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Table 1 Each diagnostic category of pleuroparenchymal fibroelastosis

From: A proposed prognostic prediction score for pleuroparenchymal fibroelastosis

 

Definite PPFE

Radiologically probable PPFE

Radiologically and physiologically probable PPFE

Symptom

 

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Histology

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Radiology

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Physiology

  

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  1. Circles indicate the required components for the diagnosis
  2. Symptoms: Dry cough or exertional dyspnea with insidious onset
  3. Histology: Subpleural zonal or wedge-shaped dense fibrosis consisting of collapsed alveoli and collagen-filled alveoli with septal elastosis
  4. Radiology: Subpleural airspace consolidation with traction bronchiectasis in upper lobes, and bilateral upward shift of hilar structures and/or volume loss in upper lobes
  5. Physiology: RV/TLC %pred. ≥ 115% and/or BMI ≤ 20 plus RV/TLC %pred. ≥ 80%
  6. Modified from Watanabe et al. [26]