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Anti-Synthetase Syndrome

Imaging findings

CT of the chest shows extensive, bilateral, subpleural, and basal-predominant ground-glass opacities mixed with patchy consolidations, highly suggestive of cellular non-specific interstitial pneumonia (NSIP) combined with organizing pneumonia.

Key takeaways

Anti-synthetase syndrome is a distinct clinical entity within the idiopathic inflammatory myopathies, characterized by myositis, interstitial lung disease, joint involvement, skin changes (mechanic's hands), and specific anti-aminoacyl-tRNA synthetase antibodies (such as anti-Jo-1). The interstitial lung disease often manifests as a mixed NSIP and organizing pneumonia pattern.

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