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Pulmonary fibrosis describes a group of lung diseases where the lungs become progressively stiffer and smaller, eventually leading to low oxygen levels in the blood. Pulmonary fibrosis (PF) affects around 70,000 people in the UK.

Pulmonary fibrosis symptoms

The symptoms of pulmonary fibrosis may include:

  • shortness of breath, which increases over time
  • a persistent dry cough
  • rounded and swollen finger tips (finger clubbing).
  • crackles over your lungs heard by your doctor using a stethoscope

Shortness of breath

Breathlessness can be an early or late PF symptom. People often write off shortness of breath, putting it down to lack of fitness and ageing.

In the early stages of PF, you may be breathless when you do strenuous exercise or you walk up hills or stairs. As the disease progresses, you may notice breathlessness with milder activities such as walking, washing, dressing, talking and eating.

Read our advice on managing breathlessness.

Cough and crackles over the lungs

A persistent dry and tickly cough is a common symptom of pulmonary fibrosis. Often patients visit their GP with a cough. On listening to the lungs, the GP hears dry or “Velcro”-type crackles that are a strong indicator of PF.

Read our advice on managing a cough

Rounded and swollen finger tips

Finger clubbing – the widening and rounding of the fingernails or toenails – is a less common pulmonary fibrosis symptom. At the moment we don’t know why some patients develop this and others don’t.

Other symptoms

Other pulmonary fibrosis symptoms may include tiredness or unintentional weight loss.

Some people develop pulmonary hypertension, where there is increased blood pressure within the arteries of the lungs. You may have chest pains, a racing heartbeat and swelling in your body. Your medical team is likely to carry out tests for pulmonary hypertension.

If the pulmonary fibrosis is related to an autoimmune disease, such as rheumatoid arthritis, scleroderma (systemic sclerosis) or mixed connective tissue disease (MCTD), you may also experience symptoms of that condition – for example, problems with joints, skin, or muscles.

Pulmonary fibrosis treatments

There are 23 specialist Interstitial Lung Disease (ILD) centres in the UK, with doctors, nurses, and allied health professionals dedicated to the treatment of pulmonary fibrosis.

Treatment depends on the types and causes of pulmonary fibrosis. There are drug treatments for IPF and treatments for other types of PF. New treatments are being developed through clinical trials all the time.

The only cure for pulmonary fibrosis is lung transplantation. This is a demanding operation with a long list of strict criteria as the procedure has its own risks. But, if successful, people can live active, healthy lives following the transplant.

Pulmonary rehabilitation can help you make the most of the lung function that you have.

Get support

Join a support group in your area

It’s important to remember that you are not alone. There are now over 75 pulmonary fibrosis support groups around the country.

Support groups meet informally to share experiences, provide expert information and raise funds and awareness. Find a group near you.

Call our support line

APF runs an email and telephone support line offering information and advice about living with PF. Find out more.

Find us on social media

We have an active and supportive online community ready to welcome you on Facebook, Twitter and Instagram for updates, news and connections to our online community.

Watch videos of how other people cope day to day with PF.

The work APF is doing for individuals and families affected by PF is great and I would like to help in any way I can.

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