Lung problems in scleroderma
Many people with scleroderma (also known as systemic sclerosis) have some form of changes to their lungs. However, not everybody gets noticeable changes.1-5
There are two key ways in which scleroderma may affect your lungs:1, 6
Lung fibrosis (also known as interstitial lung disease [or ILD]) – when the lungs become stiffer and thicker
Pulmonary arterial hypertension (or PAH) – when the pressure in the blood vessels in the lungs gets too high
It is possible to get one, or even both, of these conditions. Or you may get neither.
Breathlessness is common in scleroderma, which can be worrying, but it is not always serious and does not necessarily mean you have lung fibrosis or PAH. However, make sure you tell your doctor so they can check your lungs.
Other phrases you might hear that describe problems with your lungs:
Interstitial lung disease (or ILD)
Pulmonary arterial hypertension (or PAH)
Most people don’t notice the changes to their lungs at first. They just think they’re unfit or tired. In some cases, this may be true. But you know your own body and what you’re capable of.
Try to be aware of any little changes that could suggest a lung problem. The earlier it gets spotted, the sooner you can be tested and get some expert support.
It is important to find out whether your scleroderma is affecting your lungs as soon as possible so that your doctor can work out whether you need treatment and, if so, the best treatment for you.
Understanding lung fibrosis (interstitial lung disease)
How many people get it?
Lung fibrosis affects most people with scleroderma to some extent, but only between 13 and 16% of people will experience severe symptoms.1-4, 7, 8
The lungs can become stiff and thicker. In some people, this can stop the lungs working as well as they should.
It happens because the fibrosis and scarring affect the little air sacs in the lungs (the alveoli), making them stiff and less able to inflate and deflate. These little air sacs are responsible for transferring oxygen from the air in the lungs into the blood and removing carbon dioxide from the blood to be breathed out. The scarring and stiffness mean that they can’t do this job effectively.9
Some people with lung fibrosis have mild respiratory symptoms that they may only notice when they really exert themselves. Others might experience more severe shortness of breath that affects their daily activities.2, 10
Symptoms could include:6, 10
Feeling very tired when going about your normal day
Getting out of breath doing simple tasks like climbing stairs
Dry cough that you can’t get rid of
Feeling like your chest is tight
What could lung fibrosis mean for me?
Some, but not all, people with scleroderma-related lung problems will get changes they notice and that affect their everyday life.10
For most people with scleroderma and lung problems, their condition remains relatively stable, or declines slowly over many years:5
7 in 10 people report some shortness of breath
6 in 10 people report difficulty climbing stairs or walking
A small number of people will have symptoms that get worse quickly after they are diagnosed:
Up to half of people who will be affected by lung problems develop symptoms within 3 years of having scleroderma2, 4, 11, 12
For most people with scleroderma and lung problems, their condition remains relatively stable, or declines slowly over many years5
Over time, 1 in 10 people with scleroderma might develop serious lung symptoms that affect how they go about their life. In very severe cases, these symptoms can affect how long they live.2, 10, 13
I noticed I was getting tired a lot. I stopped climbing the stairs. Now I use the elevator.
Person with scleroderma
Understanding pulmonary arterial hypertension (or PAH)
How many people get it?
It is a rare complication, affecting between 5 and 12% of people with scleroderma, and increasing in risk with time.14
Pulmonary arterial hypertension happens because fibrosis (scarring) narrows the small blood vessels in the lungs.
This causes the blood pressure to rise in the pulmonary artery (the big blood vessel that carries blood to the lungs), because it is trying to force blood through narrower blood vessels. The result is that less blood gets to the lungs, so less oxygen can get into the blood through the lungs. The heart tries to fix the problem by making the right side of the heart work much harder.6, 15
Symptoms could include:15
Shortness of breath on climbing stairs or hills
Your doctor will be monitoring you regularly to check on the health of your heart and lungs. If you do develop pulmonary arterial hypertension, your doctor will help you manage it and will recommend a treatment that suits you.
Testing your lung health
Lung function is tested regularly in people with scleroderma. It helps to identify the early signs of lung problems and to track the progress of lung damage.2, 16
Lung problems can be a scary thought, but there are lots of things you can do to look out for the signs. Tell your doctor if you have any symptoms. They can see what’s going on and help you. The sooner they know, the more they can help you.
But it’s not just up to you to notice any changes. Specialist doctors across a number of different countries recommend that lung function should be tested regularly, and at least annually, in people with scleroderma.16-21
One way your doctor will monitor your lung health is by listening to your lungs. Other tests to check your lung health will use equipment that you may not be familiar with.
These tests can have long and complex names and it can make them sound a bit frightening or painful, but most of these tests are very simple. Your doctor will help to prepare you and can explain everything before you start.
These are a few tests that you might be asked to take:
Lung function tests
Lung function is tested regularly in people with scleroderma. It helps to identify the early signs of lung problems and to track the progress of existing lung damage.17-23
The tests will measure things to do with the air you breathe in and out - your breathing and how your lungs are working. The results can be compared against what would be expected for someone of your age, height, race and sex. They can also be compared with your previous measurements. Then your progress will be tracked over time.
There are two main tests the doctor/nurse will be interested in:6, 24
Looking at: How much air is going into your lungs and how quickly air is inhaled and exhaled. The measurements will be made by a machine called a spirometer.
What to expect: You will be required to breathe into a tube in different ways. This will enable the doctor or nurse to take measurements, such as:
- Forced vital capacity (FVC) - The amount of air that can be breathed out in one breath (breathing ability). Lung fibrosis (scarring) can reduce the capacity and flexibility of your lungs, and can change how you breathe
- Forced expiratory volume in 1 second (FEV1) - the amount of air that can be blown out quickly, in 1 second. This can be reduced if your lungs are stiff
2. Diffusing capacity of the lungs for carbon monoxide (DLCO)
Looking at: How much of the air you breathe in is exchanged with your blood (gas exchange).
If the tiny air sacs (alveoli) in your lungs are inflamed or thickened by fibrosis, gases won’t be able to go in/out of the blood so easily. Gas exchange is also affected if there are problems with the blood vessels in your lungs, like with PAH.
What to expect: DLCO is calculated by using a carbon monoxide (CO) diffusion capacity test. The doctor/nurse will ask you to breathe in a small amount of air. You then hold your breath for 10 seconds before rapidly blowing the air out.
This tells the doctor/nurse how much of the air you breathe in is exchanged with your blood. It gives a percentage that can help to predict problems in the future.
Lung volume tests
Looking at: How much air your lungs can hold. Also, how much air stays in your lungs when you breathe out fully. Fibrosis stiffens your lungs, so they won’t be able to expand as much and the volume might be smaller.
What to expect: In a test called body plethysmography, you will be asked to sit in a small, see-through booth, a bit like a phone booth. You then need to breathe against a mouthpiece.
In a test called the helium dilution test, you will be asked to breathe helium in through a tube, then blow it out.
Learning to pronounce it is probably the hardest bit of this test – ‘Pleth – is – mog – graph – ee.’ For the rest of it, you just sit in the booth and breathe.
Person with scleroderma
Looking at: The general health of the lungs.
What to expect: The X-ray creates a picture of your lungs, revealing shadows that suggest scarring. The doctor will show you what position you need to be in. You may need to wear a hospital gown. Images will be taken using a special plate of sensors.
CT (Computed Tomography) and HRCT (High Resolution CT) scan26
Looking at: How much of the lung is thickened. Fibrosis will look different to normal lung tissue.
What to expect: CTs are giant doughnut-shaped machines. They provide an image (a type of X-ray) that is sharper and more detailed than a standard chest X-ray. You will be asked to lie on a trolley. The ring will then move up and down your body. This type of scan is commonly used to look for lung problems in scleroderma. It doesn’t hurt.
CT scans are used more frequently, but it varies from hospital to hospital.
Looking at: The structure and function of your heart, as well as how well blood moves through it to the lungs.
Echocardiography uses sound waves to create a detailed moving image of your heart. It helps create a measure of the blood pressure in the pulmonary artery that takes blood from your heart to your lungs, which helps the doctors spot and treat PAH.
What to expect: The doctor/nurse will move a probe around your chest to get different images of your heart, a bit like an ultrasound. It’s completely painless and will take from 15 minutes to an hour.
Looking at: Levels of several biomarkers in your blood. One example is a protein called N-terminal pro-brain natriuretic peptide (NT-proBNP). This is linked to heart function and helps to detect and predict PAH.
What to expect: A small needle will be used to collect a blood sample from you. Your doctor/nurse will let you know if you need to do anything to prepare.
Right heart catheterisation29
Looking at: The blood pressure in the right side of your heart and in your pulmonary artery (the blood vessel that connects your heart with your lungs). It can help to diagnose PAH.
What to expect: A thin, flexible tube will be passed into your pulmonary artery to take an accurate blood pressure measurement.
It is likely to be carried out in the hospital or a specialist heart centre.
Living with lung problems
Many people with scleroderma will have some lung problems.7
As with everything to do with scleroderma, it is difficult to predict whose lungs will be affected, or how badly.
It’s important to look after your lungs. Your doctor will help you to find ways to lessen the impact on your life so that you can still do the things that are important to you:
Ensure your lungs are frequently monitored – don’t miss check-ups and report any new symptoms
Look after your lungs – that means no cigarettes. If possible, stay away from places with bad air pollution
Manage any symptoms of gastro-oesophageal reflux disease (GORD) – your doctor/nurse will be able to help you do this
Stay positive – most lung symptoms can be managed. Consider alternative ways to keep doing the things you love
Avoid lung infections – wash your hands regularly and talk to your doctor about any vaccinations that could help prevent lung infections
Try to stay active - simply light exercise will help. Always check with your doctor first, and consider making a list of any questions you might have
Talk to friends and family – they could be able to find ways to help you
It can help to talk with family, friends and colleagues right from the start. They will be able to find ways to help you do what you need or want to do.
As yet, no drugs can cure scleroderma or the lung problems it causes, but the scientists are working on it.5,23 For now, your doctors might suggest some medication to help manage the lung problems as well as possible.
It was my daughter who got me through the tougher times. She wouldn’t let me give up. She’d stand in the doorway, coat on, ready to go. A ball of those reusable bags tucked under her arm. Her energy was electric. We were going food shopping and that was that. She knew when to stop. And when to push. Without her, I think I’d have just sat at home in the dark. I’m a very lucky dad.
Person with scleroderma
Solomon J et al. Scleroderma lung disease. Eur Respir Rev 2013;127:6–19.
Steen V et al. Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 1994;66(6):1625-35.
Steen V et al. Pulmonary involvement in systemic sclerosis (scleroderma). Arthritis Rheum. 1985; 28(7):759-67.
Nihtyanova SI et al. Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheum 2014;66(6):1625-35.
Iudici M et al. Where are we going in the management of interstitial lung
disease in patients with systemic sclerosis? Autoimmunity Reviews 2015;14(7):575-78.
University of Michigan Scleroderma Program. Lung Involvement. Available at: https://www.med.umich.edu/scleroderma/patients/lung.htm (last accessed May 2017).
Schurawitzki H et al. Interstitial lung disease in progressive systemic sclerosis: High-resolution CT vs radiography. Radiography. 1990;176:755-9.
Steen V and Medsger T. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum 2000;43(11):2437-2444.
Scleroderma & Raynauds UK. Lung Fact Sheet. Updated March 2016. https://www.sruk.co.uk/media/filer_public/ac/74/ac749333-b083-4046-b688-366fd01ef19b/lungs_factsheet_v2_rgb.pdf Accessed June 2017.
Willems L. et al. Frequency and impact of disease symptoms experienced by patients with systemic sclerosis in five European countries. Clin Exp Rheumatol 2014;32(S86):S88-93.
Jaeger V et al. Incidences and risk factors of organ manifestations in the early course of systemic sclerosis: a longitudinal EUSTAR study. PLoS One 2016;11(10):e0163894.
McNearney T et al. Pulmonary involvement in systemic sclerosis: Associations with genetic, serologic, sociodemographic, and behavioural factors. Arthritis Rheum. 2007;57(2):318-26.
Steen V et al. Changes in causes of death in systemic sclerosis. Ann Rheum Dis 2007;66:940–944.
Denton C. and Haculla E. Risk factors associated with pulmonary arterial hypertension in patients with systemic sclerosis and implications for screening. Eur Respir Rev 2011;20(122):270–276.
Mayo Clinic. Pulmonary hypertension. Updated March 2016. http://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/symptoms-causes/dxc-20197481 Accessed June 2017.
BMJ Best Practice. Systemic sclerosis (scleroderma): monitoring. http://bestpractice.bmj.com/best-practice/monograph/295/follow-up/recommendations.html Accessed May 2017.
Proudman S et al. Pulmonary arterial hypertension in systemic sclerosis: the need for early detection and treatment. Internal Medicine Journal 2007;37:485-494.
McLaughlin V et al. ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension. Journal of the American College of Cardiology 2009;53(17):1573-1619.
Coghlan J et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis 2014:73:1340-1349.
Humbert M et al. Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum. 2011;63(11):3522-30.
Chatterjee S. The Cleveland Clinic Foundation. Systemic scleroderma. Published 2010. http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/rheumatology/systemic-sclerosis/ Accessed June 2017.
BMJ Best Practice. Systemic Sclerosis (scleroderma): diagnostic approach. http://bestpractice.bmj.com/best-practice/monograph/295/diagnosis/step-by-step.html Accessed May 2017.
Galiè N et al. 2016 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67-119.
Cappelli S et al. Interstitial lung disease in systemic sclerosis: where do we stand? Eur Respir Rev 2015;24:411–19.
Radiological Society of North America Inc. X-ray (Radiography) https://www.radiologyinfo.org/en/submenu.cfm?pg=xray. Accessed August 2017.
Radiological Society of North America Inc. Computed Tomography https://www.radiologyinfo.org/en/submenu.cfm?pg=ctScan. Accessed August 2017.
NHS Choices. Echocardiogram. Available at: http://www.nhs.uk/Conditions/echocardiogram/Pages/Introduction.aspx Accessed July 2017.
Thakkar V et al. N-terminal pro-brain natriuretic peptide in a novel screening algorithm for pulmonary arterial hypertension in systemic sclerosis: a case-control study. Arth Res & Ther 2012;14:R143.
NHS Choices. Diagnosing pulmonary hypertension. Available at http://www.nhs.uk/Conditions/pulmonary-hypertension/Pages/diagnosis.aspx Accessed August 2017.