The case of idiopathic pleuroparenchymal fibroelastosis diagnosed by transbronchial biopsy

Abstract

Pleuroparenchymal fibroelastosis (PPFE) is claasified in idiopathic interstitial pneumonitis group israrely seen. Etiology and pathophysiology of this entity are not completely understood. The prognosisis poor, and there is no effective treatment except for lung transplantation. Here, we presented a caseof PPFE diagnosed by transbronchial biopsy through fiberoptic bronchoscopy and its histopathologicaland clinicoradiological features.