What is scleroderma
(Pronounced: Skleer- oh, dur, muh)
Scleroderma is a rare disease that affects people in different ways.1,2 It can be complicated to understand, but it's worth taking the time to get informed.
5 min read
Scleroderma in brief:
Scleroderma is part of a family of diseases that affect connective tissue.1,2
Because connective tissue is in almost every part of the body, symptoms can occur in the skin, muscles, blood vessels and internal organs.2
Everyone with scleroderma has a slightly different combination of symptoms, which is one reason why experiences can be so varied.2
Did you know?
Scleroderma most commonly appears at two stages in life—the early 30s and mid-50s. As with other autoimmune diseases, it is mostly women that are affected.1
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"I think wanting to enjoy life is important, and I do enjoy life and I’m going to go on enjoying life."
— Mike
Why are there different names?
Different words are used to describe this family of diseases:
Scleroderma
Used to describe the sclerosis (hardening) of the skin (derma), specifically. However, scleroderma is the term that is often used to refer to all types of sclerosis; both the skin changes and the changes in other tissue and organs in the body (systemic sclerosis).
Systemic
Used when a disease affects a number of different tissues and organs in the body.
Sclerosis
Used to describe the hardening of tissues in the body.
What are the different types of scleroderma?
Scleroderma is roughly divided into two main types: ‘localised scleroderma’ (also known as morphea) and ‘systemic sclerosis’. Systemic sclerosis is then further divided into four subtypes: limited cutaneous, diffuse cutaneous, sine scleroderma and overlap syndrome.1,3
How does scleroderma affect the body?
Connective tissue is found throughout the body—it's basically what holds you together. But it's also more than that. It supports, separates and connects different parts of the body. Because scleroderma affects the connective tissue, symptoms can be diverse and occur anywhere in the body.2
When a part of your body gets damaged (for example when you are injured), it sets off a natural healing cycle to repair the damage. In scleroderma, the immune system causes the natural healing process to go into overdrive and produce too much collagen.2,4-7 The excess collagen forms hard tissue like scars.
Healing cycle in scleroderma2,4-9
1
When you are injured, the body’s natural defence system (your immune system) starts to repair the damage. The area becomes inflamed. Connective tissue is like sponge cake with cells sitting in it like pieces of fruit. It’s made up of a mesh of fibres that support and hold cells.
2
The immune system signals to the fibroblasts to repair the damage. Fibroblasts are one type of cell held within the mesh to help us heal, keep us healthy, repair damaged tissues and form scars.
3
When your immune system works correctly, fibroblasts produce enough collagen and other substances to repair any damage to the connective tissue and form a scar. However, with scleroderma, the immune system tells the fibroblasts to produce more collagen than is needed, as it thinks your own cells are a danger and tries to defend your body against itself. Collagen is one of the fibres that make up the mesh.
4
For healthy individuals, a scar is formed as the damage is repaired. This scar softens over time as the normal surrounding tissues recover. With scleroderma, the extra, unneeded collagen gathers to form thick and rigid areas like a scar. This scar tissue (fibrotic tissue) can make the problem worse, as it causes the continuing cycle of inflammation, collagen and scarring. This fibrosis and inflammation affect how the skin and organs work, causing the symptoms of scleroderma.
Your immune system has a memory7
Your immune system protects you against threats from bacteria, viruses and danger from damage to tissues. Your body’s immediate response to a threat is to produce inflammation. Inflammation is a normal response from your body’s defences. It surrounds, contains and then gets rid of whatever is causing the problem (infection, splinter, thorn etc).
Once the danger has been removed, your immune system produces special substances called antibodies. These are programmed to recognise bacteria, viruses or other dangers you have already been exposed to. Antibodies find them and let the immune system know, so that they can deal with them more quickly.
Did you know?
Scleroderma is an ‘autoimmune’ disease
‘Auto’ means self and ‘immune’ means protection against. Together this means that the immune system acts against the person’s own body.
What are the early warning signs of scleroderma?
The symptoms shown above are often the first clue, and can occur alongside other symptoms.1,8 Be sure to tell your doctor if you experience two or more of these together. If you suspect you have scleroderma, your doctor will be able to perform a blood test to check for auto-antibodies specific to scleroderma and arrange for a capillaroscopy, to help ensure prompt diagnosis.9
Remember, these are not the only symptoms of scleroderma.
Key takeaways
Scleroderma is a rare autoimmune disease that affects people in different ways.
It is a part of a family of diseases that affect connective tissue.1,2
Because connective tissue is found throughout the body, people with scleroderma may have different combinations of symptoms.1,2
You may also like to read:
Testing for scleroderma
Managing symptoms
Mindfulness
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Derrett-Smith E and Denton C. Systemic sclerosis: clinical features and management. Medicine 2010;38(2):109–15.
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Varga J and Abraham D. Systemic sclerosis: a prototypic multisystem fibrotic disorder. J Clin Invest 2007;117:557–67.
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Denton CP and Khanna D. Systemic sclerosis. 2017. The Lancet. In Press. Available: http://thelancet.com/journals/lancet/article/PIIS0140-6736(17)30933-9/fulltext [Accessed December 2021].
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Allanore Y, et al. Systemic sclerosis. Nat Rev Dis Primers 2015; 1:1–21.
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Pattanaik D, et al. Pathogenesis of systemic sclerosis. Front Immunol 2015;6 (article 272):1–40.
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MacLeod AS and Mansbridge JN. The innate immune system in acute and chronic wounds. Advances in Wound Care 2016;5(2):65–78.
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Strbo N, et al. Innate and adaptable Immune responses in wound epithelization. Advances in Wound Care 2014;3(7):492–501.
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Minier T, et al. Preliminary analysis of the Very Early Diganosis 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.
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Van den Hoogen F, et al. 2013 classification criteria for systemic sclerosis. Arthritis Rheum 2013;65(11):2737–47.
"I first went to my GP when my body was tightening and stiffening up. I'm glad I did so, as my GP provided me with the right treatment and information on how I could learn to live with my diagnosis."
– Mandy