Pleuroparenchymal Fibroelastosis: Associations and Underlying Conditions

ISSN: 1875-6387 (Online)
ISSN: 1573-398X (Print)

Volume 13, 4 Issues, 2017

Download PDF Flyer

Current Respiratory Medicine Reviews

This journal supports open access

Aims & ScopeAbstracted/Indexed in

Submit Abstracts Online Submit Manuscripts Online

Joseph Varon
The University of Texas Health Science Center
Houston, TX

View Full Editorial Board

Subscribe Purchase Articles Order Reprints

Pleuroparenchymal Fibroelastosis: Associations and Underlying Conditions

Current Respiratory Medicine Reviews, 9(4): 248-253.

Author(s): Farnoosh Tayyari, Tae-Bong Chung and David M Hwang.

Affiliation: Department of Pathology, Toronto General Hospital, Room 11th Floor, 200 Elizabeth Street, Toronto, Ontario, M5G 2C4, Canada.


Pleuroparenchymal fibroelastosis (PPFE) is a clinicopathologic entity characterized by clinical presentation suggestive of a chronic idiopathic interstitial pneumonia, radiologic features of pleural and parenchymal involvement accentuated in the upper lobes, and a constellation of histologic findings including visceral pleural fibrosis and prominent fibroelastosis of the subpleural lung parenchyma. While the large majority of cases of PPFE have been considered idiopathic, development of PPFE in post-bone marrow transplant patients and in lung transplant recipients has recently been reported. Further, with the growing number of idiopathic PPFE cases reported in the literature, interesting patterns of association between PPFE and various clinical conditions – including prior treatment for malignancy, autoimmunity, recurrent infections, and vascular compromise/ischemia – are beginning to emerge. These associations, reviewed here, may offer clues into the pathogenesis of this rare condition.


Chronic lung allograft dysfunction, graft versus host disease, interstitial lung disease, pleuroparenchymal fibroelastosis, pulmonary fibrosis, pulmonary upper lobe fibrosis, restrictive allograft syndrome.

Purchase Online Order Reprints Order Eprints Rights and Permissions

Article Details

Volume: 9
Issue Number: 4
First Page: 248
Last Page: 253
Page Count: 6
DOI: 10.2174/1573398X113096660024
Price: $58
13th Asthma & COPD

Related Journals

Webmaster Contact: Copyright © 2016 Bentham Science