Diffuse Lung Disease |

A Double Whammy? Pleuroparenchymal Fibroelastosis (PPFE) Associated With Nonspecific Interstitial Pneumonia (NSIP) FREE TO VIEW

Rajesh Zacharias, MBBS; Ryan Shipe, MD; Michael Colancecco, DO; Armando Fraire, MD; Katherine Devitt, MD
Author and Funding Information

University of Massachusetts, Shrewsbury, MA

Chest. 2014;146(4_MeetingAbstracts):380A. doi:10.1378/chest.1994098
Text Size: A A A
Published online


SESSION TITLE: Interstitial Lung Disease Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: To describe clinicopathologic features of a rare interstitial lung disease.

CASE PRESENTATION: A 36 year old,previously healthy,non-smoking,Dominican woman presented with two years of exertional dyspnea & no other symptoms.Oxyhemoglobin saturation was 97% on room air and her lungs were clear to auscultation.Chest roentgenogram was notable for extensive apical fibrosis,upper lobe volume loss and diaphragmatic tenting,different from a normal film two years prior.Computed tomography of the chest demonstrated bi-apical pleuroparenchymal scarring and ground glass opacities in the upper & mid lung zones.Pulmonary function tests showed a mixed obstructive restrictive pattern with severe gas transfer deficit.Rheumatologic work-up,tuberculin testing and sputum microbiology including AFB cultures were unrevealing.Histopathology from left upper lobe surgical lung biopsy showed central areas of NSIP, pleural thickening with extension to the parenchyma & positive trichrome and elastin stains:PPFE. As NSIP was found,corticosteroids were initiated.At follow up, she has both symptomatic & PFT improvement.

DISCUSSION: PPFE is a rare interstitial lung disease.It can be idiopathic or associated with radiation,lung or bone marrow transplant,autoimmune diseases,occupational exposures to asbestos or aluminium,hypersensitivity pneumonitis and infections.Patients tend to be young, non-smokers in their 3rd or 4th decade.PPFE is characterized by upper lobe predominant fibrosis and visceral pleural thickening,collageneous fibrosis, subpleural elastosis and intra-alveolar collagenous fibrosis.Disease course tends to be rapidly progressive & fatal or a slow decline over 10 to 20 years.Associated biopsy proven usual interstitial pneumonia has been described.To our knowledge,this may be the first reported case of PPFE with a background of NSIP.The patient’s clinical improvement may be due to the steroid responsive nature of NSIP. It also suggests a clinical variant of PPFE with a better prognosis,though her long term course is yet to be determined.

CONCLUSIONS: The diagnosis of PPFE requires a high index of suspicion & surgical lung biopsy.Consideration must be given to presence of coexistent interstitial lung disease that may respond to conventional treatment.

Reference #1: Watanabe K.Pleuroparenchymal Fibroelastosis:Its clinical characteristics.Respir Med Rev.2013 Jun;9:299-237.

Reference #2: Reddy T,Tominaga M,Hansell DM,et al.Pleuroparenchymal Fibroelastosis:Its pathological characteristics.Curr Respir Med Rev.2013 Aug;9(4):238-247.

DISCLOSURE: The following authors have nothing to disclose: Rajesh Zacharias, Ryan Shipe, Michael Colancecco, Armando Fraire, Katherine Devitt

No Product/Research Disclosure Information




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.

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