CTICasesFibrotic Nonspecific Interstitial Pneumonia (NSIP) in Anti-Synthetase Syndrome
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Imaging findings
Initial CT (2012) shows modest, basal-predominant subpleural ground-glass opacity with relative sparing of the subpleural lung regions. Over time (2013-onward), there is progression of parenchymal lung disease, particularly in the lower lung zones, with bronchi starting to dilate, consistent with fibrotic NSIP. Pathology confirmed fibrosis with a nonspecific interstitial pneumonia pattern.
Key takeaways
Fibrotic NSIP is a common manifestation of connective tissue diseases, such as anti-synthetase syndrome (e.g., anti-Jo-1 antibody, dermatomyositis, dermatomyositis). Imaging features include basal-predominant ground-glass opacities, which can progress to fibrosis and bronchiectasis. Severe pulmonary hypertension can be a complication, contributing to patient mortality.
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