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Conditions with pulmonary fibrosis

Scleroderma-associated ILD

Scleroderma (also called systemic sclerosis, SSc) is a rare multisystem autoimmune disease that affects the skin and internal organs, including lungs, which can also lead to interstitial lung disease (ILD) or pulmonary fibrosis.1

Lung involvement in scleroderma-associated ILD is very common, either in the blood vessels in the lungs or the supporting structures (interstitium) of the lungs. This is known as ILD.2,3

Watch the video to below to learn more about scleroderma-associated ILD and pulmonary fibrosis.

Symptoms of scleroderma-associated ILD

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.4 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-associated 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-associated ILD.

Cough puff
Dry cough5

Chronic coughing that is usually dry and gets worse (typically the first sign of scleroderma-associated ILD)

Shortness of breath
Shortness of breath5

First with moderate activity but becoming more noticeable as ILD worsens, eventually impacting daily activities and leading to tiredness and loss of energy

Diagnosing scleroderma-associated ILD

When diagnosing scleroderma-associated ILD, your treatment team will:

Medical History
Ask about your work history, family medical history, any potential drug and medication use, and whether or not you have ever smoked6
stethoscope
Do a physical examination to assess your lung health7
Scan
Perform other tests such as a computed tomography (CT) scan or high-resolution computed tomography (HRCT) scan and breathing (pulmonary function) tests7

Risk factors for ILD

Although scleroderma-associated ILD itself is a rare condition, your doctor is likely prepared for ILD as a commonly related condition.8

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.7 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.4

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; an imaging method to look at your lung tissue and/or pulmonary function tests, including a spirometry test.7,8 ILD can worsen over time, so it’s important to check the lungs regularly.

Risk factors for ILD

Symptoms can vary from person to person and with the type of scleroderma, but early signs of scleroderma are9:
Swollen Finger
Sore, swollen fingers
Heartburn
Acid reflux and heartburn
Hand
Reynaud’s phenomenon

The symptoms shown above are often the first clue (although other symptoms may also be present), so be sure to tell your doctor if you experience 2 or more of these together.9

Keeping a diary of your symptoms may help a conversation with your doctor if you're concerned. Download our symptom tracker.

Monitoring for progression of scleroderma-associated ILD

Scleroderma-associated 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 an HRCT scan.10

However, there is no way of predicting if, or how fast, your condition will develop. Some patients might not have any 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.11

Talk to your doctor about treatment options for scleroderma-associated ILD. They will be best able to provide guidance and support tailored to your individual situation and suggest the best way moving forward.

Managing life with scleroderma-associated ILD

Receiving a diagnosis of scleroderma-associated ILD can feel lonely and uncertain; at these times it can feel very difficult to stay positive. Daily life can be challenging, both practically and emotionally. However, there are many strategies to help people living with pulmonary fibrosis.

Managing life with scleroderma-associated ILD
Living with scleroderma-associated ILD brings its own special challenges.
Check out our series of life hacks to help you manage everyday tasks.

Explore treatment options for people with 
Scleroderma-associated interstitial lung disease.

Learn more

Abbreviations: CT, computed tomography; GERD, Gastro-oesophageal reflux disease; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; PF, pulmonary fibrosis; RA, Rheumatoid arthritis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; SSc, systemic sclerosis.

  1. Hudson M, Lo E, Lu Y, et al. Cigarette smoking in patients with systemic sclerosis. Arthritis Rheum. 2011;63(1):230-238.

  2. Olson AL, Gifford AH, Inase N, Fernández Pérez ER, Suda T. The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype. Eur Respir Rev. 2018;27(50):180077.

  3. Pulmonary Fibrosis Foundation. Scleroderma-associated interstitial lung disease (SSc-ILD). Updated August 2023. Accessed October 2, 2024. https://www.pulmonaryfibrosis.org/docs/default-source/programs/educational-materials/fact-sheets-english/pf-fact-sheet---ssc-ild.pdf 

  4. 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.

  5. Cappelli S, Bellando Randone S, Camiciottoli G, De Paulis A, Guiducci S, Matucci-Cerinic M. Interstitial lung disease in systemic sclerosis: where do we stand? 
Eur Respir Rev. 2015;24(137):411-419.

  6. National Institute of Arthritis and Musculoskeletal and Skin Diseases. Scleroderma. Updated September 2023. Accessed October 2, 2024. https://www.niams.nih.gov/health-topics/scleroderma 

  7. Fairley JL, Goh NS, Nikpour M. Systemic sclerosis-associated interstitial lung disease: Diagnostic approaches and challenges. Reumatologia. 2024;31(1):S15-S25.

  8. Solomon JJ, Olson AL, Fischer A, Bull T, Brown KK, Raghu G. Scleroderma lung disease. Eur Respir Rev. 2013;22(127):6-19.

  9. Scleroderma & Raynaud’s UK. The link between Raynaud's and scleroderma. Accessed October 2, 2024. https://www.sruk.co.uk/raynauds/link-between-raynauds-and-scleroderma/ 

  10. 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.

  11. 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.