threonyl-TRNA Synthetase (Anti-PL7) Antibodies. Eur Respir J. 2011;37(3):714-7. doi:10.1183/09031936.00104310 - Pubmed
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8. Marie I, Josse S, Decaux O et al. Clinical Manifestations and Outcome of Anti-PL7 Positive Patients with Antisynthetase Syndrome. Eur J Intern Med. 2013;24(5):474-9. doi:10.1016/j.ejim.2013.01.002 - Pubmed
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9. Maturu V, Lakshman A, Bal A et al. Antisynthetase Syndrome: An Under-Recognized Cause of Interstitial Lung Disease. Lung India. 2016;33(1):20-6. doi:10.4103/0970-2113.173055 - Pubmed
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10. Zamora A, Hoskote S, Abascal-Bolado B et al. Clinical Features and Outcomes of Interstitial Lung Disease in Anti-Jo-1 Positive Antisynthetase Syndrome. Respir Med. 2016;118:39-45. doi:10.1016/j.rmed.2016.07.009 - Pubmed
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11. Waseda Y, Johkoh T, Egashira R et al. Antisynthetase Syndrome: Pulmonary Computed Tomography Findings of Adult Patients with Antibodies to Aminoacyl-TRNA Synthetases. Eur J Radiol. 2016;85(8):1421-6. doi:10.1016/j.ejrad.2016.05.012 - Pubmed
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