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Sjögren’s syndrome
Sjögren’s syndrome is a rare autoimmune disease that primarily affects the salivary and tear glands. It can also affect the lungs1,2
As Sjögren's syndrome is rare, it is often overlooked or misdiagnosed due to its common symptoms of dry mouth, dry eyes, fatigue, and chronic pain.3,4
Nine out of ten diagnosed people are women; however, Sjögren's can be diagnosed in all ages, races, and genders.4 Sjögren’s syndrome is classified either as primary (when the condition develops by itself), or secondary (when the condition develops in combination with another autoimmune disease such as rheumatoid arthritis, lupus or scleroderma).3
What is Sjögren’s syndrome with interstitial lung disease?
One in five people with primary Sjögren’s syndrome develop interstitial lung disease (ILD).5 In Sjögren's syndrome with ILD, scarring of the lung tissue might develop. This scar tissue is known as pulmonary fibrosis and affects the tiny air sacs and blood vessels responsible for providing oxygen to all parts of the body. As healthy lung tissue is replaced by scar tissue, it becomes harder for oxygen to pass through the lungs and into the bloodstream. This results in shortness of breath.6
Download our booklet for an overview about living with pulmonary fibrosis. You may also find our page ‘What is pulmonary fibrosis’ useful.
How can we support you?
On this page you can find information that may help if you:
Have Sjögren’s syndrome 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 Sjögren’s syndrome
Have been diagnosed with Sjögren’s syndrome with interstitial lung disease (ILD) and want to know more about it
I have Sjögren’s syndrome… could I have interstitial lung disease or develop it in the future?
Know your risk for interstitial lung disease
If you have Sjögren’s syndrome, you may be wondering if you could also have ILD, or be at risk of developing it, since one in five people with primary Sjögren’s syndrome develop ILD.5
If you are worried about your risk for ILD, ask your doctor for a lung examination. Current guidelines recommend that if your doctor suspects that you may have Sjögren’s syndrome with ILD, you should undergo lung function tests to help determine the health of your lungs and decide on the next steps.7
Read our ‘Diagnosing pulmonary fibrosis’ page for information about the tests that your doctor might request to check your lungs.
If you are unsure how to talk about ILD, make sure you have booked time to specifically discuss this topic so that you won’t feel rushed. 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?
Keep in mind that ILD can be progressive, which means it can worsen over time, so it’s important that your doctor checks your lung health regularly so that any changes can be managed quickly.
Most people don’t notice the changes to their lungs at first and may think they’re becoming unfit or tired. This can be true but it’s important to always be aware of your body’s capabilities and notice any changes.2,8
If you are concerned that ILD could be a complication of Sjögren’s syndrome, don’t delay speaking to your doctor as it could get worse.
The earlier lung involvement is spotted, the sooner you can be tested and get support from your doctor. They will be able to provide guidance for you about the best way moving forward.
There are a variety of symptoms you may experience with Sjögren’s syndrome with ILD and some of these can worsen as your disease progresses. Keep in mind that there are lifestyle changes and different treatments that can help you manage them. Symptoms can also vary from one person to another, so your symptoms may not be the same as somebody else who is living with pulmonary fibrosis.6
Symptoms may include:9
Feeling very tired when going about your normal day
Shortness of breath, especially with mild physical activity
Dry cough that doesn’t go away
These symptoms are not exclusive to ILD and can be found in other respiratory or cardiovascular diseases.
When diagnosing Sjögren’s syndrome 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, medication that you are taking, and weather you smoke or 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.10
High-resolution computerised tomography (HRCT) is very sensitive in detecting mild cases where you might not even notice symptoms.5
I have interstitial lung disease… could I have Sjögren’s syndrome?
Know your risk for Sjögren’s syndrome
Only 6 people per 100,000 each year develop Sjögren’s syndrome, with about 9-20% of these people developing ILD.2,11
Most people with ILD will not have Sjögren’s syndrome, since it’s a rare disease. However, if you have any concerns, it’s always best to book some time to discuss these with your doctor. The earlier you take action, the sooner you can get the right support from your healthcare team.7
It is always best to check with your doctor for reassurance and find out:
What is my risk for developing Sjögren’s syndrome?
Could my symptoms be related to Sjögren’s syndrome?
QUICK TIP
Keep a diary of your symptoms to track how you feel over time. Also, make a note of any questions and be sure to share your symptoms with your doctor. The sooner they know, the more they can help you
Monitoring symptoms
I have Sjögren’s syndrome with interstitial lung disease, what does that mean?
Your doctor and the specialist team will work together to agree the optimal treatment pathway with you. The earlier you start treatment, the better chance you give yourself to manage ILD, which can be a serious complication in Sjögren’s syndrome.2,7,8
Treatment depends on the symptoms and severity of your condition.7 Ask your doctor about your treatment options, including pulmonary rehabilitation, which could help you manage your condition.
Sjögren’s syndrome with ILD can be progressive, which means, over time, symptoms and how well your lungs work might get worse, or that an HRCT scan has detected that pulmonary fibrosis in the lungs has increased. However, currently there is no way of predicting if, or how fast, this will happen. Some people might not have changes in their symptoms, while others might experience a faster worsening of their symptoms. It is not known why some people’s disease progresses faster than others.2,12
It’s important that your doctor checks your lung health regularly so that any changes can be managed quickly.
You can get involved in in your care by:
Communicating promptly – let your healthcare professional know how treatment is going for you
Reporting new and existing symptoms – let your healthcare professional know if your symptoms are getting any better or worse
Getting to know your treatment options – ask your healthcare professional if they could be suitable for you
Reporting any adverse effects from treatment – let your healthcare professional know how it is affecting you
Attending review appointments to check that your treatment is effective
Keeping in contact with your nurse – nurses can support you when it comes to symptom management, lifestyle changes, and be the first point of contact if you have any questions
IMPORTANT
Talk to your doctor about treatment options for Sjögren's syndrome with ILD. They will be best able to provide guidance and support tailored to your individual situation and suggest the best way moving forward
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.
“Join a support group, if somebody puts out a hand for help, somebody will always hold it”
Key takeaways
Sjögren’s syndrome with ILD is a rare condition. In patients with primary Sjögren's syndrome, patients with ILD may develop lung scarring, making it harder for oxygen to enter the blood which results in shortness of breath
When looking for the correct diagnosis, your treatment team may perform several tests to get a good understanding of your lung health
If you have risk factors for developing ILD, or are experiencing respiratory symptoms –ask your doctor for a lung examination as soon as you can
Sjögren’s syndrome with ILD can be progressive but there is no way of knowing if, or how fast, this could happen
NHS. Sjögren’s syndrome: complications. Available at: https://www.nhs.uk/conditions/sjogrens-syndrome/ [Accessed July 2023].
Flament T, Bigot A, Chaigne B, et al. Pulmonary manifestations of Sjögren's syndrome. Eur Respir Rev. 2016;25(140):110–123.
NHS Inform. Sjögren’s syndrome. Available at: https://www.nhsinform.scot/illnesses-and-conditions/immune-system/sjogrens-syndrome [Accessed July 2023].
Sjögren's Foundation. Understanding Sjögren's. Available at: https://sjogrens.org/understanding-sjogrens/symptoms [Accessed July 2023].
Luppi F, Sebastiani M, Silva M, et al. Interstitial lung disease in Sjögren’s syndrome: a clinical review. Clin. Exp. Rheumatol. 2020;38 Suppl 126(4):291–300.
Asthma + Lung UK. What is pulmonary fibrosis. Available at: https://www.asthmaandlung.org.uk/conditions/pulmonary-fibrosis/what-pulmonary-fibrosis [Accessed July 2023].
Lee AS, Scofield RH, Morland Hammitt K, et al. Consensus Guidelines for Evaluation and Management of Pulmonary Disease in Sjögren’s. Chest. 2021;159(2):683–698.
Sogkas G, Hirsch S, Olsson KM, et al. Lung involvement in primary Sjögren’s syndrome – an under-diagnosed entity. Front. Med. 2020;7:332.
Asthma + Lung UK. What are the symptoms of pulmonary fibrosis? Available at: https://www.asthmaandlung.org.uk/conditions/pulmonary-fibrosis/what-are-symptoms-pulmonary-fibrosis [Accessed July 2023].
Asthma + Lung UK. How is pulmonary fibrosis diagnosed? Available at: https://www.asthmaandlung.org.uk/conditions/pulmonary-fibrosis/how-pulmonary-fibrosis-diagnosed [Accessed July 2023].
Weng M, Huang Y, Liu M, Lu T. Incidence and Mortality of Treated Primary Sjögren’s Syndrome in Taiwan: A Population-based Study. J Rheumatol. 2011;38(4):706–708.
Asthma + Lung UK. How is pulmonary fibrosis diagnosed? Connective tissue and autoimmune diseases. Available at: https://www.asthmaandlung.org.uk/conditions/pulmonary-fibrosis/connective-tissue-autoimmune-diseases [Accessed July 2023].
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Conditions with pulmonary fibrosis
Co-existing conditions
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What is pulmonary fibrosis?
