Kentaro Watanabe
Fukuoka University School of Medicine, Respiratory Medicine,
Fukuoka
Japan
Review Article
Two Different Aspects of Pleuroparenchymal Fibroelastosis: A Disease of Pulmonary Fibrosis, and of the Chest Wall
Author(s): Kentaro WatanabeKentaro Watanabe
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 thoraci.. View More»
