title: High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis.
abstract: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described clinical-pathologic entity characterized by pleural and subpleural parenchymal fibrosis, mainly in the upper lobes. As this disease is extremely rare (only 7 cases have been described in the literature to date) poorly defined cases of IPPFE can go unrecognized.The clinical course of disease is progressive and prognosis is poor, with no therapeutic options other than lung transplantation currently available, yet. The aim of this report is to describe two further cases of this rare disease, reviewing CT, clinical and histological features.
authors.affiliation: Department of Radiology, Scientific Institute for study and treatment of Cancer-IRST; Via Piero Maroncelli 40-42; 47014; Meldola-Forlì; Italy. s.piciucchi@alice.it
authors.forename: Sara
authors.initials: S
authors.lastname: Piciucchi
authors.forename: Sara
authors.initials: S
authors.lastname: Tomassetti
authors.forename: Gianluca
authors.initials: G
authors.lastname: Casoni
authors.forename: Nicola
authors.initials: N
authors.lastname: Sverzellati
authors.forename: Angelo
authors.initials: A
authors.lastname: Carloni
authors.forename: Alessandra
authors.initials: A
authors.lastname: Dubini
authors.forename: Giampaolo
authors.initials: G
authors.lastname: Gavelli
authors.forename: Alberto
authors.initials: A
authors.lastname: Cavazza
authors.forename: Marco
authors.initials: M
authors.lastname: Chilosi
authors.forename: Venerino
authors.initials: V
authors.lastname: Poletti
mesh_terms: D000328:Adult; D000368:Aged; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D010995:Pleural Diseases / Q000000981:diagnostic imaging* / Q000473:pathology; D011658:Pulmonary Fibrosis / Q000000981:diagnostic imaging* / Q000473:pathology; D014057:Tomography, X-Ray Computed