Treatment for pulmonary fibrosis

Your treatment will depend on the cause of the fibrosis. Once lung scarring occurs in the lungs it cannot be reversed, so there is no cure for existing fibrosis, whatever the cause.

Find out more about treatments for:

• Idiopathic pulmonary fibrosis (IPF)
• Hypersensitivity pneumonitis
• Occupational interstitial lung diseases
• Pulmonary fibrosis associated with connective and autoimmune diseases
• Drug-induced pulmonary fibrosis

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Treatment of your condition

• Drug treatments aim to stabilize or slow down the rate of scarring in the lungs. Specific anti-fibrotic drugs can be used to treat IPF for people whose lung function tests fall within a certain range.
• 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.

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.

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 - just 214 lung transplants were carried out in England in 2017-18. And 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 body mass index. 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 transplant. According to UK data, 81% of people are alive one year after a lung transplant, and 56% are alive 5 years afterwards. The survival rate at 10 years is 34%.

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Supportive treatment

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.

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Controlling your symptoms

Help to stop smoking. If you smoke, stopping is very important. Your GP can refer you to free help to stop smoking.

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 and 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 farther, you feel less breathless and generally feel more positive.

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.

Next: find out how to look after yourself >