title: 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.
authors: Department of Pneumology, Centro Hospitalar de São João, Portugal. Electronic address: margarida.tredondo@gmail.com.
authors: M T
authors: MT
authors: Redondo
authors: Department of Pneumology, Centro Hospitalar de São João, Portugal.
authors: N
authors: N
authors: Melo
authors: Department of Pneumology, Centro Hospitalar de São João, Portugal; Faculty of Medicine of University of Porto, Portugal.
authors: P C
authors: PC
authors: Mota
authors: Department of Radiology, Centro Hospitalar de São João, Portugal.
authors: J M
authors: JM
authors: Jesus
authors: Faculty of Medicine of University of Porto, Portugal; Department of Pathology, Centro Hospitalar de São João, Portugal.
authors: C S
authors: CS
authors: Moura
authors: Department of Pathology, Centro Hospitalar de São João, Portugal.
authors: S
authors: S
authors: Guimarães
authors: Department of Pneumology, Centro Hospitalar de São João, Portugal; Faculty of Medicine of University of Porto, Portugal.
authors: A
authors: A
authors: Morais
mesh_terms: D000368:Aged; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000150:complications / Q000473:pathology; D008875:Middle Aged; D010995:Pleural Diseases / Q000150:complications / Q000473:pathology; D035583:Rare Diseases / Q000150:complications / Q000473:pathology
keywords: Fibroelastosis; Interstitial lung disease; Pleuroparenchymal fibrosis