Connective tissue related interstitial lung diseases

Connective tissue related interstitial lung diseases are a group of lung conditions that can happen in people with autoimmune conditions that affect the lungs, such as rheumatoid arthritis, Sjögren disease and systemic sclerosis.

Our immune system fights and protects us against germs, like bacteria and viruses, and harmful substances.

If you have an autoimmune condition, the immune system can mistakenly attack healthy tissue in the body, including the tissue that supports your lungs.

For some people, this can lead to inflammation in the tiny air sacs (alveoli) and small airways in your lungs. An interstitial lung disease (ILD) that happens because of an autoimmune condition may be called a connective tissue related interstitial lung disease (CT-ILD).

Non-urgent advice: What is an interstitial lung disease (ILD)?

An ILD is a lung condition that affects the tissues that support the tiny air sacs (alveoli) in your lungs. This group of tissue is called the interstitium.

Find out more about how ILDs like CT-ILD can affect your lungs and airways.

What autoimmune conditions cause CT-ILDs?

Autoimmune conditions that may cause an CT-ILD include:

What are the symptoms of CT-ILDs?

Common symptoms include:

  • a chronic (persistent) cough

  • breathlessness, especially after exercise

  • feeling very tired (fatigue)

  • a sharp pain when breathing, coughing or sneezing. This can happen in some types of CT-ILD, such as rheumatoid arthritis ILD. 

Tell your GP if you notice any new or worsening symptoms, especially if you have an autoimmune connective tissue disease. 

Non-urgent advice: CT-ILD affects everyone differently

When you have a CT-ILD, you may also have symptoms that are caused by your autoimmune condition. These symptoms will vary depending on which condition you have.  

Action for Pulmonary Fibrosis has more information about the symptoms of rheumatoid arthritis ILD and scleroderma

How are CT-ILDs diagnosed?

It can take time to diagnose a CT-ILD because the symptoms are often similar to other lung conditions.

Your GP will ask you about your symptoms and when you get them. They’ll also ask about your medical history and any family history of lung and autoimmune conditions. 

They may refer you for tests, including:

If they think you may have an ILD, they will refer you to a respiratory specialist. Your diagnosis will be confirmed by a team of experts, called a multi-disciplinary team.

How are CT-ILDs treated?

Your GP will refer you to different specialists who can help you find the cause and manage your condition. These include: 

  • a rheumatology team to monitor your autoimmune condition

  • a specialist ILD team to monitor your lungs, including a respiratory specialist.

Some hospitals have a combined clinic for people who have an autoimmune condition and a lung condition.

Once your specialist has found the cause of your condition, they can treat your connective tissue and autoimmune condition. This may help to protect your lungs from getting more inflamed and slow the progression of the CT-ILD.

Most CT-ILDs are treated with a corticosteroid tablet called prednisolone. Corticosteroids are copies of the steroids that your body makes naturally. 

They may prescribe another type of immunosuppressant medicine such as azathioprine, mycophenolate mofetil or methotrexate. Immunosuppressants help to control your body’s immune response.

Important: Risks and benefits of your medication

Before starting any medication, talk to your GP about the risks and benefits, and any side effects you’re worried about.

Very rarely, some medicines which treat autoimmune conditions can cause lung scarring. This is called a drug-induced ILD. 

Most drug-induced ILDs do not get worse once you stop taking the medicine that is causing your symptoms. Talk to your GP before stopping any medication you’ve been prescribed. 

How are CT-ILDs managed?

Most people will have a review at least once a year. If you’re having treatment at a specialist hospital, you may be seen every 3 to 4 months until your condition is stable.

Your specialist will review your symptoms and do some lung function tests to see how well your treatments are working.

If you’re breathless, your specialist may refer you for pulmonary rehabilitation (PR). You may also be prescribed home oxygen therapy if tests show you have low oxygen levels in your blood.

Your specialist can support you to make changes to your lifestyle to improve your symptoms, such as eating healthily and stopping smoking.

H3 What’s the outlook for CT-ILD? 

The progression of a CT-ILD can depend on:

  • the type of autoimmune condition you have

  • the severity of your autoimmune condition

  • how the autoimmune condition affects your lungs

  • your general health, such as if you smoke or have any other medical conditions. 

Early diagnosis and treatment may help reduce inflammation and lower your risk of progressive lung scarring, called pulmonary fibrosis.  

Help and support for CT-ILDs

Meeting other people who are living with lung conditions, who relate with your experiences, can help you build a supportive network.

  • Join one of our online communities on Health Unlocked forum to connect with other people with similar experiences to you.

  • Find support in your area, including online and face to face support groups, mobility and wellbeing sessions, and singing for lung health sessions.

  • Get in touch with our friendly helpline to talk about any aspects of your lung condition. We’ll give you as much time as you need to talk about what’s important to you.  

Action for Pulmonary Fibrosis has information about support groups for anyone living with and affected by pulmonary fibrosis, including friends, family and carers.   

Arthritis UK has information and support on arthritis conditions such as rheumatoid arthritis, Sjögren disease and systemic sclerosis (scleroderma).This covers things like living with arthritis and friendly online communities. 

How we develop our health information

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About our health information

Learn more about how we make sure our health information is accurate, up-to-date and easy to use.

All our information is reviewed and updated by Asthma + Lung UK’s experienced Health Advice Team. People living with lung conditions, qualified healthcare professionals and specialists also review our content.

This information has been clinically reviewed and approved by Asthma + Lung UK’s Clinical Leads, Dr Matthew Swallow, a practising GP, and Nazir Hussain, a specialist pharmacist, who both share a passion for improving respiratory care.

Our information is based on the latest, trustworthy evidence and guidelines. Below is a sample list of the sources referenced in our connective tissue related interstitial lung disease (CT-ILD) information.

  • KM Antoniou, O Distler, AM Gheorghiu et al. ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease. European Respiratory Journal. 2026
  • Wells, AU & Hirani N. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax. 2008.
  • YH Khor, CC Moor, W Merkt et al. Treating connective tissue disease-associated interstitial lung disease -think outside the box: a perspective. European Respiratory Review. 2025 

If you'd like a full list of the sources we've used to produce our health information, you can email us at healthadvicequeries@asthmaandlung.org.uk

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Call or email our helpline for support with any aspect of living with a lung condition – whether you need practical advice, emotional support, or answers to health-related questions. You can also find support through our groups.

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