CTICasesPleuroparenchymal Fibroelastosis
Imaging findings
Chest CT demonstrates prominent, bilateral, upper-lobe predominant pleural thickening and subpleural consolidation associated with traction bronchiectasis and architectural distortion. A left-sided pneumothorax is also present, highlighting the thickened visceral pleura.
Key takeaways
Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease characterized by elastic subpleural fibrosis and pleural thickening predominantly involving the upper lobes. It can be idiopathic or secondary to bone marrow transplant, chemotherapy, or recurrent infections, and is frequently complicated by pneumothorax.
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