CTICasesPleuroparenchymal Fibroelastosis in Graft-Versus-Host Disease
Imaging findings
High-resolution chest CT demonstrates progressive, peripheral, upper-lobe predominant pleural and subpleural consolidation with associated volume loss and architectural distortion. High-resolution expiratory scans demonstrate prominent mosaic perfusion and air trapping, reflecting co-existing constrictive bronchiolitis in a patient with history of leukemia and allogeneic stem cell transplant.
Key takeaways
Pleuroparenchymal fibroelastosis (PPFE) is a rare form of interstitial lung disease characterized by pleural and subpleural alveolar fibrosis. It is increasingly recognized as a late pulmonary complication of graft-versus-host disease (GVHD) following allogeneic hematopoietic stem cell transplantation. It often co-exists with constrictive bronchiolitis, representing a combined obstructive and restrictive injury process.
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