0
Signs and Symptoms of Chest Diseases |

Idiopathic Pleuroparenchymal Fibroelastosis

Elif Sen, MD; Ugur Gonullu, MD; Cetin Atasoy, MD; Hatice Taslak, MD; Ozlem Ozdemir Kumbasar, MD
Author and Funding Information

Ankara University School of Medicine Pulmonary Diseases Department, Ankara, Turkey


Chest. 2013;144(4_MeetingAbstracts):937A. doi:10.1378/chest.1703792
Text Size: A A A
Published online

Abstract

SESSION TITLE: Miscellaneous Global Case Reports

SESSION TYPE: Global Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Idiopathic pleuorparanchymal fibroelastosis is a very rare disease defined recently which is characterized pleural and subpleural parenchymal fibrosis predominantly on upper lobes. Marked apical pleural thickening associated with superior hilar retraction is present at chest X ray analysis and pleural thickening, fibrosis, architectural distortion, traction bronchiectasis and honeycomb lung.in high resolution computed tomography are radiological findings of this rare disease. We present a very rare case of idiopathic pleuraparanchymal fibroelastosis defined according to the radiological findings.

CASE PRESENTATION: We present a twenty two year-old male patient admitted with the complaint of progressive dyspnea since 9 months. He is a nonsmoker, he had any exposures to asbestosis, drugs or he had no environmental exposures. In his physical examination bibasillar crackles were determined. Chest X-ray showed bilateral reticular pattern and bilateral apically located pneumothorax. He had moderate hypoxemia on his arterial blood gases tests. In the thorax CT, bilateral apically located pneumothorax, interstitial fibrotic changes on upper lobes and tractional bronchiectasis were determined. Pulmonary function tests revealed severe restriction, and very low diffusing capacity. Six minute walking distance was 220 m with a severe desaturation. Immunological markers were negative for collagen tissue disorders. The radiological evaluation of this case suggested the diagnosis of “Idiopathic pleuorparanchymal fibroelastosis”. The only therapeutic option is lung transplantation, and we discussed this option with our patient, however he didn’t accept. He is on home oxygen therapy and pirfenidone was started, after a 3 month treatment period a- follow-up evaluation will be planned.

DISCUSSION: This rare form of fibrosis involving pleura and lung parenchyma had been suggested by the thorax radiologist which is characterized pleural and subpleural parenchymal fibrosis predominantly on upper lobes. We haven't perform an open lung biopsy since it could cause serious complications described in the literature (prolonged air leaks...).The differential diagnosis of IPPFE includes: asbestosis, connective tissue diseases, advanced fibrosing sarcoidosis, radiation or drug induced lung disease and chronic hypersensitivity pneumonitis. In this case any of this conditions was present. We describe the characteristic features of idiopathic pleural fibroelastosis radiologically.

CONCLUSIONS: This case presentation aimed to describe radiological and clinical features of this rare and progressive disease.

Reference #1: Piciucchi S et al. High resolution CT and histological findings in idiopathic pleuroparanchymal fibroelastosis features and differential diagnosis

DISCLOSURE: The following authors have nothing to disclose: Elif Sen, Ugur Gonullu, Cetin Atasoy, Hatice Taslak, Ozlem Ozdemir Kumbasar

No Product/Research Disclosure Information


Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Figures

Tables

References

NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
CHEST Journal Articles
PubMed Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543