CTICasesPleuroparenchymal Fibroelastosis (PPFE)
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Imaging findings
Chest CT demonstrates sparse subpleural reticulation with a diffuse distribution that lacks a basilar predominance, without clear findings of advanced fibrosis such as honeycombing, traction bronchiectasis, or bronchiolectasis.
Key takeaways
Pleuroparenchymal fibroelastosis (PPFE) is a rare form of interstitial lung disease characterized by pleural and subpleural parenchymal fibroelastosis. Definitive diagnosis often requires a surgical lung biopsy evaluated with special elastic stains to confirm elastosis, helping to differentiate it from idiopathic pulmonary fibrosis (IPF).
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