title: Idiopathic pleuroparenchymatous fibroelastosis: A case report and brief review of the literature.
abstract: We describe a patient with idiopathic pleuroparenchymal fibroelastosis (IPPFE). This rare clinicopathological syndrome is characterized by typical apical alterations op chest imaging, such as pleural thickening and subpleural fibrosis. Thickened visceral pleura and subpleural fibrosis consisting of dense collagen and elastin, are the main histopathological features. Etiology is unknown but a link between recurrent infections (in particular aspergillosis) and autoimmune diseases is suspected. At this time there is no standardized treatment regimen and the prognosis is variable.
authors.affiliation: Respiratory Medicine Department, University Hospitals Leuven, Belgium.
authors.forename: Kristof
authors.initials: K
authors.lastname: Cuppens
authors.affiliation: Pathology Department, University Hospitals Leuven, Belgium.
authors.forename: Eric
authors.initials: E
authors.lastname: Verbeken
authors.affiliation: Radiology Department, University Hospitals Leuven, Belgium.
authors.forename: Johan
authors.initials: J
authors.lastname: Coolen
authors.affiliation: Radiology Department, University Hospitals Leuven, Belgium.
authors.forename: Johny
authors.initials: J
authors.lastname: Verschakelen
authors.affiliation: Respiratory Medicine Department, University Hospitals Leuven, Belgium.
authors.forename: Wim
authors.initials: W
authors.lastname: Wuyts
keywords: Case report; Interstitial lung disease; Pleuroparenchymal fibroelastosis