CTICasesAnti-Synthetase Syndrome (EJ Antibody Subtype)
Imaging findings
Chest radiograph demonstrates multiple poorly marginated, non-segmental, patchy opacities in bilateral lungs with mildly diminished lung volumes. High-resolution CT demonstrates bilateral, peripheral, and lower-lobe predominant ground-glass and consolidative opacities with subtle subpleural sparing, consistent with a cellular NSIP and organizing pneumonia pattern. Follow-up imaging shows significant clearing of the opacities after corticosteroid therapy.
Key takeaways
Anti-synthetase syndrome is a systemic autoimmune disease characterized by interstitial lung disease, myositis, Raynaud's phenomenon, and arthritis. It is defined by the presence of anti-aminoacyl-tRNA synthetase antibodies; while anti-Jo-1 is the most common, other mutually exclusive antibodies such as anti-EJ, anti-PL-7, or anti-PL-12 can be positive. Lung involvement typically presents with a mixed NSIP and organizing pneumonia pattern that is highly steroid-responsive.
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