Pleuroparenchymal fibroelastosis (PPFE) treated with lung transplantation and review of the literature

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.

Keywords: cardiothoracic surgery; interstitial lung disease; transplantation.

Figure 1

Chest X-ray shows bilateral (left more than right) interstitial infiltrates. There is volume loss most marked in the left upper lobe with upward traction of the left hilum.

Figure 2

(A)–(D) Axial cuts of the chest CT (cranial to caudal). Upper lobe predominant pleural thickening (yellow asterisks), upper lobe predominant lung parenchymal fibrosis (red asterisk) and volume loss (blue outline) are seen. Pectus excavatum (blue arrows) and well-preserved lung parenchyma in mid to lower lung zones (C and D) are also appreciated.

Figure 3

Coronal cuts of the chest CT, demonstrating the upper lobe predominant pleural thickening (yellow arrows).

Figure 4

Histology staining demonstrates pleural thickening (red asterisks) and subpleural parenchymal fibrosis and elastic tissue deposition (blue asterisks). Lung parenchyma further away from the pleura is relatively well preserved (yellow asterisk). (A) H&E stain. (B) Movat’s stain. Scanning power microscopy ~40×.