Idiopathic Pleuroparenchymal Fibroelastosis

The most common symptoms of PPFE are:

• Dyspnea⁸
• Nonproductive cough⁸
• Weight loss⁸
• Pneumothorax^1,8
• Chest pain⁹
• Thoracic cage deformity⁸
• Crackles (occasionally audible⁹)

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

• 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

• 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

• 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