Two Different Aspects of Pleuroparenchymal Fibroelastosis: A Disease of Pulmonary Fibrosis, and of the Chest WallKentaro Watanabe*
Fukuoka University School of Medicine, Respiratory Medicine, Fukuoka, Japan
- *Corresponding Author:
- Kentaro Watanabe
Fukuoka University School of Medicine
Respiratory Medicine, Fukuoka, Japan
E-mail: [email protected]
Received date: February 19, 2016; Accepted date: March 21, 2016; Published date: March 25, 2016
Citation: Watanabe K (2016) Two Different Aspects of Pleuroparenchymal Fibroelastosis: A Disease of Pulmonary Fibrosis, and of the Chest Wall. J Med Surg Pathol 1:111.doi: 10.4172/jmsp.1000111
Copyright: © 2016 Watanabe K. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution and reproduction in any medium, provided the original author and source are credited.
The essential histology of PPFE is subpleural fibroelastosis in the upper lobes, which is different from that of usual interstitial pneumonia (UIP). However, PPFE might share a pathologic process leading to end-stage fibrosis with UIP: it might have an antecedent inflammatory or acute lung injury process prior to the development of the thick subleural bands of fibroelastosis, possibly corresponding to a honeycomb lung in UIP. Fibroblastic foci are found in the leading edge of fibroelastosis, and acute exacerbation occurs in patients with PPFE. The numbers of fibroblastic foci might be correlated with acute exacerbation or poor prognosis in patients with PPFE, as in those with UIP.
Flattened thoracic cage and increased ratio of reserve volume/total lung capacity (RV/TLC) are distinctive characteristics seen in patients with PPFE, but not seen in those with UIP. Flattened thoracic cage that occurs secondary to the fibrotic shrinkage of bilateral upper lobes further decreases the distensibility of thoracic cage, restricting the expansion of the lungs and enhancing the atelectatic shrinkage of upper lobes. Such pathophysiology is similar to that of kyphoscoliosis and ankylosing spondylitis. Therapeutic interventions including the use of steroids and antifibrotic agents for patients with PPFE have disappointing results to date. Chest wall mechanics need to be given more attention in the treatment of patients with PPFE.
Heterogeneous clinical background and clinical course of PPFE remind us that PPFE might need to be named “PPFE syndrome” rather than a single disease.