The case of idiopathic pleuroparenchymal fibroelastosis diagnosed by transbronchial biopsy

Abstract

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