CTICasesPleuroparenchymal fibroelastosis
Imaging findings
Chest CT in a 28-year-old male with a history of pediatric Ewing sarcoma treated with cyclophosphamide shows striking upper lobe volume loss, apical pleural thickening, and subpleural reticulation, with surgical biopsy confirming pleuroparenchymal fibroelastosis.
Key takeaways
Pleuroparenchymal fibroelastosis is a rare form of interstitial lung disease characterized by pleural and subpleural parenchymal fibroelastosis, predominantly in the upper lobes. It can be idiopathic or secondary to prior chemotherapy or stem cell transplantation.
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