Anti-synthetase syndrome
Updates to Article Attributes
Anti-synthetase syndrome is a systemic, inflammatory, autoimmune disease that is characterised by inflammatory myositis, polyarthritis associated with interstitial lung disease, and anti-synthetase autoantibodies.
Pathology
Anti-synthetase syndrome can result from autoantibodies to eight of the aminoacyl-transfer RNA synthetases. These 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)
Radiographic features
Imaging findings vary depending on the organ involved.
CT
The associated interstitial lung disease of anti-synthetase syndrome may usually givegives either a NSIP pattern (, an organising pneumonia (OP) pattern pattern or a combination of the two (NSIP-OP pattern) 6 6. A UIP pattern may also occasionally occur 8. The 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 ciclosporin), rituximab, cyclophosphamide and intravenous immunoglobulin (IVIG) 9.
-<p><strong>Anti-synthetase syndrome </strong>is a systemic, inflammatory, autoimmune disease that is characterised by <a href="/articles/inflammatory-myositis">inflammatory myositis</a>, <a href="/articles/polyarthritis">polyarthritis</a> associated with <a href="/articles/interstitial-lung-disease">interstitial lung disease</a>, and anti-synthetase autoantibodies.</p><h4>Pathology</h4><p>Anti-synthetase syndrome can result from autoantibodies to eight of the aminoacyl-transfer RNA synthetases. These include:</p><ul>- +<p><strong>Anti-synthetase syndrome </strong>is a systemic, inflammatory, autoimmune disease that is characterised by <a href="/articles/inflammatory-myositis">inflammatory myositis</a>, <a href="/articles/polyarthritis">polyarthritis</a> associated with <a href="/articles/interstitial-lung-disease">interstitial lung disease</a>, and anti-synthetase autoantibodies.</p><h4>Pathology</h4><p>Anti-synthetase syndrome can result from autoantibodies to eight of the aminoacyl-transfer RNA synthetases. These include:</p><ul>
-</ul><h4>Radiographic features</h4><p>Imaging findings vary depending on the organ involved. </p><h5>CT</h5><p>The associated interstitial lung disease of anti-synthetase syndrome may usually give either a <a href="/articles/non-specific-interstitial-pneumonia-1">NSIP</a> pattern, an <a href="/articles/organising-pneumonia">organising pneumonia (OP)</a> pattern or a combination of the two (NSIP-OP pattern)<sup> 6</sup>. A <a href="/articles/usual-interstitial-pneumonia">UIP</a> pattern may also occasionally occur <sup>8</sup>. 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.</p><h4>Treatment and prognosis</h4><p>Medications used for anti-synthetase syndrome include corticosteroids, azathioprine, mycophenolate mofetil (MMF), calcineurin inhibitors (tacrolimus and ciclosporin), rituximab, cyclophosphamide and intravenous immunoglobulin (IVIG) <sup>9</sup>.</p>- +</ul><h4>Radiographic features</h4><p>Imaging findings vary depending on the organ involved. </p><h5>CT</h5><p>The associated interstitial lung disease of anti-synthetase syndrome usually gives either a <a href="/articles/non-specific-interstitial-pneumonia-1">NSIP</a> pattern (, an <a href="/articles/organising-pneumonia">organising pneumonia (OP)</a> pattern or a combination of the two (NSIP-OP pattern)<sup> 6</sup>. A <a href="/articles/usual-interstitial-pneumonia">UIP</a> pattern may also occasionally occur <sup>8</sup>. 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.</p><h4>Treatment and prognosis</h4><p>Medications used for anti-synthetase syndrome include corticosteroids, azathioprine, mycophenolate mofetil (MMF), calcineurin inhibitors (tacrolimus and ciclosporin), rituximab, cyclophosphamide and intravenous immunoglobulin (IVIG) <sup>9</sup>.</p>
References changed:
- 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. <a href="https://doi.org/10.1016/j.ejrad.2016.05.012">doi:10.1016/j.ejrad.2016.05.012</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/27423682">Pubmed</a>
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