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The similarities and differences between pleuroparenchymal fibroelastosis and idiopathic pulmonary fibrosis

Hiroshi Ishii1, Yoshiaki Kinoshita2, Hisako Kushima3

  • 11 Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Fukuoka, Japan.

Chronic Respiratory Disease
|August 8, 2019
PubMed

Insights

Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease affecting upper lobes, distinct from idiopathic pulmonary fibrosis (IPF). PPFE presents unique clinical and physiological features, including preserved lung volumes despite reduced capacity.

Area of Science:

  • Pulmonology
  • Rare Diseases
  • Interstitial Lung Diseases

Background:

  • Pleuroparenchymal fibroelastosis (PPFE) is classified as a rare idiopathic interstitial pneumonia.
  • While often idiopathic, PPFE can be associated with predisposing factors or comorbidities.
  • Histologically, PPFE predominantly affects the upper lobes, unlike idiopathic pulmonary fibrosis (IPF).

Purpose of the Study:

  • To differentiate the clinical and physiological characteristics of PPFE from IPF.
  • To highlight the unique features of upper lobe fibroelastosis.
  • To discuss the proposed diagnostic criteria for idiopathic PPFE.

Main Methods:

  • Comparative analysis of clinical and physiological data between PPFE and IPF patients.
  • Review of histological findings and imaging characteristics.
  • Evaluation of recently proposed diagnostic criteria for idiopathic PPFE.

Main Results:

  • PPFE exhibits distinct features from IPF, including a flattened thoracic cage and preserved residual volume despite reduced forced vital capacity (FVC).
  • Patients with PPFE show milder decreases in walking distance compared to IPF, with characteristic radiographic findings like elevated hilar opacities.
  • Prognosis may correlate with lower lobe fibrosis and elevated Krebs von den Lungen-6, but significant variability exists.

Conclusions:

  • PPFE presents unique clinical and physiological characteristics that distinguish it from IPF.
  • Understanding these differences is crucial for accurate diagnosis and management.
  • Recently proposed diagnostic criteria may aid in identifying idiopathic PPFE.
Keywords:
Pleuroparenchymal fibroelastosisclinical differenceclinical similaritydiagnostic criteriaidiopathic pulmonary fibrosis

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