Pleuroparenchymal fibroelastosis (PPFE) treated with lung transplantation and review of the literature. BMJ Case Rep 2019 Apr 20;12(4)
Date
04/22/2019Pubmed ID
31005872Pubmed Central ID
PMC6506043DOI
10.1136/bcr-2019-229402Scopus ID
2-s2.0-85065096490 (requires institutional sign-in at Scopus site) 9 CitationsAbstract
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.
Author List
Ali MS, Ramalingam VS, Haasler G, Presberg KAuthor
Kenneth W. Presberg MD Professor in the Medicine department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdultBiopsy
Dyspnea
Elastic Tissue
Female
Humans
Lung Transplantation
Parenchymal Tissue
Pleural Diseases
Pulmonary Fibrosis
Respiratory Function Tests
Thoracic Wall
Treatment Outcome
