Article Text

Download PDFPDF
CASE REPORT
Pleuroparenchymal fibroelastosis (PPFE) treated with lung transplantation and review of the literature
  1. Muhammad Sajawal Ali1,
  2. Vijaya Sivalingam Ramalingam1,
  3. George Haasler2 and
  4. Kenneth Presberg1
  1. 1 Department of Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
  2. 2 Department of Cardiothoracic Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
  1. Correspondence to Dr Vijaya Sivalingam Ramalingam, vramalingam{at}mcw.edu

Abstract

A 26-year-old woman presented with a 15-year history of non-progressive dyspnoea. Chest imaging showed bilateral apical pleural and parenchymal scarring, pleural thickening and bronchiectasis. Pulmonary function tests showed a moderate restrictive defect. Non-invasive workup was non-revealing; therefore, the patient was referred for video-assisted thoracic surgery and lung biopsy. Histopathology revealed pleural thickening and, subpleural parenchymal fibrosis and elastic tissue deposition. Lung parenchyma further away from the pleura was well preserved. Based on these findings, the patient was diagnosed with pleuroparenchymal fibroelastosis (PPFE). Since PPFE is a progressive disorder without effective medical therapies, and given our patient’s worsening symptoms, she underwent bilateral lung transplantation. It has been almost 4 years since the lung transplantation, our patient continues to do well. To the best of our knowledge, to date, this is the longest follow-up reported for a PPFE patient undergoing lung transplantation.

  • interstitial lung disease
  • cardiothoracic surgery
  • transplantation
View Full Text

Statistics from Altmetric.com

Footnotes

  • Contributors MSA and VSR conceived the idea and design of this case report. MSA performed the literature search. All authors (MSA, VSR, GH and KP) contributed to the drafting of the manuscript, read the final manuscript and provided their approval.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Obtained.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.