What is scleroderma

(Pronounced: Skleer – oh, dur, muh)

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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

Did you know?

The terms ‘scleroderma’ and ‘systemic sclerosis’ are often both used to mean the type of scleroderma that affects several organs in the body, but the correct medical term is 'systemic sclerosis'.1

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

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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.

  • Connective tissue

    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.

  • Collagen

    Collagen is one of the fibres that make up the mesh.

  • Fibroblasts

    Fibroblasts are one type of cell held within the mesh to help us heal, keep us healthy, repair damaged tissues and form scars.

The healing cycle of a person without scleroderma
  1. When you are injured, the body’s natural defence system (immune system) gets involved. The area then becomes inflamed.

  2. The immune system signals to the fibroblasts to repair the damage.

  3. Fibroblasts produce collagen and other substances to repair any damage to the connective tissue; this forms a scar.

  4. The damage is repaired. The scar will soften over time, as the normal surrounding tissues recover. In fibrosis, this normal softening of the scar does not occur enough.

The healing cycle of a person without scleroderma
  1. The immune system sends the wrong messages to fibroblasts, telling them to produce lots of collagen. This is because it thinks your own cells are a danger and tries to defend your body against itself.

  2. Fibroblasts then produce too much collagen.

  3. The extra, unneeded collagen, gathers to form thick and rigid areas like a scar. The scar tissue (fibrotic tissue) can make the problem worse, as it causes the cycle of inflammation, collagen and scarring to go on and on.
    Fibrosis and inflammation in the skin and other organs affect how they work and cause the symptoms of scleroderma.

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.

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.

What are the early warning signs of scleroderma?

Scleroderma is So RaRe that it’s easy to miss.

  • Sore swollen fingers icon fingers
  • Raynaud‘s phenomenon icon raynaudss
  • Reflux & heartburn icon reflux-heart-burn

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

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Key takeways:

  • 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