CTICasesPleuroparenchymal Fibroelastosis
Imaging findings
Chest radiography and CT in a patient status post-single lung transplant show severe volume loss, pleural thickening, and a dense rind of subpleural consolidation with traction bronchiectasis predominantly involving the upper lobes of the native right lung. Histopathology using a Movat pentachrome stain reveals prominent intra-alveolar fibrosis and dense elastic fibers, confirming the diagnosis.
Key takeaways
Pleuroparenchymal fibroelastosis (PPFE) is a rare form of interstitial lung disease characterized by pleural and subpleural parenchymal fibrosis with elastic fiber deposition, typically showing a marked upper-lobe predominance. It can present as idiopathic disease, but is also associated with bone marrow or lung transplantation, chemotherapy, and autoimmune conditions.
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