Pulmonary fibrosis (PF) describes a variety of disorders that may lead to scarring in the lung.
What is Pleuroparenchymal Fibroelastosis (PPFE)?
Pleuroparenchymal Fibroelastosis (PPFE) is a rare form of PF that tends to affect the upper parts of the lung. The scarring tends to involve the areas just beneath the lining of the lungs (the pleura) and the lung itself (the parenchyma).
What are the symptoms?
In PPFE it can be more difficult for the lungs to transfer oxygen into the bloodstream. This means the body may not get the oxygen it needs to work properly. This can cause breathlessness and/or a cough from the irritation. Some people experience a dull pain around the lungs and feel fatigued. Weight loss and recurrent lung infections may occur.
Why does PPFE happen?
It is not entirely clear why PPFE happens. It is sometimes associated with a separate lung fibrosis condition affecting the bottom of the lungs or following previous treatment for cancer.
It is difficult to predict how the condition will develop in a person as it can vary a lot.
What tests will I have?
Your specialists will take a detailed medical history and perform a thorough physical examination. You may be asked to have the following investigations:
- Chest X-ray.
- Lung function tests. These are breathing tests which show how well your lungs are working. They are used later to monitor your lung disease, to see if it is progressing. You may also be asked to have a walk test where you will be asked to rate how breathless you are and measure your walking distance and oxygen levels.
- A series of blood tests will be done to screen for systemic inflammatory conditions.
- A CT scan of your chest will show a detailed picture of your lungs that allow your specialist to identify scarring of the lungs.
- Some people may also have a bronchoscopy, where a small flexible tube is passed down into your lungs to allow collection of cells. This may help with diagnosis and management.
What treatments are available?
Treatments may differ depending on the symptoms you have, and your specialist will discuss which treatment is right for you.
Antifibrotic medications, like nintedanib, can slow down the build-up of scar tissue in the lungs. They may be used to treat PPFE if there is evidence that it is getting worse. These treatments may reduce the rate that your lung fibrosis progresses, but they don’t stop the lung scarring completely. And they won’t get rid of any scar tissue that has already formed.
You may be prescribed an antibiotic to prevent lung infections.
Other medications and therapies are used to help relieve symptoms, such as cough and breathlessness. Your specialist will discuss options with you on an individual basis.
Pulmonary rehabilitation is a supervised exercise and education programme that can help you to learn to manage your breathlessness and remain active. The programmes are multidisciplinary, meaning that the team includes respiratory physiotherapists, nurses, dieticians, doctors and others, and can help improve your energy, strength, and quality of life.
As the lung fibrosis stops enough oxygen getting into the bloodstream, some people may need supplemental oxygen therapy. Oxygen therapy may help with breathlessness and help you to be more active. Corrected levels of oxygen in the blood are necessary for normal body functions and reducing additional health problems.
You should also discuss with your doctor if there are any clinical trials you can participate in. Clinical trials are voluntary research studies designed to answer specific questions about the safety and/or effectiveness of medications.
A small minority of patients may require assessment for and be suitable for lung transplantation.
How can I help myself?
Have your seasonal vaccinations (COVID-19 and flu) and the pneumonia vaccination (you only have this once).
You may be eligible for a variety of benefits such as Attendance Allowance or Personal Independence Payment if you need help with personal care or getting about.
Our specialist nurses run a regular Pulmonary Fibrosis Support Group which is a space for discussion with other patients with lung fibrosis. Here we also aim to have several presentations from a variety of guest speakers and charities.
Keep active and do what you enjoy!
Further information and resources
How to contact us
- Bristol Interstitial Disease Service
Southmead Hospital
Bristol
BS10 5NB - 0117 414 7762
- ILD@nbt.nhs.uk
© North Bristol NHS Trust. This edition published June 2025. Review due June 2028. NBT003790.
Support your local hospital charity
See the impact we make across our hospitals and how you can be a part of it.
