ARRS 2022 Abstracts


E1401. Pleuroparenchymal Fibroelastosis and Other Mass-Like Lesions of the Pleura
  1. Sean Hagaman; George Washington University
  2. David Kauffman; George Washington University; George Washington University School of Medicine
  3. Aletta Ann Frazier; University of Maryland School of Medicine
  4. Kamel Gharaibeh; University of Maryland School of Medicine
  5. John Lichtenberger; George Washington University
Pleuroparenchymal fibroelastosis (PPFE) is a rare disease primarily reported in the pathology literature but without clear imaging definitions or characteristics. PPFE primarily involves the visceral pleura and subjacent lung parenchyma of the upper lobes. The etiology is considered to be idiopathic, though there are a number of case reports suggesting correlation with various diseases and syndromes. The progression of PPFE is extremely variable and may lead to respiratory failure and death. The only proven treatment is lung transplantation. A diagnosis is usually made by CT, though the entity is usually first apparent on radiography. Given the lack of awareness of PPFE, there have been suggestions in the literature that this disease is underdiagnosed. The goal of this exhibit is to review the imaging findings associated with pathologically proven PPFE and to highlight how to differentiate this entity from other diseases of the pleura and subpleural parenchyma.

Educational Goals / Teaching Points
This exhibit aims to review the clinical, anatomic, and pathological characteristics and definitions of PPFE; examine the imaging findings most indicative of PPFE; provide a differential of other diseases affecting the pleura and subpleural parenchyma; and highlight features that can help distinguish PPFE from similar disease entities.

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques
PPFE is most prominent in the upper lungs (apical-caudal distribution), affecting both the visceral pleura as well as the subpleural parenchyma. Importantly, it spares the parenchyma distant from the pleura. The key pathologic features of PPFE are subpleural intra-alveolar fibrosis with alveolar septal elastosis (IAFE) and pleural fibrosis of the upper lung zone. The majority of cases also feature vascular fibrointimal thickening of vessels. The key CT findings associated with PPFE include upper lobe pleural thickening with subpleural fibrosis that is markedly more significant than disease in the lower lobes. Other common features include tractional distortion and mosaic attenuation of affected lung parenchyma, volume loss in the upper lobes, traction bronchiectasis of affected airways, honeycombing, and anterior-posterior flattening of the chest (platythorax).

PPFE is a disease only recently recognized in the literature that primarily involves the pleura and subpleural parenchyma of the upper lobes. Although this is primarily a pathology diagnosis, CT features in pathology-proven cases tend to be characteristic. Though the prognosis of affected individuals is highly uncertain, there are documented cases of patients declining rapidly and dying within a year of diagnosis. It is important that radiologists of all levels of training and experience familiarize themselves with this disease entity to improve patient care.