Pleuroparenchymal Fibroelastosis: A Rare Entity

This is the authors' abstract. We don't add key points for this paper.

Pleuroparenchymal fibroelastosis (PPFE) is a rare variant of interstitial lung disease (ILD). PPFE is characterizsed by peculiar clinical, radiological and histological features. Initially considered an idiopathic entity, this disease is now classified in the rare category of the recent classification of idiopathic interstitial pneumonias (IIPs). However, it largely continues to be underdiagnosed owing to the lack of awareness about this condition. PPFE may be misdiagnosed as the other commonly diagnosed ILDs, especially IIP. The diagnosis is clinched with lung biopsy. We report a case of PPFE diagnosed by transbronchial lung biopsy.