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Pleuroparenchymal Fibroelastosis (PPFE) — An Update

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Abstract

Purpose of Review

This review highlights the clinical, radiological and histological features of pleuroparenchymal fibroelastosis (PPFE) as well as the typical disease course and management.

Recent Findings

PPFE has been associated with a number of conditions including post-transplantation, respiratory infection and connective tissue disease. The clinical course of PPFE is varied although the overall prognosis is poor. Pneumothorax occurs frequently but can often be managed conservatively. Lung transplant outcomes comparable with those in idiopathic pulmonary fibrosis (IPF) have been reported although long-term outcome data is limited. The efficacy of nintedanib in slowing PPFE progression remains unclear.

Summary

PPFE is a rare disease characterised by progressive breathlessness due to upper lobe pleural thickening and subpleural lung fibrosis. Although awareness of PPFE has increased over the last decade, the underlying pathogenesis remains unknown and the condition carries a poor prognosis with little in the way of established treatment options.

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  1. Respiratory Department, University Hospital Llandough, Penlan Road, Cardiff, CF64 2XX, Wales

    Philip Evans

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Evans, P. Pleuroparenchymal Fibroelastosis (PPFE) — An Update. Curr Pulmonol Rep 12, 171–179 (2023). https://doi.org/10.1007/s13665-023-00320-1

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