CTICasesProgressive NSIP-Pattern Fibrosis Suspecting Connective Tissue Disease
Imaging findings
A woman in her 70s with dyspnea has basal-predominant, ground-glass-predominant lung disease with dilated airways, traction bronchiectasis, subtle reticulation, and relative subpleural sparing, plus esophageal debris. Over three years it progressed with coarsening, greater volume loss, a displaced right oblique fissure, and increased, almost out-of-proportion central traction bronchiectasis, with preserved subpleural sparing.
Key takeaways
Basal-predominant ground glass with traction bronchiectasis and relative subpleural sparing is a classic NSIP pattern that, particularly with esophageal dilatation/debris, should prompt evaluation for a connective tissue disease such as scleroderma. Identifying an underlying CTD matters because treating it can stabilize the lung disease, and antifibrotic therapy (nintedanib) is approved for scleroderma-associated and other progressive fibrotic lung diseases.
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