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Insights into Imaging
Published in: Insights into Imaging 1/2016

Open Access 01-02-2016 | Report

Pleuroparenchymal fibroelastosis: role of high-resolution computed tomography (HRCT) and CT-guided transthoracic core lung biopsy

Authors: Cátia Esteves, Francisco R. Costa, Margarida T. Redondo, Conceição S. Moura, Susana Guimarães, António Morais, José M. Pereira

Published in: Insights into Imaging | Issue 1/2016

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Abstract

Objectives

Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia (IIP) with variable clinical and radiological features. Diagnosis is based on histology obtained by surgical lung biopsy, which is associated with significant mortality and morbidity. This study aims to briefly review PPFE and discuss the role of CT-guided transthoracic core lung biopsy (TTB) in its diagnosis.

Materials/Methods

Four cases of PPFE diagnosed at our institution with TTB are reported and discussed.

Results

Clinical, radiological and histological features are in agreement with the previous literature cases. TTB provided the diagnosis in all cases. Iatrogenic pneumothorax was the main complication in all patients. Placement of a chest tube was needed in three patients. An overlap between PPFE and other interstitial lung diseases (ILD) was documented.

Conclusion

PPFE is an underdiagnosed IIP, so radiologist awareness of it needs to be widespread in patients with fibrosis with apical-caudal distribution. Coexistence of different lung diseases strengthens the idea of a predisposing factor. TTB proved to be a good diagnostic tool and can be considered the first choice for invasive assessment of these patients. PFFE has a variable course with no established therapeutic options; therefore a multidisciplinary team is crucial in the approach to patients with ILD.

Main messages/Teaching Points

• PPFE should be considered in the differential diagnosis of fibrosis with apical-caudal distribution.
• CT-guided TTB can be considered the first choice for invasive assessment of PPFE.
• Site of biopsy has to be chosen carefully in order not to miss PPFE.
• Coexistence of different lung diseases strengthens the idea of a predisposing factor.
• A multidisciplinary team is crucial in the approach to patients with ILD.
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Metadata
Title
Pleuroparenchymal fibroelastosis: role of high-resolution computed tomography (HRCT) and CT-guided transthoracic core lung biopsy
Authors
Cátia Esteves
Francisco R. Costa
Margarida T. Redondo
Conceição S. Moura
Susana Guimarães
António Morais
José M. Pereira
Publication date
01-02-2016
Publisher
Springer Berlin Heidelberg
Published in
Insights into Imaging / Issue 1/2016
Electronic ISSN: 1869-4101
DOI
https://doi.org/10.1007/s13244-015-0448-3

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