MSD Manual

Please confirm that you are a health care professional

Loading

Idiopathic Pleuroparenchymal Fibroelastosis

By

Joyce Lee

, MD, MAS, University of Colorado Denver

Last full review/revision Sep 2019| Content last modified Sep 2019
Click here for Patient Education
NOTE: This is the Professional Version. CONSUMERS: Click here for the Consumer Version
Topic Resources

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 but sometimes requires lung biopsy. 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.

General reference

  • 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.

Etiology

The cause of idiopathic pleuroparenchymal fibroelastosis 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 with idiopathic pleuroparenchymal fibroelastosis 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.

Diagnosis

  • High-resolution CT (HRCT)

  • For confirmation, surgical lung biopsy

The imaging findings in idiopathic pleuroparenchymal fibroelastosis include upper lobe thickening of the pleura and subpleural regions. Patients 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.

Prognosis

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

Treatment

  • Possibly corticosteroids

The appropriate treatment for idiopathic pleuroparenchymal fibroelastosis is unknown. The majority of the literature reports the use of corticosteroids.

Click here for Patient Education
NOTE: This is the Professional Version. CONSUMERS: Click here for the Consumer Version
Professionals also read

Also of Interest

Videos

View All
How to do Needle Thoracostomy
Video
How to do Needle Thoracostomy
3D Models
View All
Pneumothorax
3D Model
Pneumothorax

SOCIAL MEDIA

TOP