CTICasesScleroderma-Associated UIP with Exuberant Honeycombing
Imaging findings
Serial CT scans over 13 years show progressive basilar-predominant fibrotic lung disease that evolved into extensive cystic spaces resembling exuberant honeycombing. A straight edge sign demarcating the disease from normal lung is seen on coronal images.
Key takeaways
Scleroderma-associated ILD can slowly progress to an exuberant honeycombing pattern with a straight edge sign. Although the imaging appearance is highly suggestive of a connective tissue etiology, surgical lung biopsy typically confirms usual interstitial pneumonia (UIP) pathology.
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