“Scleroderma didn’t take anything away from me. It gave me another view of life.”
Person with scleroderma

Symptoms



Symptoms of scleroderma

How scleroderma (also known as systemic sclerosis) may affect you depends on a few things:

  • The parts of your body affected
  • How mild or severe the symptoms are
  • How the symptoms change over time

Most patients start with just one or two symptoms, but they can appear in any order. They can be mild or more severe, and can change as time passes.1-­4

It may look like there are a lot of symptoms. But you won’t necessarily get them all. People get different combinations of symptoms, and have different experiences and problems with those symptoms.5-­7 It’s one of the reasons why it can be hard for doctors to recognise scleroderma early on.

With so many different types of symptoms it can sometimes be hard to know which ones are important. It is essential that you report all your symptoms to your doctor, no matter how insignificant they may seem. These could include:

  • Tiredness and fatigue
  • Skin symptoms
  • Lung problems
  • Heart problems
  • Hand symptoms
  • Kidney problems
  • Joint and muscle symptoms
  • Digestive system symptoms
  • Foot pain and walking problems
  • Mental health and emotional wellbeing

Skin symptoms

Nearly all people with scleroderma (more than 90%) develop some skin symptoms.3 The symptoms can include:

  • Skin thickening, particularly on the joints of the fingers8
  • Tight or shiny skin on the face, arms, and legs. Tightened skin around the joints can prevent you from moving properly. These are known as contractures. You may also get itchy skin9
  • Hard lumps (called calcinosis) develop in around 25% of people with scleroderma.3 These are a collection of calcium salts beneath the skin. They develop on pressure points or around joints usually when you have had scleroderma for some time
  • Small red spots (called telangiectasia) develop in around 75% of people with scleroderma.3, 8 These are little widened blood vessels, usually on the hands and face
Scleroderma skin thickening, shiny skin, small red spots (telangiectasia), calcinosis
Images above (from left to right) show: skin thickening, shiny skin, small red spots (telangiectasia), calcinosis.

There are many things that can help to manage skin symptoms and the way they affect how you feel or look.

  • Using creams or ointments every day helps with dry, tight or itchy skin. They’ll keep your skin well moisturised.10 Ask your doctor or nurse for help in choosing the right product for you
  • Wear rubber gloves when you wash up to protect the skin on your hands10
  • Avoid using strong soaps because these can irritate your skin10
  • Exercises help to keep the movement around your joints. Your doctor or nurse may be able to show you how to do these. Physiotherapy can help to keep your joints moving11
  • Soak your hands or feet in warm paraffin wax baths to help to soften the skin12
  • If you are worried about how telangiectasia looks, you may be able to get some advice about how to conceal them. Ask your doctor or nurse for details

Your doctor or nurse will be able to give you more information and advice about your symptoms and may be able to offer other ways of helping you to manage them.

I look a bit like I’ve got chicken pox. I was worried people would think I’m contagious. That was until my sister showed me this amazing make-up tutorial online. I’ve learnt some great tricks and am feeling more confident by the day.

Person with scleroderma

Hand symptoms

Many of the hand symptoms are linked to skin involvement, except for Raynaud’s phenomenon. This is linked to the tiny blood vessels in your hand affecting how blood circulates. More than 90% of people with scleroderma get Raynaud’s phenomenon.3, 13 It’s one of the most common early symptoms.5, 14

Symptoms in the hands include:

  • Raynaud’s phenomenon – when your fingertips change colour (usually white to blue/purple, then to red), get very cold and sometimes hurt or feel numb. You may also develop red, swollen areas on the fingers and toes that are painful to touch (sometimes called chilblains).15 These changes often happen in cold weather or when you are stressed. They are caused by spasming of small blood vessels in your fingertips. This reduces blood supply to the area.3, 13 Keep an eye on the red, swollen areas to make sure the skin doesn’t open over them because they can become infected
  • Puffy hands and fingers14
  • Tightening and thickening of the skin on the fingers (called sclerodactyly). Sometimes this can make it hard to keep your hand flat. You may find your fingers don’t move as well as they should or you have trouble holding objects5
  • Ulcers or open sores occur in around 40% of people with scleroderma, particularly on their fingertips or toes. These may occur after minor injury or spontaneously due to poor blood supply3, 8
    You should always see your doctor if you get an ulcer or open sore on your fingers or toes, no matter how small or mild it may seem, to make sure it doesn’t get infected
  • Pain or stiffness in the joints of your fingers6
Raynaud's phenomenon, tightening and thickening of the skin (called sclerodactyly), ulcers or open sores.

Images above (from left to right) show: Raynaud's phenomenon, tightening and thickening of the skin (called sclerodactyly), ulcers or open sores.

My hands are icy and the tips go a brilliant white – my daughter calls me Snow White. I find that wearing gloves really helps and I have quite a collection now!

Person with scleroderma

There are things you can do to manage your hand symptoms.

  • Wear gloves or carry handwarmers.10 With Raynaud’s phenomenon, keeping your hands warm is important
  • Try to give up smoking. Nicotine constricts the small blood vessels and makes Raynaud’s phenomenon worse16
  • Wear several layers of loose clothing rather than one thick layer – this can help prevent rubbing if you have chilblains17
  • Keep your fingers supple with exercises. Your doctor or nurse can show you some exercises to do or may advise you to see a physiotherapist17

Your doctor or nurse will be able to give you more information and advice to help you manage problems with your hands.

Foot symptoms

  • Raynaud’s phenomenon can affect your toes as well as your fingers18
  • Uncomfortable walking because of changes under the ball of your foot. Some people with scleroderma describe this as feeling as though they are walking on pebbles18

Similar to your hands and skin, there are things you can do yourself to help manage the symptoms in your feet:

  • Wear several layers of socks and thick soled shoes to keep your feet warm. This can also help provide padding for your feet if you find it uncomfortable to walk on uneven surfaces18
  • Moisturise the skin on your feet to keep it supple17
  • Inspect your feet daily. Keep an eye out for any ulcers so you can spot them early. If you do get an ulcer, see your doctor so that he or she can check it is not infected17

Your doctor or nurse will be able to give you more help and advice about managing your foot symptoms.

Raynaud’s Phenomenon toes

Image above shows: Raynaud's phenomenon

Joint and muscle symptoms

Around 65% of people with scleroderma have problems with their joints or muscles at some time.3 Joint symptoms can affect the big joints, like your knees, elbows or hips. Or they may affect the smaller joints, like the ones in your fingers and toes. Your muscles may also be involved, which can make it harder to move freely and stay active.

Joints may be painful because the skin becomes tight, making it more difficult to move. Pain might also be caused by inflammation occurring in or around the joint.19 Sometimes people with scleroderma also have other conditions, like rheumatoid arthritis, that makes their joints painful.9

Muscles can become weaker if you become less fit because you are doing less exercise.

Symptoms can include:

  • Tight skin or swollen joints3, 5
  • Joint pain or tenderness might also be caused by inflammation3
  • Other joint conditions can sometimes occur alongside scleroderma, such as rheumatoid arthritis5, 9
  • Muscle fatigue and weakness or aching6

There are ways to manage these symptoms. Exercises to keep the joints moving can help. It is important to tell your doctor or nurse about the problems with your joints or muscles, so that they can work out what is causing the problems and how to help.

It’s like the opposite of knobbly knees. My knees have no shape, they’re just a like a big orange. But now I have discovered long skirts, the only one who knows is me.

Person with scleroderma

Tiredness and fatigue

Over 90% of people with scleroderma experience severe tiredness (sometimes called fatigue). It is one of the most common symptoms.6

There are ways to manage the way fatigue affects you. Often, it’s about allowing yourself the time to rest. Planning and pacing your activities can help, so that you can still do what you want or need to do, but maybe over a slightly longer time, in shorter bursts, or with rest times in between.

It is important to tell your doctor if you have severe tiredness so that they can make sure the fatigue is not because of some other condition.

You just grind to a halt – like you just run out of gas. Don’t fight it – give yourself time to refuel. Settle in and catch up on your favourite programs.

Person with scleroderma

Digestive system symptoms

Your digestive system includes many different organs in your body, such as your mouth, stomach, and intestines (gut). It is involved in lots of activity so it’s not surprising that most people with scleroderma (around 90%) get some symptoms related to it.3 There are several different symptoms related to the digestive system, and they can differ from person to person.1, 5, 6, 20

In scleroderma, fibrosis (scarring) makes the walls of the gut a bit thicker and the muscles don’t work as effectively. This affects the way food is digested and moved through your body and causes the different symptoms that people experience.

 

Digestive system symptoms include:6, 21

  • Heartburn or indigestion
  • Changes in appetite
  • Constipation
  • Diarrhoea
  • Feeling sick (called nausea)
  • Finding it hard to swallow

There are ways to manage how digestive system symptoms affect you.

  • If you have difficulty swallowing:21
     
    • Keep your mouth moist by taking sips of water or other fluids throughout the day
    • Eat foods that are soft and moist or puree them if that helps you to swallow
    • Use lemon or other sharp tastes to make your mouth produce more saliva
    • Allow plenty of time for your meals

     
  • If you experience heartburn or indigestion this may be because the muscle that closes the top of your stomach doesn’t work very well any more, allowing stomach acid to return up your oesophagus (food pipe):3, 21
     
    • Eat smaller, more frequent meals, rather than a lot all at once
    • Make lunch your main meal, rather than dinner, so that you are not going to bed too soon after eating a large meal
    • Try to sit straight during and after meals, to let gravity help your digestion
    • Avoid eating and drinking just before bedtime. You don’t want to be uncomfortable during the night
    • You may find it helpful to raise the top end of your bed by 10 to 15 cm, so that gravity helps keep any acid down in your stomach

     
  • If you experience diarrhoea or constipation, talk to your doctor. They can rule out other causes and might advise you to change the balance of fibre and fluids that you consume:21
     
    • Avoid insoluble fibre if you have diarrhoea, such as the skins on fruit and vegetables, as this simply passes through your system
    • Make sure you drink plenty of fluids so that you don’t become dehydrated
    • If needed, your doctor may be able to prescribe medications to help with your diarrhoea or constipation

     
  • If you have a poor appetite or have lost a lot of weight:21
     
    • Try to eat little and often to allow your digestive system time to absorb as many nutrients as it can
    • You may find it helps to add some supplements to your meals and drinks so that you get extra protein and energy. Ways in which you can do this include:
      • Adding dried milk powder to full fat milk
      • Adding butter/margarine or olive oil to potatoes and vegetables, soups and sauces
      • Sprinkling extra cheese over food

     
  • If you have problems with your mouth, teeth and gums:21, 22

    • Dry mouth can be helped by sipping water, chewing sugar­‐free gum to stimulate saliva, or speaking to your doctor/nurse about salivary substitutes​
      • Dry mouth can also cause problems with the fit of dentures, causing discomfort and sores – speak to your dentist about the changes you could make to help your dentures fit better
    • Mouth stretching exercises can help with tight skin around the mouth that stops you from opening it fully
    • Oral ulcers can appear, particularly with certain medications – these can be managed with good oral hygiene and salicylate gels to help reduce any pain

Your doctor or nurse will be able to offer additional advice and help for digestive system symptoms.

I’d been feeling so bloated, it was very uncomfortable. But a few little switches made a massive difference. Pasta’s now whole wheat and meals are packed full of veg. The whole family’s looking and feeling better for it.

Person with scleroderma

Lung problems

While most people with scleroderma get some fibrosis (thickening or scarring) in their lungs, only 13 to 16% develop severe symptoms.23, 24 Up to half of people who will be affected experience symptoms within the first 3 years of the disease.1, 23, 25-­28

It is important to find out whether scleroderma is affecting your lungs as soon as possible so that your doctor can determine whether you need treatment and, if so, the best treatment for you.

There are key two ways in which scleroderma might affect your lungs:29

  1. Pulmonary arterial hypertension (or PAH) is a rare complication, affecting between 5 and 12% of people with scleroderma and increasing with time.30

    It happens because fibrosis 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. The heart tries to fix the problem by making the right side of the heart work much harder.31

    Symptoms of pulmonary arterial hypertension include:29, 31
    • Shortness of breath on climbing stairs or hills
    • Feeling tired
    • Dizziness

    Your doctor will be checking regularly to make sure you don’t have pulmonary arterial hypertension. If you do develop this, your doctor will help you manage it and find the treatment that best suits you.

     
  2. Lung fibrosis (also known as interstitial lung disease [or ILD]). This affects most people with scleroderma to some extent, but only between 13 and 16% of people will develop severe symptoms.23-­25

    This 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 we breathe into the blood and removing carbon dioxide from the blood to be breathed out. The scarring and stiffness prevent the lungs from doing this job effectively.32

    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.6, 23

    Symptoms of lung fibrosis include:6, 29
    • 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
    • Feeling dizzy

Specialist doctors across a number of different countries recommend that lung function should be tested regularly, and at least annually, in people with scleroderma.33-­38 This is so they can help to manage the symptoms as early as possible.

If you notice any symptoms, tell your doctor. The sooner you say something, the sooner you can do something about it.

In an effort to be healthy I started taking the stairs at work instead of the lift. But as the weeks went on, I was getting more out of puff, not less. My doctor has been really supportive. I’m not going to win any races, but I’m a long way off from needing that lift.

Person with scleroderma

Heart conditions

Heart disease is more common as we get older, and it’s not surprising that people with scleroderma get the same heart conditions as everyone else. However, there are some specific heart problems that can happen because of scleroderma.

They happen for several reasons, but usually because fibrosis (scarring) can affect the muscle of the heart, the valves, or structures in and around the heart that help it work properly.39

Your doctor or nurse will check your heart regularly to look for problems. Make sure you attend your appointments and tell your doctor if you have had any problems.

The symptoms of heart conditions are varied and can be hard to spot. There may be no symptoms at all.39

 

Symptoms of heart conditions can include:39

  • Chest pain
  • Swollen legs
  • Breathlessness
  • Feeling like your heart is pounding, fluttering or beating irregularly (palpitations)
  • Light headedness
  • Fainting
  • Tiredness (fatigue)

Kidney problems

Just under half of people with scleroderma get some kidney problems.40 Scarring and fibrosis in the kidneys can reduce their ability to work properly, but in most cases, this won’t cause any symptoms. It’s usually not serious, but your doctor will monitor your kidneys regularly to make sure they’re working well.

A few people with scleroderma (5–10%) experience a more serious issue, called renal crisis.3 It happens quickly and needs urgent treatment in hospital.

 

Symptoms of a renal crisis include:41

  • Headache
  • Feeling feverish/generally unwell
  • Tiredness
  • Chest pain
  • Changes in vision
  • Seizures

If you experience these symptoms, contact your doctor straight away. You may need urgent treatment.

If you're at risk of renal crisis, you should regularly monitor your blood pressure. Your doctor will let you know if you're at risk.

My doctor said it’s like a heart attack of the kidneys. When my renal crisis hit, I just knew something was really wrong. But they were fantastic. Got me into hospital for treatment and now I’m back to normal.

Person with scleroderma

Mental health and emotional wellbeing7

While not an actual symptom of scleroderma, some people find that the disease has an impact on their mental and emotional health.

Some people find scleroderma changes how they see themselves, or how others see or treat them. It can affect the way you do your job or the role you play in your family.

Adjusting to life with a new set of limits can be hard. This disease of unknowns can create uncertainty or sometimes loneliness.

Whatever your situation, whatever you’re feeling, you don’t have to go through it alone. Sometimes, one of the hardest things to do is to ask for help.

Help can come from anywhere – your friends and family, your doctor or nurse, a local support group, or simply a story on this website.

Make sure you speak to someone if you’re findings things difficult.

I feel like my mind has not yet figured out the limitations of this new body I’m in. It just doesn’t feel like me. Then my husband took me to the theatre to see my favourite play, followed by a meal at the restaurant where we had our first date. He showed me that the things I love – the things that make me, me – haven’t changed at all.

Person with scleroderma

References

  1. 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.

  2. Muangchan C et al. The 15% rule in scleroderma: the frequency of severe organ complications in systemic sclerosis. A systematic review. J Rheumatol 2013;40:1545-1556.

  3. Allanore Y et al. Systemic sclerosis. Nat Rev Dis Primers 2015;1:1-21.

  4. Varga J, Abraham D. Systemic sclerosis: a multisystem fibrotic disorder. J Clin Invest. 2007;117:557-67.

  5. BMJ Best Practice. Systemic Sclerosis (scleroderma): Diagnostic approach. http://bestpractice.bmj.com/best-practice/monograph/295/diagnosis/step-by-step.html Accessed May 2017.

  6. 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-S93.

  7. Nakayama A et al. Patients’ perspectives and experiences living with systemic sclerosis: a systematic review and thematic synthesis of qualitative studies. Rheumatol 2016;43:1363-1375.

  8. van den Hoogen F et al. 2013 classification criteria for systemic sclerosis. Arthritis Rheum 2013;65(11):2737-47.

  9. Denton CP and Khanna D. Systemic sclerosis. 2017. The Lancet. In Press.

    http://thelancet.com/journals/lancet/article/PIIS0140J6736(17)30933J9/fulltext

  10. Scleroderma & Raynaud’s UK. Treatments. https://www.sruk.co.uk/scleroderma/managing-scleroderma/treatments/ Accessed June 2017.

  11. Scleroderma & Raynaud’s UK. Skin. https://www.sruk.co.uk/scleroderma/scleroderma-and-your-body/skin/ Accessed August 2017.

  12. Scleroderma & Raynaud’s UK. Wax bath therapy. https://www.sruk.co.uk/scleroderma/scleroderma-and-your-body/wax-bath-therapy Accessed June 2017.

  13. Silver R. Clinical aspects of systemic sclerosis. Ann Rheum Dis 1991;50:854-861.

  14. Minier T 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;73:2087–93.

  15. Scleroderma & Raynaud’s UK. Chilblains Fact Sheet. Updated March 2016.

    https://www.sruk.co.uk/raynauds/chilblains/

    94474d2e21ab/chilblains_factsheet_v2_rgb.pdf Accessed August 2017.

  16. Scleroderma & Raynaud’s UK. What is Raynaud’s https://www.sruk.co.uk/raynauds/what-raynauds/ Accessed June 2017.

  17. Scleroderma & Raynaud’s UK. Hands and feet. https://www.sruk.co.uk/scleroderma/scleroderma-and-your-body/hands-and-feet Accessed June 2017.

  18. Scleroderma & Raynaud’s UK. Foot conditions. https://www.sruk.co.uk/raynauds/managing-raynauds/foot-conditions/ Accessed August 2017.

  19. Derrett-Smith EC, Denton CP. Systemic sclerosis: clinical features and management. Medicine 2010;38(2):109J15.

  20. Forbes A, Marie I. Gastrointestinal complications: the most frequent internal complications of

    systemic sclerosis. Rheumatology 2008;48:iii36–iii39.

  21. Scleroderma & Raynaud’s UK. Gastrointestinal. https://www.sruk.co.uk/scleroderma/scleroderma-and-your-body/gastrointestinal/ Accessed June 2017.

  22. Scleroderma & Raynaud’s UK. Oral and dental. https://www.sruk.co.uk/scleroderma/scleroderma-and-your-body/oral-and-dental/ Accessed August 2017.

  23. Steen V et al. Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 1994;66(60):1625-35.

  24. Steen V and Medsger T. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum 2000;43(11):2437-2444.

  25. Solomon J et al. Scleroderma lung disease. Eur Respir Rev 2013;127:6-19.

  26. Steen V et al. Pulmonary involvement in systemic sclerosis (scleroderma). Arthritis Rheum. 1985;28(7): 759-67.

  27. Nihtyanova SI et al. Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheum 2014;66(6):1625-35.

  28. McNearney T et al. Pulmonary involvement in systemic sclerosis: Associations with genetic, serologic, sociodemographic, and behavioural factors. Arthritis Rheum. 2007;57(2):318-26.

  29. University of Michigan Scleroderma Program. Lung Involvement. https://www.med.umich.edu/scleroderma/patients/lung.htm Accessed May 2017.

  30. Denton C. and Hachulla 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.

  31. Mayo Clinic. Pulmonary hypertension. Updated March 2016. http://www.mayoclinic.org/diseases- conditions/pulmonary-hypertension/symptoms-causes/dxc-20197481 Accessed June 2017.

  32. Scleroderma & Raynaud’s 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.

  33. Proudman S et al. Pulmonary arterial hypertension in systemic sclerosis : the need for early detection and treatment. Int Med Journal 2007;37:485-494.

  34. McLaughlin V et al. Expert consensus document on pulmonary hypertension. J Am Coll Cardiol 2009;53(17) :1573-1619.

  35. Coghlan JG et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis 2014;73:1340-1349.

  36. 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.

  37. Chatterjee S. The Cleveland Clinic Foundation. Systemic scleroderma. Published 2010. http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/rheumatology/systemic-sclerosis/ Accessed June 2017.

  38. BMJ Best Practice. Systemic sclerosis (scleroderma): monitoring. http://bestpractice.bmj.com/topics/en-gb/295/monitoring Accessed May 2017.

  39. Lambova S. Cardiac manifestations in systemic sclerosis. World-Cardiol 2014;6(9):993-1005.

  40. Cannon P et al. The relationship of hypertension and renal failure in scleroderma (progressive systemic sclerosis) to structural and functional abnormalities of the renal cortical circulation. Medicine. 1974;53(1):1-46.

  41. Denton C. et al. Renal complications and scleroderma renal crisis. Rheumatology 2009; 48,32-35.

Back to top