How is pulmonary fibrosis treated and managed?

Treatment for pulmonary fibrosis can vary depending on the type you have, what has caused it, and whether or not it’s progressing.

Your pharmacist can explain how your medicines work, discuss possible side effects, and check whether medicines are safe to take together.’ 

Nazir Hussain, NHS pharmacist and Asthma + Lung UK's Clinical Lead.

Antifibrotics

Pulmonary fibrosis causes scarring (fibrosis) in your lungs. It affects the tissue in and around the tiny air sacs in your lungs (interstitial tissue). Antifibrotics can help slow down scarring. However, they cannot treat scarring that has already developed. 

The two main antifibrotics used for pulmonary fibrosis are: 

• Pirfenidone, which is mainly used for idiopathic pulmonary fibrosis (IPF) 
• Nintedanib, which is used for IPF, but can also be used for other types of interstitial lung diseases (ILDs) which are progressive and fibrotic.

NICE guidelines on prescribing antifibrotics have changed, so more people with progressive pulmonary fibrosis can benefit from them.

Your specialist team will consider your diagnosis, test results, how your condition is changing, and your overall health when deciding whether an antifibrotic medicine is suitable for you. 

If you are prescribed antifibrotics, regular reviews are important to check: 

• any changes to your lung function
• if the medicines are affecting your liver
• how you’re coping with any side effects.

Action for Pulmonary Fibrosis has information for patients taking antifibrotic medications.

Immunosuppressant medicines 

You may need medicines that calm down your immune response and reduce inflammation. These may be oral corticosteroids like prednisolone or other immunosuppressant medicines.

Your specialist team may prescribe these if you have fibrosis caused by an inflammatory or autoimmune interstitial lung disease (ILD). 

Action for Pulmonary Fibrosis has more information about immunosuppressant therapies for pulmonary fibrosis. 

Oxygen therapy

Oxygen therapy is a therapy where you breathe in air with a higher concentration of oxygen in it. You may need oxygen therapy to use at home if tests show that the level of oxygen in your blood is too low. 

Some people only need oxygen when they’re active or doing exercise. But for others, blood oxygen levels may be low even when resting.

If your oxygen levels drop when you’re active, you may be offered ambulatory oxygen. This is when you have a small oxygen cylinder you can use while moving around or exercising. 

Oxygen therapy may:

• help you manage your breathlessness 
• improve oxygen levels in your blood
• make it easier for you to be more active 
• make it easier to take part in pulmonary rehabilitation classes
• help you do everyday activities.

Find out more about home oxygen therapy and how it is prescribed. 

See our patient guide for living with home oxygen. Action for Pulmonary Fibrosis also has information for patients on home oxygen, including advice on coping with the cost of using an oxygen concentrator.

Lung transplant

If you have progressive pulmonary fibrosis, your specialist team may talk to you about whether a lung transplant could be an option for you. 

They will consider:

• your general health, and if you’re well enough for a lung transplant 
• any other medical conditions you may have
• if you’re at a healthy weight for you, neither underweight, nor overweight.

If your specialist team thinks you may be suitable for a lung transplant, they’ll refer you to a transplant unit for an assessment. 

The transplant team will consider your lung condition, your general health and whether the benefits of a lung transplant are likely to outweigh the risks for you. If they think a transplant could be an option for you, they’ll add you to a waiting list. 

It’s hard to say how long you may need to wait for a suitable donor lung. The NHS has more information about waiting times for a lung transplant.

Action for Pulmonary Fibrosis has more information about lung transplants.

Being referred for a lung transplant assessment doesn’t mean you need a transplant straight away, or that you’re guaranteed one. Your specialist team may refer you early so there’s enough time for the transplant team to assess you and plan ahead if your condition changes. 

Leanne Hale, Respiratory Nurse Specialist, Asthma + Lung UK. 

Symptoms such as breathlessness, cough and feeling very tired (fatigue) can affect everyday life for people with pulmonary fibrosis. Your specialist team can help you find ways to manage your symptoms and stay as active as possible.

Breathing techniques

Breathing techniques help you control and slow your breathing. They help to manage breathlessness and to recover after being physically active. 

Different techniques work for different people. Your specialist team, respiratory physiotherapist or pulmonary rehabilitation team can help you find and practise techniques that work for you.

Find out more about managing breathlessness and download our Living with breathlessness booklet.

 We know that feeling breathless can be one of the most difficult symptoms for people living with pulmonary fibrosis. Your specialist team can help you find ways to manage your breathing and stay active, as well as supporting you with the worry or anxiety that breathlessness can sometimes cause. Always contact your GP or call 111 as soon as possible if your breathing gets worse.” 

Leanne Hale, Respiratory Nurse Specialist, Asthma + Lung UK. 

Pulmonary rehabilitation

Pulmonary rehabilitation (PR) classes include exercises and advice about staying active and managing symptoms like breathlessness. There’s also advice about emotional wellbeing and eating healthily.

Your specialist team may refer you to PR classes if breathlessness is making it harder for you to stay active or manage everyday activities. 

If your oxygen levels fall when you’re active, oxygen therapy can help you take part in pulmonary rehabilitation classes. 

If you’re waiting to start pulmonary rehabilitation, find out more about staying active with our Keep Active Programme 

Find out more about pulmonary rehabilitation.

Managing a cough

When you have pulmonary fibrosis, simple everyday activities like talking or moving position may make you cough. Constant coughing can affect the quality of your everyday life. 

Coughing a lot over a long period of time can irritate your throat and make you more sensitive to things that trigger your cough, leading to a cycle of coughing. 

Other conditions can also cause a cough or make it worse. Tell your specialist team about any other symptoms such as: 

• heartburn, indigestion or a sour taste at the back of your mouth (acid reflux)
• post-nasal drip – this is when extra mucus builds up and drips from your nose into your throat.
• respiratory infections, such as chest infections

Some studies suggest that antifibrotics can improve cough in some people. Treating a pulmonary fibrosis cough can sometimes be difficult, because the usual over-the-counter cough medicines may not work.

Get advice from your GP or specialist team if you’re coughing more than usual, or if your cough has changed or got worse

Action for Pulmonary Fibrosis have a factsheet on cough management with ideas for coping with a cough. 

Singing for Lung Health 

Singing for lung health classes have lots of physical and emotional benefits for people with lung conditions. 

Singing helps strengthen your voice and can improve breathing control. Singing as part of a group means you can share experiences about your lung condition. We know that singing for lung health groups can help some people feel less isolated. 

Find out more about Asthma + Lung UK’s support groups, including our Motivational Mondays singing group.

Going to regular reviews can help you make sure you’re getting the right treatment and support.

How often you have reviews will depend on the type of pulmonary fibrosis you have, how stable your condition is, your treatment, and your individual needs. 

Some types of pulmonary fibrosis may not need to be reviewed as often, because they can be quite stable. This includes fibrotic ILDs which are not progressive (non-progressive fibrotic ILDs). 

If you have progressive pulmonary fibrosis (PPF) or idiopathic pulmonary fibrosis (IPF), you may need to have more regular reviews.

At your review, your specialist team can:

• review your pulmonary fibrosis self-management plan
• talk about your medicines and treatments, and any side effects 
• talk about your symptoms, how you’re managing them, and if they’ve got worse
• support you with any other health conditions you have, including anxiety and depression, sleep apnoea, COPD or pulmonary hypertension
• check your lung function tests to see how your lungs are working and if your condition has changed
• do some tests to see if you may benefit from oxygen therapy
• see if you may benefit from pulmonary rehabilitation
• refer you to other services for support including palliative care
• make sure you’re having all the vaccines you’re eligible for including flu, COVID, RSV and shingles.
• support you to set simple goals for staying active and eating well.

You can use our pulmonary fibrosis care passport to check that you're getting the best pulmonary fibrosis care. It also gives you a list of things that you can ask your GP or healthcare professional at your next review or appointment.

Action for Pulmonary Fibrosis also has a useful list of questions you might want to ask your healthcare team. 

Take someone with you to your appointments if you can. It can be hard to remember everything that’s said sometimes, and everything you want to ask. Taking someone with you can make it all feel easier.’ 

Person living with pulmonary fibrosis.

Supportive or palliative care can help people living with serious health conditions, including pulmonary fibrosis. It can help you and your family, friends and carers from diagnosis onwards. 

Palliative care is not just end-of-life care. You can be referred to palliative care to help manage difficult symptoms and for emotional and practical support.

It focuses on helping you live as well as possible, helping with:

• information and advice to help you cope with your condition
• relief for symptoms
• managing other conditions 
• emotional support 
• decisions about your care and treatment
• supporting carers, family and friends.

Action for Pulmonary Fibrosis has more information about palliative care for pulmonary fibrosis. 

Clinical trials help find out whether new treatments are safe and how they will work. 

Taking part in a clinical trial gives you the chance to try a new treatment that is not widely available yet. It can also be rewarding because you know you’re doing something that could help others with pulmonary fibrosis in the future.

Find out more about getting involved in a trial. Action on Pulmonary Fibrosis also has information and FAQs about clinical trials.