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Area of Science:

  • Pulmonology
  • Radiology
  • Fibrotic Lung Diseases

Background:

  • Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia.
  • It is characterized by fibrosis and elastosis predominantly in the subpleural regions of the upper lobes.
  • The clinical course, especially of unilateral variants, is not well-characterized.

Purpose of the Study:

  • To describe the long-term clinical course and imaging findings of patients with unilateral upper lobe PPFE.
  • To investigate potential radiologic clues for pleural involvement in this specific PPFE phenotype.

Main Methods:

  • Case report of two patients with unilateral upper lobe PPFE.
  • Longitudinal follow-up of clinical progression and serial imaging (radiography, CT scans).
  • Analysis of imaging features, including subpleural nodules.

Main Results:

  • Both patients survived >10 years with unilateral upper PPFE.
  • Initial rapid disease progression was observed in the years post-diagnosis.
  • A subsequent period of slower progression was noted, with stable subpleural nodules suggestive of rounded atelectasis observed over the 10-year period.

Conclusions:

  • Unilateral upper lobe PPFE can have a variable long-term course, including an indolent phase.
  • Stable subpleural nodules resembling rounded atelectasis may indicate pleural involvement in PPFE.
  • These findings aid in understanding the spectrum of PPFE and managing similar cases.