CTICasesPleuroparenchymal Fibroelastosis
Imaging findings
A patient in their 70s referred with a diagnosis of NSIP (without biopsy or known connective tissue disease) has an upper-zone-predominant process with marked upper lobe volume loss, peripheral fibrosis with traction bronchiectasis, apical pleural and subpleural thickening with wedge-shaped dense fibrotic consolidation, and distorted, somewhat corrugated central airways. The trachea measures about 28 mm without frank tracheobronchomegaly, and the apical thickening was misinterpreted elsewhere as pleural plaques.
Key takeaways
Marked upper-zone volume loss with apical wedge-shaped dense fibrosis and traction bronchiectasis suggests pleuroparenchymal fibroelastosis, distinguished from the trivial upper lobe apical scarring common in the elderly by the amount of traction bronchiectasis and volume loss. The apical pleural thickening represents pseudoplaques, not asbestos plaques (which spare the apices), and adjacent fibrotic traction on the airway can produce a corrugated tracheal appearance (traction tracheopathy) without true tracheobronchomegaly; biopsy is not needed as it would not change management.
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