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Pleuroparenchymal Fibroelastosis (PPFE)

Imaging findings

Progressive apical fibrosis, traction bronchiectasis, and severe architectural distortion.

Key takeaways

Current clinical practice allows for the use of antifibrotic therapy in cases of progressive pulmonary fibrosis, regardless of whether it is strictly classified as Idiopathic Pulmonary Fibrosis (IPF) or PPFE.

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