What are interstitial lung diseases (ILDs)?

Interstitial lung disease (ILD) is the name for a group of conditions that damage the tissue in your lungs. 

There are more than 200 types of ILD. Some cause inflammation. Some cause scarring of the lungs. Others cause a mix of both inflammation and scarring. 

Scarring and thickening of lung tissue is known as pulmonary fibrosis.

An ILD usually affects the tissue in your lungs, rather than your airways (breathing tubes). This means your lungs cannot fill with as much air as normal. 

This is different from conditions like asthma and chronic obstructive pulmonary disease (COPD), which mainly affect the airways.

Who is most at risk of getting an ILD?

Your risk of getting an ILD increases if you:

• are over 50 years old, but they can happen at any age
• have been exposed to harmful chemicals and other substances such as mould and certain dusts
• smoke or have ever smoked
• have an autoimmune conditions
• have taken certain medicines
• have a family member with an ILD.

Idiopathic pulmonary fibrosis (IPF)

Idiopathic pulmonary fibrosis (IPF) is the most common type of ILD. Idiopathic means that no specific cause for the pulmonary fibrosis has been found.

IPF can be a progressive condition which means that the scarring on your lungs gradually gets worse over time. 

Around 1 in 20 people with IPF have another family member with the condition.

Children's interstitial lung disease (chILD)

Some people are born with an ILD or develop one in childhood or as a teenager. These are called children’s interstitial lung disease (chILD).

There are more than 200 types of chILD. You can find out more about the different types on the chILD Lung Foundation website.

Pulmonary sarcoidosis

Sarcoidosis happens when inflamed cells join together and make tiny lumps called granulomas. When this happens in your lungs it’s called pulmonary sarcoidosis.

Having a family member with sarcoidosis can increase your risk of developing the condition, but this is still rare.

Connective tissue disease related ILD (CT-ILD)

Connective tissue disease related interstitial lung diseases (CT-ILD) may happen when an autoimmune condition affects your lungs. 

For example, CT-ILD can happen in people with rheumatoid arthritis, systemic sclerosis (scleroderma) and Sjögren’s syndrome.

Drug-induced interstitial lung disease 

Drug-induced ILDs can happen when you have a rare side effect of medicines, such as certain antibiotics, chemotherapy (cancer) drugs and immunosuppressants.

Hypersensitivity pneumonitis

Hypersensitivity pneumonitis can happen when your immune system overreacts to something you breathe in, in large amounts, or breathe in often, from your environment. 

There are more than 200 substances that can cause hypersensitivity pneumonitis. These include certain dusts, moulds, animal and plant proteins, chemicals and metals.

Some types of hypersensitivity pneumonitis, such as farmer’s lung, can be caused by breathing in substances at work. These are called occupational ILDs.

Occupational interstitial lung disease 

Occupational ILDs are a group of lung conditions caused by breathing in certain harmful substances in the workplace. 

They can be triggered by asbestos, coal dust, silica dust and beryllium metal.  

Common symptoms of ILDs include:

• breathlessness that gets worse after movement or exercise
• a cough that does not go away
• feeling very tired (fatigue)
• losing weight for no reason.

You may have other signs and symptoms related to the type of ILD you have.

What is an ILD flare-up?

If your ILD symptoms suddenly get worse, you may be having a flare-up of your lung condition. Flare-ups are sometimes called exacerbations.

If you’re having a flare-up, one or more of your usual symptoms may get worse. For example:

• you feel more breathless
• your cough has changed or become worse
• you feel more tired than usual
• your oxygen levels are lower than usual, if you have been advised to monitor your levels at home by your specialist team.

If your symptoms are getting worse, speak to your specialist nurse or other healthcare professional for advice. 

Getting a diagnosis for an ILD can take time. 

This is because your GP and specialist team will want to find the cause of your symptoms and rule out other lung conditions, like asthma or chronic obstructive pulmonary disease (COPD). 

At your first appointment, your GP may ask you the following questions:

• What are your symptoms?
• How are these symptoms affecting your life?
• What medicines are you already taking and do they help?
• Does anyone in your family have an ILD?
• What medicines have you taken in the past?
• Do you smoke or have you ever smoked?
• Where have you worked?
• What are your hobbies?
• Do you have any pets?

Your GP may also listen to your chest for sounds which can be heard using a stethoscope.

Tests for diagnosing ILDs

If your GP thinks you may have an ILD, they will refer you to a specialist team for some more tests. 

A group of specialists (multidisciplinary team) will then work together to make a diagnosis and develop a plan to help you manage your condition. This may include ILD specialist nurses and doctors, respiratory physicians, radiologists, pathologists and other healthcare professionals with an expertise in ILDs.

You should have access to a specialist team if you have an ILD. Your team may include:

• ILD specialist nurses
• respiratory physiotherapists
• occupational therapists
• specialists like rheumatologists.

They will work with you to find the best treatment for your type of ILD. This will depend on whether you have inflammation, scarring of the lungs or a mix of both.

Some of the first steps are:

• working out what may be causing your symptoms. Once you know this, you can take steps to avoid it. This is most helpful for ILDs triggered by a specific substance such as occupational ILDs and hypersensitivity pneumonitis.
• stopping or changing medicines that may be causing scarring in your lungs, if you have a drug-induced ILD.
• treating autoimmune conditions, if you have a connective tissue-related ILD.

Treatments for inflammation

Medicines like corticosteroids (such as prednisolone) and other immunosuppressants (such as azathioprine) can improve inflammation by calming your body’s immune system.

These medicines are used to treat ILDs which mainly cause inflammation such as hypersensitivity pneumonitisand cryptogenic organising pneumonia (COP)., 

If you have pulmonary sarcoidosis or connective tissue related ILD (CT-ILD), you may be prescribed corticosteroids and another type of immunosuppressant to take together.

Treatment for lung scarring

Treatments can slow down how quickly you develop scarring of the lungs, but the scarring cannot be reversed. Medicines called antifibrotics, such as nintedanib and pirfenidone, are used to treat some people with IPF.

If you have a CT-ILD such as rheumatoid arthritis-ILD or systemic sclerosis-ILD, you may be prescribed an antifibrotic and immunosuppressant together. 

Lung transplant

In very rare cases where treatments are not helping, you may be assessed for a lung transplant. The NHS have more information about lung transplants and who can have them. 

Action for Pulmonary Fibrosis also run a support group for anyone with pulmonary fibrosis who has had or is waiting for a lung transplant.

Your ILD review

Most people will have a review of their condition at least once a year.

How often you see your specialist team for a review will depend on the stage of your condition and treatment. 

If you’re having treatment at a specialist centre or your condition is getting worse, you may see your specialist team every 3 to 4 months.

At your review, you can talk about your symptoms. You may also have lung function tests to see if your ILD is progressing and how well your treatments are working.

Tracking your ILD symptoms

Tracking your symptoms and going to your ILD check-ups can help you and your specialist team:

• recognise when your symptoms are getting worse, so you know when to seek urgent help
• understand how well your treatments are working
• help you answer any questions about your symptoms at your next appointment.

Your specialist team can talk to you about the best way to track your symptoms. This could be keeping a symptom diary, using a self-management plan or an app on your phone. 

Oxygen therapy

ILDs can cause low oxygen levels in your blood which can make you feel breathless.

If your oxygen levels are low, your specialist team may refer you for an assessment for home oxygen therapy. 

Pulmonary rehabilitation (PR)

Pulmonary rehabilitation (PR) is a treatment programme for people with lung conditions that cause breathlessness. 

It's made up of:

• support and advice to help you understand your condition
• exercises to help you feel stronger, walk further and have more energy
• breathing techniques
• lifestyle advice such as healthy eating
• physical and emotional support.

We have more information about PR, including how to get a referral. 

Your specialist team can give you support and advice about how to manage your ILD.

Stop smoking or vaping

If you smoke, stopping is one of the best things you can do for your health.

It’s easier to quit smoking with the right help. We have lots of advice and support to help you stop smoking, including information about vaping. 

Keeping active

Regular movement and exercise can help you to manage symptoms like breathlessness. It can also help to improve your mood.

Find out more about our Keep Active programme.

Getting your vaccinations

Getting your annual flu vaccine and one-off pneumonia vaccine will help to protect you from becoming seriously ill from an infection. If you’re aged over 75 years old or are 28 weeks or more pregnant, you are also eligible for the RSV vaccine.

Eating a healthy balanced diet

A well-balanced diet with the right vitamins and minerals can help you to stay well and keep your lungs healthy. If you need support with healthy eating or your weight, your GP might also be able to refer you to a dietitian.

We have more advice about living well with a lung condition, including advice about travel, sleep and weather and how they can affect your lungs. 

Managing breathlessness

Breathing techniques can help you feel more in control of your breathing and help you manage better when you’re out of breath. 

We have more information about breathlessness, including breathing positions and videos to help you manage your breathing.

Managing a cough

We know that coughing can make talking and keeping active more challenging.

Treatments cannot stop you from coughing completely, but there are things you can do to help you cough less. You could try:

• drinking water regularly throughout the day
• breathing in gently through your nose and out through your mouth
• avoiding anything that worsens your cough such as cold air, smoke and perfumes, if you can. You could try wrapping a loose scarf over your mouth and nose to warm the air you breathe in.

It’s hard to predict how your ILD will progress because it can depend on things like the type of ILD you have, how well you respond to treatment, your age and your lifestyle. 

Some ILDs can stay stable for years. This means that with the right management and treatment your condition does not get worse. 

Some ILDs are progressive which means they get worse over time, and they do not have a cure. In the later stages, this may be known as an advanced ILD or end-stage ILD.

End of life

If you have an end-stage ILD, palliative care can help you to be as well and comfortable as possible in the last months or years of your life. You can have palliative care at any stage of a life-limiting (terminal) illness.

"Palliative care is about helping people with advanced ILD live as well as possible for as long as possible. It focuses on improving quality of life by managing symptoms such as breathlessness, fatigue and cough, while also providing emotional support for both patients and those close to them." - Dr Matt Swallow, GP and Clinical Lead, Asthma + Lung UK

We have more support if you or someone you care for is nearing end of life.

What complications are caused by ILDs?

Some people develop complications from their ILD, particularly if the condition progresses. These may include:

• scarring of the lungs (pulmonary fibrosis)
• longer recovery from respiratory infections, such as chest infections, pneumonia and flu
• high blood pressure in the lungs (pulmonary hypertension). This is more common in ILDs that cause lung scarring.
• collapsed lung (pneumothorax). This can cause sudden breathlessness and chest pain.
• lung cancer
• respiratory failure - this is very rare and only happens in advanced ILDs.

Going to regular check-ups with your specialist team and starting the right treatment as soon as you have a diagnosis can improve inflammation and slow down the development of lung scarring.

It’s not always possible to know why complications happen and not everyone will have them. If you have any questions or concerns, contact your specialist ILD team.

We know from speaking to people with ILDs that living with a long-term lung condition can be challenging. There are various help and support networks available to you:

• talk to your GP or specialist team. They can signpost you to counselling and wellbeing services.
• join one of our free online or in-person support groups such as Singing for Lung Health and Harmonica for Lung Health
• join our Health Unlocked forum to connect with other people with a lung condition.
• call our friendly helpline. You can talk to use about any aspect of living with a lung condition.
• find out more about looking after your mental health and wellbeing when you have a lung condition.

If you care for someone with an ILD, get practical advice and emotional support. 

Is an ILD considered a disability?

Your ILD may be considered a disability under the Equality Act 2010 in Scotland, England and Wales, and the Disability Discrimination Act 1995 in Northern Ireland.

This means if you’ve been diagnosed with an ILD, you may be able to get financial support and benefits.

You can also ask your employer for any adjustments you may need at work.Citizens Advice have more advice and support on how to do this.