CTICasesPleuroparenchymal Fibroelastosis (PPFE)
Imaging findings
A patient presenting with pneumothorax shows diffuse reticular abnormality with architectural distortion in the upper lobes. There is peribronchovascular retraction with traction bronchiectasis and clumping of upper lobe bronchi. Nodularity on the septa and thickening along the pleura and subpleural parenchyma in the lung apices are noted, with findings becoming less prominent inferiorly.
Key takeaways
Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease characterized by fibroelastic thickening of the pleura and subpleural lung parenchyma, predominantly in the upper lobes. It often presents with apical scarring, architectural distortion, and traction bronchiectasis, and can be complicated by spontaneous pneumothorax. Pathologically, it has similarities to apical scarring seen in older patients, possibly representing a continuum of disease.
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