High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: Features and differential diagnosis
1 Department of Radiology, Scientific Institute for study and treatment of Cancer-IRST; Via Piero Maroncelli 40-42; 47014; Meldola-Forlì; Italy
2 Department of Pulmonology; Morgagni-Pierantoni Hospital; Via Carlo Forlanini 34, 47121; Forlì; Italy
3 Department of Radiology; University of Parma; Via Gramsci 14; 43100; Parma, Italy
4 Department of Radiology; Santa Maria Hospital; Via Tristano di Joannuccio 1, 05100; Terni, Italy
5 Department of Pathology; Morgagni-Pierantoni Hospital; Via Carlo Forlanini 34, 47121; Forlì; Italy
6 Department of Pathology; Arcispedale Santa Maria Nuova; Viale Risorgimento 57; 42100; Reggio Emilia; Italy
7 Department of Pathology; University of Verona; Piazzale A. Stefani 1; 37126; Verona; Italy
Respiratory Research 2011, 12:111 doi:10.1186/1465-9921-12-111Published: 23 August 2011
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.