Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease, usually associated with scarring (fibrosis) of the lungs. It tends to affect the upper lobes of the lungs and cause scarring to the area beneath the lung lining (the pleura) and the lung (parenchyma) itself. Until 2017, only 100 cases of PPFE were thought to have been identified, although it is increasingly being recognised as a condition in its own right. 

Common symptoms

PPFE image




PPFE symptoms include:

  • a dry cough
  • shortness of breath, particularly when exercising
  • a dull pain around the lungs
  • episodes of pneumothorax, which is caused by air entering the chest cavity and collapsing the lung

PPFE causes

We don't really know why PPFE happens but it is associated with:

  • a history of bone marrow, lung, or other organ, transplant 
  • previous radiotherapy
  • previous treatment with some (but not all) chemotherapies
  • recurrent lung infections
  • an overactive immune system (in some patients)

PPFE can happen at any age, and can affect younger or older individuals. Smoking cigarettes is not thought to be a risk factor. Sufferers are often of a lean build and have a tendency to lose weight over time. 

At Royal Brompton Hospital, PPFE is managed by our interstitial lung disease (ILD) unit.

Find out more about PPFE.

Managing PPFE

PPFE is a serious disease. It is highly variable which makes it difficult to predict how it will manifest itself in different people. The prognosis can range from poor to relatively stable over a long period of time. 


To monitor your health we will:

  • use lung function tests at regular intervals, usually every 6-12 months, to monitor your condition
  • take x-rays or CT scans of your chest (one of the many tests which can be done), if your condition worsens, to check for any changes, such as pneumothorax or a chest infection
  • conduct walking tests to check whether you need oxygen during exercise
  • conduct an echocardiogram to check that your PPFE is not affecting your heart

Other information 


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Interstitial lung disease (ILD) unit contact details


Lind ward, Fulham wing/South block, Royal Brompton Hospital, Fulham Road

Telephone: +44(0)20 7351 8327
Fax: +44(0)20 7351 8918