Treatment for pulmonary fibrosis

Treatment for pulmonary fibrosis will depend on the cause of the fibrosis. On this page we explain some of the different treatments available.

Preparing for your pulmonary fibrosis appointments

We know that sometimes people are unsure about how to speak to their healthcare professional about their lung condition or are confused about what questions to ask during a pulmonary fibrosis review. 

Our pulmonary fibrosis Patient Passport best care checklist can help. It’s a simple tool that only takes around five minutes to complete. It gives you a personalised report about your pulmonary fibrosis care, including things you could ask your healthcare professional at your review about the care you’re receiving.

You can take this report to your healthcare professional and use it to start a conversation about how to get the best care for your pulmonary fibrosis.

Use our pulmonary fibrosis Patient Passport.

Can pulmonary fibrosis be cured?

Once lung scarring occurs in the lungs it cannot be reversed. This means there is no cure for existing fibrosis, whatever the cause. But there are things you can do to control your symptoms and look after yourself. Read more about the outlook for pulmonary fibrosis.

How is pulmonary fibrosis treated?

Help to stop smoking

If you smoke, stopping is very important for your lung condition and overall health. Your GP can refer you to free help to stop smoking.

Drugs and medication

Drug treatments aim to stabilise or slow down the rate of scarring in the lungs. Specific drugs can be used to treat some people with pulmonary fibrosis who reach certain criteria:

  • in people with IPF, Pirfenidone and Nintedanib can be used for those whose lung function tests fall within a certain range. Read more about this in our IPF information
  • in people with progressive fibrotic ILD (PF-ILD), Nintedanib may be used as well as or instead of immunosuppressant drugs. Forms of PF-ILD include systemic sclerosis associated ILD, autoimmune ILDs, and hypersensitivity pneumonitis.

Steroids or other immunosuppressant drugs are used to suppress inflammation in the lungs.

In conditions like hypersensitivity pneumonitis, it’s key to avoid being exposed to the allergen causing symptoms, if the allergen can be identified.

Any drugs causing problems with your lungs will be stopped.

Lung transplant

For a very few people, having a lung transplant might be an option if the pulmonary fibrosis progresses and isn’t stabilised by treatment. Transplants are rare – according to the NHS Annual Activity Report, 91 lung transplants were carried out in the UK in 2020-21, although this statistic was affected by the coronavirus pandemic. Not all of these were for people with pulmonary fibrosis.

Whether you can be considered for a lung transplant depends on factors that influence the chance of a successful outcome, such as your general health, other medical conditions and your weight. There is no age cut-off, but it’s unusual to accept people much over 65 years old. There are not enough donor lungs available to meet demand. If your doctor thinks you might be suitable, you’ll be referred to a transplant unit for further assessment and a decision.

There are significant risks in having a lung transplant. According to UK data, 81% of people are alive one year after a lung transplant, and 58% are alive five years afterwards. The survival rate after 10 years is 38%.

Best supportive care

Treating just the symptoms, rather than the cause of a disease, is often called best supportive care. As soon as you are diagnosed, your doctor, nurse or physiotherapist should talk to you about ways to reduce your symptoms and support your mental wellbeing. They may be able to do this at your usual place of care or you may be referred to a specialist in this area, usually a palliative care consultant.

Palliative care specialists are experts in symptom management and may help at various times during your illness, not just in the final stages. Palliative care focuses on controlling your symptoms, such as breathlessness, fatigue and anxiety. It may also stop treatments that are not working or causing side effects. The focus is on addressing issues to improve the quality of life for you, your family and carers.

Controlling your symptoms

For coughing, your doctor might treat problems that could be making it worse, such as heartburn (acid reflux) or a stuffy nose.

Tell your doctor if you have symptoms of acid reflux, such as heartburn, indigestion or a sour taste at the back of your mouth. There’s evidence this may make inflammation and fibrosis worse. It can also make a cough worse.

Feeling out of breath can have a serious effect on your everyday life. It can be a frightening experience, too.

Pulmonary rehabilitation is an important way to help you cope with breathlessness by increasing your fitness and ability to cope with feeling out of breath. The course is designed to support and reassure you, as well as help your condition. You may find you can walk further, feel less breathless and generally feel more positive. Ask your team about this.

I was amazed at how helpful pulmonary rehabilitation was. It told me things I needed to know. It gave me the confidence to go to the gym, even with my oxygen. I made a friend on the course and we went together. Lesley

If your condition gets worse, the level of oxygen in your blood may fall and this may make you feel more breathless. If this happens, your doctor will refer you for an assessment for oxygen therapy. Using oxygen is a way of keeping more active, and later some people will use it at rest too. Your oxygen prescription will be tailored to meet your individual needs.

If you have distressing symptoms of breathlessness and really troublesome coughing, tell your doctor. They may prescribe you low doses of morphine and sedatives to help.

You may struggle to cope emotionally with having a serious condition like pulmonary fibrosis. You are not alone. Many people with a long-term lung condition feel anxious, have a low mood or symptoms of depression. It’s important to look after your mental wellbeing. Your doctor or nurse will understand and can help.

Clinical trials

As well as existing therapies, you might be invited to take part in a medical study, also called a clinical trial, to investigate new treatments. It’s not an option for everyone - if you want to know more, ask your doctor.

Treatment can vary depending on the type of pulmonary fibrosis you have. Find out more about treatments for:

Looking after yourself

If you have pulmonary fibrosis, you can do a lot to help yourself by leading a healthy lifestyle. Feeling very tired is a common symptom and health problems that used to be minor - such as catching a cold – can become more serious.

These tips will help you stay as healthy as possible:

  • Have a flu jab each year and avoid being around people with colds. Talk to your doctor about the coronavirus vaccination to make sure you’re up to date.
  • Ask for a pneumonia vaccination. This is a jab you have just once.
  • Stay as fit as you can. There are many different ways to be active – find one that you enjoy. Take a look at our exercise handbook and our exercise videos.
  • Eat a healthy, balanced diet and maintain a healthy weight. Ask your doctor or nurse to refer you to a dietician, who can give you tailor-made advice, especially if you are losing weight.
  • Try techniques and positions to help your breathing. If you have pulmonary fibrosis, you may tend to breathe very fast and shallowly - a bit like panting. You can use techniques and positions to help you control and slow down your breathing. You can also use them to avoid getting too breathless when you exert yourself, and to help you recover when you do get out of breath. Try the different breathing techniques to find what helps you and practice the ones that help. Talk to your respiratory physiotherapist or nurse for help to find out what works for you.

“I have done a lot of research into my condition and how I can best look after myself. You have to do the best you can to look after yourself, and that includes discovering more about your own condition and how your lungs work.

I make sure to do my breathing exercises regularly. I do them sitting down, moving my chest and taking deep breaths. I also try to keep my diet healthy and balanced and I make sure that I don’t stress over the little things anymore.” Sofia, who was diagnosed with an interstitial lung disease (ILD) aged 28.

Read Sofia’s story.


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