Idiopathic pleuroparenchymal fibroelastosis: A rare but increasingly recognized entity

Abstract

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described rare entity, characterized by pleural and subpleural parenchymal fibrosis and elastosis mainly in the upper lobes. The etiology and pathophysiology are unknown. The prognosis is poor, with no effective therapies other than lung transplantation. IPPFE should be properly identified so that it can be approached correctly. This report describes two clinical cases with clinical imaging and histological features compatible with IPPFE. Keywords: Interstitial lung disease, Pleuroparenchymal fibrosis, Fibroelastosis