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Pleuroparenchymal Fibroelastosis

Imaging findings

CT chest shows upper-lobe predominant pleural thickening and subpleural consolidation, associated with moderate volume loss. The patient is a young female geologist.

Key takeaways

Pleuroparenchymal fibroelastosis (PPFE) is a rare form of interstitial lung disease characterized by elastic tissue deposition in the visceral pleura and subpleural alveolar walls. It typically locates in the upper lobes, causing progressive volume loss, and can be idiopathic or secondary to exposures.

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