Idiopathic Pulmonary Fibrosis (IPF)

What is pulmonary fibrosis?

Pulmonary fibrosis is when normal lung tissue is replaced by scar tissue and/or inflammation. This makes the lungs stiff and not work as well.

• Pulmonary is the medical term for your lungs.
• Fibrosis means scarring.

We know what causes some types of pulmonary fibrosis. But sometimes we cannot find a cause, including in Idiopathic Pulmonary Fibrosis (IPF). We do know that it is not a form of cancer or cystic fibrosis, and it is not contagious.

How common is IPF?

IPF is rare - only around 14 to 43 people out of 100,000 have IPF. It can affect anyone but is much more common in older people.

What are the symptoms?

At first, people with IPF can have no or only mild symptoms. If the pulmonary fibrosis worsens then you may have:

• Difficulty breathing.
• Dry cough.
• Feeling tired.
• Poor appetite.

IPF can cause weight loss. You should weigh yourself regularly and speak to your GP or team if you are losing weight over time.

What tests will I have?

The doctors will take a detailed medical history from you and do a physical exam. You may also have the following tests:

• Chest X-ray.
• Lung function tests – to see how well your lungs work. They can also monitor how your condition is progressing. This may include a walking test to see how breathless you get.
• CT scan – this will give us a detailed picture of your lungs including any scarring or inflammation.
• Blood tests – to rule out other causes of pulmonary fibrosis.
• If the diagnosis is still unclear, you might have surgery to take a lung biopsy (sample).

Will my lungs get worse?

In the past we thought all patients with IPF would get worse quickly. Now we know that some patients get worse more slowly, and some can stay the same for some time.

Unfortunately we cannot tell which patients will get worse and how quickly. This is an important area of research so we can understand this more and find better treatments.

If your symptoms change, that might mean your condition is getting worse. Speak to your respiratory team and mention any changes you have noticed.

If your symptoms suddenly get worse, speak to your GP.

How is IPF treated?

The fibrosis in IPF is permanent and cannot be cured but treatment can help slow progression or manage symptoms.

Medication

• Your specialist might suggest you try medications to slow the scarring - antifibrotic medications.
• They may suggest advise different medications to help your symptoms.

Pulmonary rehabilitation

• Pulmonary rehabilitation is a supervised exercise and education programme.
  • It helps you manage breathlessness, stay active, and improve strength and energy.
  • The team may include physiotherapists, nurses, dietitians, and doctors.

Supplemental oxygen

• Lung scarring can reduce the amount of oxygen that can get into your blood.
• Some people may need oxygen therapy if oxygen levels in the blood are low.
• This can improve breathlessness and help you stay active.

Clinical trials

• You can ask about clinical trials which test new treatments. Joining is voluntary.

Lung transplants

• A small number of patients may be referred for lung transplant assessment.

How can I help myself?

• Have your seasonal vaccinations (COVID-19 and flu) and the pneumonia vaccination (only once).
• You may be eligible for benefits like PIP (Personal Independence Payment) if you need help with personal care or getting about.
• Our specialist nurses runs a regular Pulmonary Fibrosis Support Group which is a space for discussion with other patients with similar lung conditions. Here we also aim to have presentations from a variety of guest speakers and charities.
• Keep active and do what you enjoy!

Resources and how to contact us

© North Bristol NHS Trust. This edition published April 2026. Review due April 2029. NBT003582