What is Pulmonary Fibrosis?

Pulmonary fibrosis is a term that describes the formation of scar tissue within the lungs.

Pulmonary fibrosis (PF) is thought to affect around 70,000 people in the UK. There are around 200 different lung diseases which cause damage to the lungs, making it hard for oxygen to leave the lungs and get around the body, resulting in being short of breath.

One of the most aggressive and common forms of PF is Idiopathic Pulmonary Fibrosis (IPF). Idiopathic means it has no known cause. IPF normally affects people over 50 and is slightly more common in men than women. There are limited treatments and there is no cure. Life expectancy can be between three to five years following diagnosis, and around 6,000 people die from it every year in the UK.

Generalised signs of pulmonary fibrosis include:

  • shortness of breath
  • a persistent dry cough
  • in the presence of both of the above, rounded and swollen finger tips (clubbed fingers).

In some people scarring of the lungs develops very quickly, while in others it happens over a longer period of time.

“I find I can do less and less as my breathlessness increases and my muscles don’t get enough oxygen. But when I get out of breath I just stop and admire the view.”

Mick Donoghue, passionate walker and IPF patient.

What causes pulmonary fibrosis?

The type of fibrosis you are diagnosed with will indicate what has caused it. For example, this could be as a result of rheumatoid arthritis, certain drugs or exposure to birds. When there is no known cause it is classed as idiopathic pulmonary fibrosis.

Read more about causes of pulmonary fibrosis and potential factors relating to IPF.

What are the symptoms of pulmonary fibrosis?

The most common symptom is a persistent dry and tickly cough. Often patients visit their GP with this symptom and on listening to the lungs the GP hears a particular sound (known as Velcro crackles) that is a strong indicator of IPF. They will then recommend the patient for further investigation.

Shortness of breath can be an early or late symptom. People often write off shortness of breath, putting it down to lack of fitness and ageing. In the early stages this may occur with more strenuous exercise or walking up hills or stairs. However as the disease progresses, you may notice breathlessness with milder activities such as washing, dressing, talking and eating.

Other symptoms may include tiredness or unintentional weight loss. A less common symptom is finger clubbing – the widening and rounding of the fingernails or toenails. At the moment we don’t know why some patients develop this and others don’t.

If the pulmonary fibrosis is related to another disease you will also experience symptoms of that condition, such as lethargy or swollen and aching joints in rheumatoid arthritis.

Unfortunately, as PF is relatively rare, diagnosis can take several months or even years. A large-scale survey of people with IPF, undertaken in 2018 by Action for Pulmonary Fibrosis, uncovered a widespread and unacceptable level of misdiagnosis. Our survey revealed that 35% of respondents were misdiagnosed while only 46% said they had been correctly diagnosed within six months of visiting their GP.

However, there are a number of specialist centres with doctors dedicated to the treatment of lung disease once a diagnosis had been made.

What treatments are available?

Treatment will depend on the type and cause of your PF. If your disease is immune related steroids are often a first line treatment. If the disease is hypersensitivity pneumonitis avoiding the trigger is paramount (eg. sorting out housing damp, avoiding hot tubs and birds). If the PF is drug related medication may need to be stopped, in discussion with your doctor.

Effective drug treatments for IPF are relatively recent. There are two drug therapies which have been shown to slow the progression of the disease in some people called nintedanib and pirfenidone. They can only be prescribed by a specialist doctor under rules set by NICE, the National Institute of Health and Care Excellence. However, the latest research shows that these drugs may also be effective for patients with other types of progressive pulmonary fibrosis.

Ambulatory (portable) oxygen helps with day-to-day living: you may be able to walk further, and feel less tired. Long term oxygen therapy keeps the oxygen level in the blood stable for 15 hours or more. Oxygen therapy at night improves oxygen levels during sleep, protecting your internal organs and improving life expectancy, by preventing the development of pulmonary hypertension. Oxygen therapy involves having large cylinders, tubes and pipes in the home, and smaller cylinders with backpacks to use when out and about.

You can have a special assessment to see whether using oxygen will help.

The only ‘cure’ for pulmonary fibrosis remains a lung transplant. This is a demanding operation, and patients need to be fit, both physically and mentally, to undergo it. The procedure has its own risks but, if successful, people can live active, healthy lives following the transplant. Read more about lung transplantation.

How can I find support?

It’s important to remember that you are not alone. There are now over 75 pulmonary fibrosis Support Groups around the country and we have a dedicated support line.

Join a support group

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

Call us

APF also runs a telephone Support Line offering information and advice about living with PF. You can call Lucy, our specialist nurse, on 01223 785 725. Just leave a message giving your name and phone number, and Lucy or one of our team of specialist nurses and patient ambassadors will call you back as soon as we can. You can also email us at  

Find us online

If you just want to keep in touch you can follow us on Facebook, Twitter and Instagram for updates, news and connections to our online community.

“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.”

Katy Gallagher, fundraiser who lost her mum to IPF.