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Symptoms of scleroderma

It may look like there are a lot of symptoms, but you won’t necessarily get them all. People get different symptoms, and have different experiences with them.1,2

How scleroderma may affect you depends on the parts of your body involved, the severity of your symptoms and how these change over time. Most people start with just one or two symptoms, which can appear in any order.3-6

Some symptoms will have a bigger impact on your day to day life than others, but it’s very important your doctor knows about every symptom you experience. You should report all your symptoms to your doctor, no matter how insignificant they may seem.

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Skin

More than 90% of people with scleroderma develop some skin symptoms1,4

Skin thickening

This particularly affects the joints of the fingers.7

Skin thickening

Tight or shiny skin

Tight skin around the joints (known as contractures) can stop you moving freely. You may also get itchy skin.1

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Hard lumps (calcinosis)

Calcinosis develops in around 25% of people, usually those with longstanding scleroderma.4 They occur on pressure points or around joints.

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Red spots (telangiectasia)

These widened blood vessels appear in around 75% of people with scleroderma,4 usually on the hands and face.

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Fortunately, there are ways your doctor can help you manage skin symptoms.

Skin symptoms

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Hands

Raynaud’s phenomenon

One of the most common early symptoms of scleroderma is Raynaud’s phenomenon.8,9 More than 90% of people with scleroderma develop it.4,10

In Raynaud’s phenomenon, spasming of small blood vessels in your hands reduces the blood supply to the fingertips.4,10 Your fingertips may change colour (usually white to blue/purple, then to red), get very cold and sometimes hurt or feel numb. They may also develop red, swollen areas which are painful to touch (sometimes called chilblains)11 often in cold weather or when you are stressed.

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There are several other possible hand symptoms which can be caused by scleroderma:

Puffy hands and fingers

Puffy hands and fingers are considered an early sign of scleroderma.9

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Skin tightening

Known as sclerodactyly, this can sometimes make it hard to keep your hand flat, move your fingers or hold objects.8

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Ulcers or open sores

These occur on the fingertips or toes of around 40% of people with scleroderma due to minor injury or poor blood supply.4

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Joint pain or stiffness

Pain and stiffness in the joints frequently occur in scleroderma.2

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There are exercises and other techniques to help manage hand symptoms.

Hand symptoms

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Feet

Raynaud’s phenomenon

Caused by spasming of small blood vessels that reduces blood supply, Raynaud’s phenomenon commonly affects the fingers but can also affect your toes.12

Raynaud’s phenomenon

Uncomfortable walking

Changes under the ball of your foot can lead some people with scleroderma to feel like they are walking on pebbles.12

Raynaud’s phenomenon

Changing your footwear and other techniques can help you manage foot symptoms.

Foot symptoms

Joints and muscles

Joints and muscles

Around 65% of people with scleroderma have problems with their joints or muscles at some stage.4 Symptoms can affect big joints like knees, elbows or hips, or smaller joints, like the ones in your fingers and toes.

Symptoms can include:

  • Tight skin or swollen joints.2,8
  • Joint pain or tenderness.2
  • Muscle fatigue and weakness or aching.2

Joints may be painful because the skin becomes tight, making it more difficult to move. Pain might also be caused by inflammation.13 Sometimes people with scleroderma also have other conditions that make their joints painful, like rheumatoid arthritis.1

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

Joint and muscle symptoms can be painful but your doctor can help you.

Joint and muscle symptoms

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Digestive system

Your digestive system includes many different organs in your body, such as your mouth, stomach, and intestines (gut). It is involved in lots of activities so it’s not surprising that around 90% of people with scleroderma get some symptoms related to it.4

Symptoms can differ from person to person, and can include:2,5,14

  • Heartburn or indigestion.
  • Changes in appetite.
  • Constipation.
  • Diarrhoea.
  • Feeling sick (called nausea).
  • Difficulty swallowing.

In scleroderma, fibrosis (scarring) makes the walls of the gut thicker and the muscles don’t work as effectively. This affects the way food is moved through your body and digested. It’s also what causes the digestive symptoms.

Changing how, what and when you eat can help you manage your symptoms.

Digestive system symptoms

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Lungs

Many people with scleroderma have some form of changes to their lungs. However, not everybody gets noticeable changes.15-19

 

There are two key ways in which scleroderma may affect your lungs:15,20

Interstitial lung disease (or ILD or lung fibrosis)—when the lungs become stiffer and thicker.

ILD happens because fibrosis (scarring) affects the little air sacs in the lungs (the alveoli). Alveoli help transfer oxygen in the lungs into the blood supply and also help to remove carbon dioxide from the blood to be breathed out. 

lung-symptoms

Pulmonary arterial hypertension (or PAH)—when the pressure in the blood vessels in the lungs gets too high.

PAH 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.

heart-symptoms

Symptoms of the above lung conditions may include:21

  • 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.22

  • Difficulty taking deep breaths.

  • Feeling like your chest is tight.

  • Feeling dizzy.

  • Chest pain.

  • Swollen legs.

  • Bluish colour to your lips and skin (cyanosis).

  • Racing pulse or heart palpitations.
     

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Contact your doctor

If you experience the above symptoms, contact your doctor. 

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Tiredness

Over 90% of people with scleroderma experience severe tiredness. It is one of the most common symptoms.2

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.

Changing how you plan your day, can help manage your symptoms.

Tiredness

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Kidneys

Just under half of people with scleroderma get some kidney problems.23 Fibrosis in the kidneys can reduce their ability to work properly, but in most cases, this won’t cause symptoms and is not serious.

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.4

Symptoms of renal crisis include:24

  • Headache.
  • Feeling feverish/generally unwell.
  • Tiredness.
  • Chest pain.
  • Changes in vision.
  • Seizures.
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Contact your doctor

If you experience the above symptoms, contact your doctor. 

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Heart

Heart disease becomes more common in everyone as we get older. However, some specific heart problems can happen because of scleroderma. These usually occur because fibrosis (scarring) can affect the muscle of the heart, the valves or structures in and around the heart that help it work properly.25

The symptoms of heart conditions are varied and can be hard to spot. There may be no symptoms at all.25 Your doctor or nurse will check your heart regularly to look for problems. Make sure you attend your appointments.

Symptoms of heart conditions can include:26

  • Chest pain.

  • Swollen legs.

  • Breathlessness.

  • Feeling like your heart is pounding, fluttering or beating irregularly (palpitations).

  • Light headedness.

  • Fainting.

  • Tiredness (fatigue).

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Contact your doctor

If you experience the above symptoms, contact your doctor. 

Life hacks to overcome symptoms of scleroderma

If you have scleroderma, daily life can be challenging. We can help you with a few easy life hacks to help you get on top of practical issues like cold feet, raynaud's and dry skin.

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Monitoring scleroderma

Symptoms of scleroderma may change over time. Learn how regular monitoring can help keep the disease in check.
 
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  12. Scleroderma & Raynaud’s UK. Foot conditions. Available: https://www.sruk.co.uk/raynauds/managing-raynauds/foot-conditions/ [Accessed December 2021].

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  14. Forbes A, Marie I. Gastrointestinal complications: the most frequent internal complications of systemic sclerosis. Rheumatology 2008;48:iii36–iii39.

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

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

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

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

  19. Iudici M et al. Where are we going in the management of interstitial lung disease in patients with systemic sclerosis? Autoimmunity Reviews 14 (2015) 575–578.

  20. University of Michigan. Scleroderma Program: Lung Involvement. Available: https://medicine.umich.edu/dept/intmed/divisions/rheumatology/research/scleroderma-program/patients/lung.html [Accessed December 2021].

  21. Mayo Clinic. Pulmonary hypertension. Updated March 2016. Available: http://www.mayoclinic.org/diseases- conditions/pulmonary-hypertension/symptoms-causes/syc-20350697 [Accessed December 2021].

  22. Hummers L, Wigley F. Systemic Sclerosis, 2nd Edition. Johns Hopkins Scleroderma Center. 2018. Available: https://www.hopkinsscleroderma.org/patients/scleroderma-treatment-options/ [Accessed December 2021].

  23. 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.

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

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

  26. Mayo clinic. Heart Disease. March 22, 2018. Available: https://www.mayoclinic.org/diseases-conditions/heart-disease/symptoms-causes/syc-20353118 [Accessed December 2021].

 

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"When they confirmed I had scleroderma, it was a shock. I educated myself from the internet, reading everything i could find."

– Doris