10 min read
Sarcoidosis
Sarcoidosis is a systemic granulomatous disease of unknown cause. It can affect different organs, including lungs1
Sarcoidosis is a condition where lumps called granulomas develop at different sites within the body. Granulomas are made up of clusters of cells involved in inflammation. If many granulomas form in an organ, they can prevent that organ from working properly.2
Sarcoidosis can affect many different parts of the body, with the lungs most commonly affected, and can lead to the development of lung disease involving pulmonary fibrosis.1
What is sarcoidosis with pulmonary fibrosis?
Sarcoidosis affects only around 1-36 people in 100,000 each year, where 1 in 5 of these people develop interstitial lung disease (ILD).1
In this form of 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.3
Watch a short video about sarcoidosis involving the lungs.
Download our booklet for an overview about living with pulmonary fibrosis.
How can we support you?
On this page you can find information that may help if you:
Have sarcoidosis and want to know if you are at risk of developing pulmonary fibrosis in the future
Have pulmonary fibrosis and want to know if you could have sarcoidosis
Have been diagnosed with sarcoidosis with pulmonary fibrosis and want to know more about it
I have sarcoidosis… could I have sarcoidosis with pulmonary fibrosis or develop it in the future?
Know your risk for pulmonary fibrosis
It’s difficult to know how sarcoidosis will affect a person as it can affect any organ and symptoms can vary widely. If you feel concerned about your risk of pulmonary fibrosis, don’t delay asking your doctor for a lung examination. As pulmonary fibrosis can get worse over time, recognising it early is key to getting the help you need.4,5
Talk to your healthcare professional; they evaluate your lungs and monitor their function with various tests and scans. If you are unsure how to talk about sarcoidosis with pulmonary fibrosis, book some 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 pulmonary fibrosis?
What tests could I have to find out if I have pulmonary fibrosis?
I’m concerned I might have pulmonary fibrosis, can we look into this?
What can we do to monitor for pulmonary fibrosis?
Nine in ten people with sarcoidosis will experience lung symptoms6 and 1 in 5 will develop pulmonary fibrosis1 so it is important to talk to your doctor if you have any of the following symptoms:6
Symptoms of pulmonary fibrosis may include:7
Breathlessness
Dry cough
Abnormal sounds when you breathe
Chest pain
Extreme tiredness
Clubbing (widening and rounding at the end of your fingers or toes)
These symptoms are not specific to ILD or sarcoidosis with pulmonary fibrosis and may also be present in other autoimmune diseases, such as rheumatoid arthritis or lupus.6 Your doctor may need to perform several tests to find the cause of your symptoms.6
When looking for pulmonary fibrosis in sarcoidosis, 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.6
A physical examination is also used to give the healthcare professional a better understanding of your lung health. However, other tests may be needed to make a clear diagnosis.8,9
Read our ‘Diagnosing pulmonary fibrosis’ page for information about the tests that your doctor might request to check your lungs.
I was diagnosed with interstitial lung disease… could it be sarcoidosis?
Know your risk
ILD is an umbrella term that groups together more than 200 different disorders/diseases that lead to inflammation and scarring in the lungs.10 Sarcoidosis is a complicated disease that can affect multiple organ systems, in addition to the lungs.6 The exact cause of sarcoidosis is not known, although there are a few risk factors for sarcoidosis. Some factors, such as your work or living environment might have room for change, but there are other factors that cannot be changed, such as older age, family history and genetics, certain essential medications, race and female sex.11
If you’ve noticed any of these symptoms and they persist or are getting worse, check with your doctor to rule out whether they are caused by sarcoidosis:
Tender bumps on the skin
Shortness of breath
Persistent cough
Tender and swollen glands
Fatigue
Painful joints/pain in the bones
Red or sore eyes
Abnormal heart rhythm
Blocked or stuffy nose
Kidney stones
Headache
Depending on the individual, symptoms can appear suddenly and then go away, some people have sarcoidosis but don’t have any obvious symptoms and others may develop symptoms gradually and get worse over time.4 Due to the wide-ranging responses to sarcoidosis, it is always best to check with your doctor for reassurance and find out:
What is my risk for developing sarcoidosis?
Could my symptoms be related to sarcoidosis?
What can we do to test for sarcoidosis?
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.
Monitoring symptoms
I have sarcoidosis with pulmonary fibrosis, what does that mean?
The general management of pulmonary disease in sarcoidosis is relatively established and most people respond well to treatment.
Some people might experience very few symptoms while pulmonary fibrosis is occurring, which could delay people from seeking medical attention until the disease gets to a stage where it becomes more challenging to treat. The earlier you ask for a lung examination, get a diagnosis and commence treatment, the better chance you give yourself to slow or control sarcoidosis with pulmonary fibrosis. Your doctor and the specialist team will work together to agree the optimal treatment pathway with you. The aim of treatment is to improve or preserve lung function and your quality of life, for instance, by treating fatigue.12 Ask your doctor about your treatment options, including pulmonary rehabilitation, which could help you manage your condition.
For a small number of people, their symptoms and lung function might progress (get worse), or the HRCT scan might show the pulmonary fibrosis in the lungs has increased and is difficult to control.13
Currently there is no way of predicting if, or how fast progression 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.2,14 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
Talk to your doctor about treatment options for sarcoidosis with pulmonary fibrosis. 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
Sarcoidosis is a systemic granulomatous disease. Pulmonary fibrosis can occur in patients with sarcoidosis, which means that there is a build-up of scarring in the lungs. This makes it harder for oxygen to enter the blood resulting in shortness of breath
If you have risk factors for developing pulmonary fibrosis, or are experiencing respiratory symptoms –ask your doctor for a lung examination as soon as you can
The general management of pulmonary disease in sarcoidosis is relatively established and most people respond well to treatment
In a small number of people, sarcoidosis with pulmonary fibrosis may get worse over time; it is not clear why some people’s disease progresses faster than others. Keeping on top of your symptoms can help you and the healthcare team manage your condition
When looking for pulmonary fibrosis in patients with sarcoidosis, your treatment team may perform several tests to get a good understanding of your lung health
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.
Sarcoidosis UK. What is sarcoidosis? Available at: https://www.sarcoidosisuk.org/information-hub/what-is-sarcoidosis/ [Accessed July 2023].
Asthma + Lung UK. Pulmonary Fibrosis. Available at: https://www.asthmaandlung.org.uk/conditions/pulmonary-fibrosis [Accessed July 2023].
American Lung Association. Stages of pulmonary fibrosis. Available at: https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/introduction/stages-of-pulmonary-fibrosis [Accessed July 2023].
Richeldi L, Cottin V, Würtemberger G, et al. Digital lung auscultation: will early diagnosis of fibrotic interstitial lung disease become a reality? Am J Respir Crit Care Med.2014;200(2):262–263.
NHS. Sarcoidosis. Available at: https://www.nhs.uk/conditions/sarcoidosis/ [Accessed July 2023].
National Heart, Lung, and Blood Institute. Interstitial lung diseases. Symptoms. Available at: https://www.nhlbi.nih.gov/health/interstitial-lung-diseases/symptoms [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].
Sarcoidosis UK. Sarcoidosis and the lungs. Available at: https://www.sarcoidosisuk.org/information-hub/types-of-sarcoidosis/lung-sarcoidosis/ [Accessed July 2023].
Mueller-Mang C, Ringl H, Herold D. Interstitial lung diseases. Multislice CT. 2017;261–288.
National Heart, Lung, and Blood Institute. Interstitial lung diseases. Causes and risk factors. Available at: https://www.nhlbi.nih.gov/health/interstitial-lung-diseases/causes [Accessed July 2023].
Baughman RP, Valeyre D, Korsten P, et al. ERS clinical practice guidelines on treatment of sarcoidosis. Eur Respir J. 2021;58:2004079.
Weertunga P, Moller DR, Ho L-P. Immune mechanisms in fibrotic pulmonary sarcoidosis. Eur Respir Rev 2022;31:220178.
Criado E, Sánchez M, Ramírez J, et al. Pulmonary Sarcoidosis: Typical and Atypical Manifestations at High-Resolution CT with Pathologic Correlation. RadioGraphics. 2010;30(6):1567–1586.
Also in this section
Conditions with pulmonary fibrosis
Co-existing conditions
You may also be interested in
What is pulmonary fibrosis?
