High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: Features and differential diagnosisReport as inadecuate




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Respiratory Research

, 12:111

First Online: 01 December 2011Received: 18 February 2011Accepted: 23 August 2011

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.

Electronic supplementary materialThe online version of this article doi:10.1186-1465-9921-12-111 contains supplementary material, which is available to authorized users.

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Author: Sara Piciucchi - Sara Tomassetti - Gianluca Casoni - Nicola Sverzellati - Angelo Carloni - Alessandra Dubini - Giampaolo Ga

Source: https://link.springer.com/







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