Idiopathic Pleuroparenchymal Fibroelastosis

Chapters

 

DIAGNOSIS

SYMPTOMS

The most common symptoms of PPFE are:

  • Dyspnea8
  • Nonproductive cough8
  • Weight loss8
  • Pneumothorax1,8
  • Chest pain9
  • Thoracic cage deformity8
  • Crackles (occasionally audible9)

DIAGNOSTIC TESTS

Pulmonary function tests9

  • Decreased FVC
  • Increased FEV1/FVC
  • Decreased total lung capacity (TLC)
  • Increased residual volume (RV)/TLC
  • Decreased DLCO
  • Normal or minimally decreased DLCO/alveolar volume (VA)

HRCT1

  • Dense subpleural consolidation with traction bronchiectasis, architectural distortion, and upper lobe volume loss

Surgical lung biopsy is required to confirm the diagnosis10

  • Intra-alveolar fibrosis with prominent elastosis of the alveolar walls and dense fibrous thickening of the visceral pleura

Chest X-ray9

  • Abnormally narrowed anterior-posterior thoracic dimension (flattened thoracic cage)
  • Elevated hilar opacities
  • Reticular and nodular opacities in the bilateral upper lung fields
  • Fibrocystic opacities in the upper lung fields and occasional reticular opacities in the lower lung fields in the advanced stage

Serum tests9

  • Krebs von den Lungen-6 (KL-6) levels that are normal or around the upper limit of normal
  • Elevated Surfactant protein D
  • Autoantibodies may be elevated

See also

Man smiling

Test your knowledge

Take the test 

Bridging gaps in ILD education image

Bridging educational gaps in ILD

Learn more 

Interstitial lung disease in systemic sclerosis with a focus on chest CT

Media library

See media