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Park, Chae, and Song: Idiopathic Pleuroparenchymal Fibroelastosis, a Rare Entity of Interstitial Pneumonia: A Case Report
Chest Radiology

Journal of the Korean Society of Radiology 2014; 70(4): 255-259.

Published online: 9 April 2014

DOI: https://doi.org/10.3348/jksr.2014.70.4.255

Idiopathic Pleuroparenchymal Fibroelastosis, a Rare Entity of Interstitial Pneumonia: A Case Report

Jong Chun Park, MD1, 2, Eun Jin Chae, MD1, Joon Seon Song, MD3

1Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.

2Department of Radiology, School of Medicine, Catholic University of Daegu, Daegu, Korea.

3Department of Pathology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.

Corresponding author: Eun Jin Chae, MD. Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 138-736, Korea. Tel. 82-2-3010-5389, Fax. 82-2-476-4719, ejinchae@amc.seoul.kr

Received 22 December 2013       Accepted 14 February 2014

Copyright © 2014 The Korean Society of Radiology

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described, very rare type of fibrotic interstitial lung disease predominantly involving subpleural areas of both upper lungs. IPPFE has distinctive radiologic and pathologic features: progressive subpleural opacity with fibrotic changes, predominantly in upper lungs, and dense elastic component on histology. We experienced one case of surgically confirmed IPPFE, with progression of radiologic findings on the serial CT examinations. We herein report the characteristic radiologic features of IPPFE with pathologic and clinical manifestations.

Keywords: Idiopathic Pleuroparenchymal Fibroelastosis, Interstitial Lung Disease, Upper Lobe Fibrosis, Lung

Figures and Tables

Fig. 1
A 57-year-old female who showed fibrotic interstitial disease predominantly involves both apical lungs.
A. Chest radiograph shows bilateral irregular opacities with volume loss and hilar elevation in the subpleural area of both upper lungs.
B, C. CT shows bilateral subpleural irregular opacities, pleural thickening (long arrows) and traction bronchiolectasis (short arrows) in both upper lobes on lung window setting (B) and mediastinal setting (C).
D. CT shows subpleural reticular opacities (long arrows) with mild traction bronchiectasis (short arrow) in both lower lobes.
jksr-70-255-g001
Fig. 2
Coronal reformation CT images shows progressive subpleural irregular opacities and traction bronchiectasis with volume loss in both upper lungs, and less progressive subpleural reticular opacities with volume loss in both lower lungs than upper lungs; 8 years ago (A), 2 years ago (B), present (C).
jksr-70-255-g002
Fig. 3
Histopathologic features suggesting idiopathic pleuroparenchymal fibroelastosis, which revealed by surgical lung resection.
A. Right upper lobe lung shows dense fibrosis along the subpleura (arrow). The fibrosis extends into away from the pleura and shows perilobular distribution with alveolar septum (haematoxylin and eosin, × 2).
B. Dense elastic fibers (arrows) are irregularly scattered throughout the fibrosis on Elastic Van Geison stain (elastic Van Gieson staining, × 20).
C. Right lower lobe lung parenchyma shows typical microscopic features of idiopathic pulmonary fibrosis including subplerual fibrosis, patchy distribution, temporal heterogeneity and interstitial fibroblastic foci with microscopic honeycomb change (arrow) (haematoxylin and eosin, × 2).
D. The fibrotic area of subpleura shows small amount of deposition of elastic fiber (arrow) which is as same as normal lung pleura and parenchyma (elastic Van Gieson staining, × 20).
jksr-70-255-g003

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