Format

Send to

Choose Destination
Respir Res. 2011 Aug 23;12:111. doi: 10.1186/1465-9921-12-111.

High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis.

Author information

1
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

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.

PMID:
21861891
PMCID:
PMC3197499
DOI:
10.1186/1465-9921-12-111
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for BioMed Central Icon for PubMed Central
Loading ...
Support Center