Although no treatment is known to lessen or stop the course of PPFE, there are treatments which can help. 


  • low dose steroids and/ or immunosuppressive agents may be prescribed if your immune overactivity is thought to be a factor
  • antibiotics may be prescribed as a precautionary measure to prevent lung infections, which seem to be a frequent feature of PPFE


  • pulmonary rehabilitation - an exercise programme to improve breathlessness - is thought to help. It helps maintain fitness and teaches techniques for managing symptoms
  • patients with more advanced disease may be prescribed oxygen which can help with breathlessness if their levels drop below a certain level during a walking test

You may also be referred to the palliative care team to help manage symptoms of cough and breathlessness. This may be with medications such as oramorph (liquid morphine) at a very low dose. Please contact your ILD specialist nurse for more information.

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

Pleuroparenchymal fibroelastosis (PPFE) is a rare but increasingly recognised chronic lung disease associated with scarring of the lungs.

Interstitial lung disease (ILD) unit

Lind ward, Fulham wing/South block, Fulham Road

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