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Idiopathic Pleuroparenchymal Fibroelastosis

By Joyce Lee, MD, MAS, Assistant Professor, Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, University of Colorado Denver

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Idiopathic pleuroparenchymal fibroelastosis is a rare idiopathic interstitial pneumonia that predominantly involves the upper lobes of the lungs and is slowly progressive. Patients often have recurrent infections, shortness of breath, and dry cough. Diagnosis is with high-resolution CT. Corticosteroids may be given.

Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare condition that is classified as an idiopathic interstitial pneumonia (1). It involves upper lobe fibrosis of the pleura and subpleural lung parenchyma.


  • 1. Travis WD, Costabel U, Hansell DM, et al: An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med 188 (6):733–748, 2013.


The cause is unknown, but clinical data suggest a link to recurrent pulmonary infection. Genetic and autoimmune mechanisms are also thought to play a role in this disease.

Symptoms and Signs

The median age of presentation is around 57 years, with no sex predilection. Most patients are nonsmokers. Patients often report a history of recurrent infections, shortness of breath, and dry cough. Pneumothorax is common during the course of the disease.


  • High-resolution CT (HRCT)

  • For confirmation, surgical lung biopsy

The imaging findings include upper lobe thickening of the pleura and subpleural regions. They can have co-existing findings of other interstitial pneumonias, including usual interstitial pneumonia and nonspecific interstitial pneumonia pattern. Patients can also have areas of consolidation and bronchiectasis.

The pathology is characterized by intra-alveolar fibrosis with the alveolar walls in these areas showing prominent elastosis and dense fibrous thickening of the visceral pleura. In some patients, there is co-existent interstitial pneumonia in the lower lobes. Surgical lung biopsy is required for confirmation of the diagnosis.


The clinical course in patients with PPFE tends to be progressive in the majority of patients. Disease progression occurs in 60% of patients.


  • Possibly corticosteroids

The appropriate treatment for this condition is unknown. The majority of the literature reports the use of corticosteroids.