Anti-synthetase syndrome

Last revised by Henry Knipe on 3 Aug 2023

Anti-synthetase syndrome is a systemic, inflammatory, autoimmune disease that is characterized by inflammatory myositis, polyarthritis associated with interstitial lung disease, and anti-synthetase autoantibodies.

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. 

The associated interstitial lung disease of anti-synthetase syndrome may usually give 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.

Medications used for anti-synthetase syndrome include corticosteroids, azathioprine, mycophenolate mofetil (MMF), calcineurin inhibitors (tacrolimus and cyclosporine), rituximab, cyclophosphamide and intravenous immunoglobulin (IVIG) 9.

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Cases and figures

  • Case 1: with anti-Jo1 elevation
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  • Case 1: with anti-Jo1 elevation
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  • Case 2
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  • Case 3: anti-PL-7 myofasciitis
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