CTICasesPleuroparenchymal Fibroelastosis
Imaging findings
Serial radiographs show progressive volume loss and reticulonodular opacities. High-resolution CT shows upper-lobe predominant, subpleural reticular thickening and pleural thickening that reach the pleural surface, characteristic of pleural-parenchymal fibroelastosis.
Key takeaways
Pleuroparenchymal fibroelastosis (PPFE) is a rare form of interstitial lung disease characterized by upper-lobe-predominant pleural and subpleural parenchymal fibrosis. While it can occur after lung transplantation or bone marrow transplantation, it can also present sporadically.
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