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Scleroderma with interstitial lung disease
Systemic sclerosis (also called scleroderma) is a rare multisystem autoimmune disease that affects the skin and internal organs, including lungs, which can also lead to interstitial lung disease (ILD)1
What is scleroderma with interstitial lung disease (scleroderma with ILD)?
Having an autoimmune disease such as scleroderma means that the body attacks and damages its own cells. There are two main types of scleroderma: localised and systemic. Generally speaking, localised scleroderma is limited to different kinds of skin changes and does not have internal organ involvement. Systemic sclerosis affects organs throughout the body in addition to skin changes and is the focus of this page.2
Scleroderma is a rare condition that affects 0.6-2.3 and 1.4-5.6 people per 100,000 each year in Europe and North America, respectively.3 Lung involvement by scleroderma is very common, either involving the blood vessels in the lung or the supporting structures (interstitium) of the lung leading to interstitial lung disease (ILD).4
ILD can be associated with scarring in the lungs. This lung (pulmonary) scarring (fibrosis), occurs between the oxygen containing sacs in the lung and the blood vessels that carry oxygen to the rest of the body. The scarring pulls on the airways in the lung causing cough, which is often the first symptom, and makes it harder for oxygen to get into the body, causing shortness of breath.5,6
Watch a short, informative video about scleroderma and the lungs.
How can we support you?
On this page you can find information that may help if you:
Have scleroderma and want to know if you could have or may develop interstitial lung disease (ILD) in the future
Have interstitial lung disease (ILD) and want to know if you could have scleroderma
Have been diagnosed with scleroderma with interstitial lung disease (ILD) and want to know more about it
I have scleroderma… could I have scleroderma with interstitial lung disease or develop it in the future?
Know your risk for interstitial lung disease
As many as 90% of people with scleroderma will have some changes in their lungs,7 but not everyone will develop pulmonary fibrosis (scarring). The risk of developing ILD is greatest early in the disease8 and could be related to the form of scleroderma (diffuse, cutaneous type of scleroderma), while older age and lung capacity could influence whether and how fast the ILD worsens over time.9 For this reason, some experts recommend screening for ILD in the first few years after diagnosis and that people whose ILD has a high risk of worsening quickly should be identified as early as possible.8,10
If you have scleroderma and are worried about your risk for ILD, ask your doctor for a lung examination. They might decide to check your lungs using HRCT (high-resolution computed tomography; an imaging method to look at your lung tissue) and/or Pulmonary Function Test (spirometry).8,10 ILD can worsen over time, so it’s important to check the lungs regularly.
Could I have scleroderma with interstitial lung disease?
The earlier lung involvement is spotted, the sooner you can be tested and get the right treatment for you.
Most people don’t notice the changes to their lungs at first and may think they’re unfit or tired.11 This can be true but it’s important to always be aware of your body’s capabilities and notice any changes.
There are a variety of symptoms you may experience with scleroderma with ILD and some of these will worsen as your disease progresses. Symptoms can also vary from one person to another, so your symptoms may not be the same as somebody else who has scleroderma with ILD.
Symptoms of scleroderma associated ILD may include:12
Chronic coughing that is usually dry. Worsening cough is often the first sign of ILD in scleroderma
As the ILD worsens, shortness of breath develops, first with moderate activity but as things progress this may occur even with your normal daily activities and can be associated with feeling very tired with less energy.
Although scleroderma itself is a rare condition, your doctor is likely prepared for ILD as a commonly-related condition, as sophisticated testing can find evidence of ILD in almost all people with the disease.7 If you are worried about your risk, tell your doctor about your concerns and ask for a lung examination. You might like to start the conversation with:
Could I be at risk of having ILD?
What tests could I have to find out if I have ILD?
I’m concerned I might have ILD, should we look into this?
What can we do to monitor for ILD?
“I first noticed it at work – I got out of breath if I walked up the stairs”
Monitoring symptoms
When diagnosing scleroderma with ILD, your treatment team will look at many areas of your health. For example, they will ask you about your work history, family medical history, any potential drug and medication use and whether you have ever smoked.
A physical examination is also used to give the doctor a better understanding of your lung health. However, other tests may be needed to make a clear diagnosis.5 Most commonly these will include a CT or HRCT scan and breathing (Pulmonary Function) tests.
To read more about the tests involved in diagnosis ILD, visit ‘Diagnosing pulmonary fibrosis’, which explains the tests.
I have interstitial lung disease… could I have scleroderma?
Know your risk for scleroderma
Scleroderma in itself is a rare condition. Although the cause is unknown, it is thought to involve genetics, the environment, your immune system, and hormones.13 When a person is diagnosed with ILD, their doctor will do blood tests and a clinical examination to look for possible causes, such as underlying autoimmune disease, including scleroderma. If ILD signs are coupled with thickening or tightening of the skin on the hands (puffy fingers), change in colour and pain in fingers and toes with cold or stress (Raynaud's), or gastric reflux and heartburn, these might be a clue that scleroderma is present.14
Symptoms can vary from person to person and with the type of scleroderma, but early signs of scleroderma are:15
An easy way to remember the symptoms is by using the acronym SoRaRe. The symptoms shown above are often the first clue15-17 (although other symptoms may also be present), so be sure to tell your doctor if you experience two or more of these together.
If you are concerned that you may have, or be at risk for scleroderma, ask your doctor:
What is my risk for developing scleroderma?
Could my symptoms be related to scleroderma?
What can we do to test for scleroderma?
I have scleroderma with interstitial lung disease, what does that mean?
Scleroderma with ILD can progress, which means over time, symptoms and how well your lungs work might get worse, or that pulmonary fibrosis in the lungs has increased, which can be detected by a HRCT scan.18
Your symptoms could limit physical activity, as well as your ability to perform basic everyday tasks.
Download our booklet for an overview about living with pulmonary fibrosis.
Your nurse would be a helpful source of support when it comes to symptom management, lifestyle changes, and be the first point of contact if you have any questions.
Keep in mind that there are lifestyle changes and different treatments that can help you manage your condition. These include smoking cessation and treating heartburn if present, as both of these factors worsen scleroderma ILD.
However, there is no way of predicting if, or how fast, your condition will develop. Some patients might not have changes in their symptoms, while other patients might experience a faster worsening of their symptoms. It is not known why some people’s disease progresses faster than others.19
Talk to your doctor about treatment options for scleroderma with ILD. They will be best able to provide guidance and support tailored to your individual situation and suggest the best way moving forward
You can get involved in managing your condition by:
Communicating promptly – let your doctor know how the treatment is going for you, to ensure you are on the right track to prevent disease progression
Reporting new and existing symptoms – let your doctor know if your symptoms are getting any better or worse
Reporting any adverse effects from treatment – let your doctor know how it is affecting you
Attending review appointments to monitor your response to treatment
Your mental wellbeing
Your wellbeing as a carer
Some days may be tougher than others. Regardless of how you’re feeling we can point you in the direction of help.
Other scleroderma life hacks
If you have scleroderma, daily life can be challenging, both practically and mentally. We can help you with a few easy life hacks helping you get on top of your mental health, holiday preparation and childcare.
Cold hacks
stories ∙ 2.15
The cold weather can be challenging if you have scleroderma. But watch Annelise explain some tips that can make your day easier, including a hand warming trick using rice, a sock and a microwave.
Kitchen hacks
stories ∙ 1.48
Scleroderma can mean a few challenges in the kitchen.... Edith gives us a few handy tips that help her, including how to use hair rollers to help grip cutlery.
Mental health hacks
stories ∙ 3.34
Nathan is a counsellor, working in partnership with people living with chronic disease. He explains how to express any difficult feelings you have and ways to help yourself out of a low mood.
Holiday hacks
stories ∙ 2.56
Preparing for a big family gathering can be tiring. Watch to see how Joyce does it, saving precious energy and overcoming dexterity issues to wrap beautiful gifts.
Childcare hacks
stories ∙ 2.02
Looking after a family can be even more of a challenge with Scleroderma, but Natalie shows how a little creative thinking can help with everything from changing diapers to getting the kids out of the door with less fuss.
Key takeaways
Scleroderma (systemic sclerosis) is a rare condition. ILD can occur in patients with scleroderma, which means that the lung tissue might get scarred, making it harder for oxygen to enter the blood resulting in shortness of breath
If you have scleroderma, or are experiencing respiratory symptoms –ask your doctor for a lung examination as soon as you can
When looking for the correct diagnosis, your treatment team may perform several tests to get a good understanding of your lung health
Scleroderma with ILD can get worse over time but it is not known why some people’s disease progresses faster than others. Ask your doctor what treatments are available to help you
Hudson M, Lo E, Lu Y, et al. Cigarette smoking in patients with systemic sclerosis. Arthritis Rheum. 2011;63(1):230–238.
The Scleroderma Education Project. Scleroderma Overview. Available at: http://sclerodermainfo.org/guide/overview/ [Accessed July 2023].
Bergamasco A, Hartmann N, Wallace L, Verpillat P. Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease. Clin Epidemiol. 2019;11:257–273.
Olson A, Gifford A, Inase N, et al. The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype. Eur Respir Rev. 2018;27(150):180077.
Asthma + Lung UK. Pulmonary Fibrosis. Available at: https://www.asthmaandlung.org.uk/conditions/pulmonary-fibrosis [Accessed July 2023].
Cappelli S, Bellando Randone S, et al. Interstitial lung disease in systemic sclerosis: where do we stand? Eur Respir Rev. 2015;24(137):411–419.
Solomon JJ, Olson AL, Fischer A, et al. European Respiratory Update. Scleroderma lung disease. Eur Respir Rev. 2013;22(127):6–19.
Rahaghi FF, Hsu VM, Kaner RJ, et al. Expert consensus on the management of systemic sclerosis-associated interstitial lung disease. Respir Res. 2023;24(1):6.
Schoenfeld SR and Castelino FV. Interstitial Lung Disease in Scleroderma. Rheum Dis Clin North Am. 2015;41(2):237–248.
Khanna D, Distler O, Cottin V, et al. Diagnosis and monitoring of systemic sclerosis-associated interstitial lung disease using high-resolution computed tomography. J Scleroderma Relat Disord. 2022;7(3):168–178.
Saketkoo LA, Scholand MB, Lammi MR, Russel AM. Patient-reported outcome measures in systemic sclerosis-related interstitial lung disease for clinical practice and clinical trials. J Scleroderma Relat Disord. 2020;5(2 Suppl):48–60.
NHS. Scleroderma. Available from: https://www.nhs.uk/conditions/scleroderma/ [Accessed September 2023].
National Institute of Arthritis and Musculoskeletal and Skin Diseases. Scleroderma. Available at: https://www.niams.nih.gov/health-topics/scleroderma [Accessed July 2023].
Pulmonary Fibrosis Foundation. Scleroderma-associated interstitial lung disease (SSc-ILD). Available at: https://www.pulmonaryfibrosis.org/docs/default-source/programs/educational-materials/fact-sheets-english/pf-fact-sheet---ssc-ild.pdf [Accessed July 2023].
Scleroderma & Raynaud’s UK. The link between Raynaud's and scleroderma. Available at: https://www.sruk.co.uk/raynauds/link-between-raynauds-and-scleroderma/ [Accessed July 2023].
Minier T, Guiducci S, Bellando-Randone S, et al. Preliminary analysis of the very early diagnosis of systemic sclerosis (VEDOSS) EUSTAR multicentre study: evidence for puffy fingers as a pivotal sign for suspicion of systemic sclerosis. Ann Rheum Dis. 2014;73(12):2087–2093.
Allanore Y, Simms R, Distler O, et al. Systemic sclerosis. Nat Rev Dis Primers. 2015;1:15002.
Cottin V, Hirani NA, Hotchkin DL, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180076.
Chowaniec M, Skoczyńska M, Sokolik R, Wiland P. Interstitial lung disease in systemic sclerosis: challenges in early diagnosis and management. Reumatologia. 2018;56(4):249–254.
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