CTICasesPleuroparenchymal Fibroelastosis
Imaging findings
CT chest shows bilateral, dense, apical pleural thickening and subpleural consolidation with marked upper lobe volume loss and severe retraction of the hila superiorly, while the lung bases are relatively spared.
Key takeaways
Pleuroparenchymal fibroelastosis (PPFE) is a rare form of interstitial lung disease characterized by dominant fibroelastic thickening of the visceral pleura and subpleural parenchyma in the upper lobes. Clinically, it causes progressive dyspnea and cough, and can lead to lung transplantation in young adults.
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