"I had two choices when I found out I had scleroderma: to feel miserable forever or be positive. I chose to be positive. Life’s not a party, but I’m happy.”
Person with scleroderma

Scleroderma explained



Scleroderma or systemic sclerosis? Why are there two names?

Scleroderma is a family of diseases usually characterised by hardening of the skin.1  In medical definitions, different words are used to describe this family of diseases and they all have slightly different meanings:

  • 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 tissues 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

To use the words correctly, it can help to understand how they relate to each other.

‘Scleroderma’ is roughly divided into two forms, ‘localised scleroderma’ (also called morphoea) and ‘systemic sclerosis’.1, 2

Systemic sclerosis is then divided into four subtypes: limited cutaneous, diffuse cutaneous, sine scleroderma and overlap syndrome.1, 3, 4

The subtypes of systemic sclerosis help doctors get a better picture of the symptoms and problems you may experience, and to develop a treatment plan you might benefit from.

 

The scleroderma family of diseases1-4

Scleroderma family of diseases

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 term is systemic sclerosis.


What is scleroderma?

Scleroderma (also known as systemic sclerosis) is part of a family of diseases that affect connective tissue. Connective tissue is in almost every part of your body. It is what helps to hold your body together. It supports, connects and separates different parts of your body. Because scleroderma affects the connective tissue, symptoms can occur in any area of the body including the skin, muscles, blood vessels and internal organs.5

 

Understanding connective tissue5-9

Understanding connective tissue

Connective tissue is in almost every part of your body. It is what helps to hold your body together. It supports, connects and separates different parts of your body. 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 is one of the fibres that make up the mesh.

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

 

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

Natural healing process
  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

Did you know?

Your immune system has a memory

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 danger you have already been exposed to.

Antibodies find them and let the immune system know, so that it can deal with them more quickly.

In scleroderma, the immune system causes the natural healing process to go into overdrive and produce too much collagen.

scleroderma and collagen
  1. The immune system sends the wrong messages to the 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 scars.
    Extra scarring can make the problem worse. The scar tissue (fibrotic tissue) itself can cause damage, setting off the inflammation, collagen, and scarring response; the cycle goes on and on. Fibrotic tissue is stiff and doesn’t work in the same way as normal tissue. 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.

(This means the immune system causes the natural healing process to go into overdrive and produce too much collagen.)

‘Auto’ = self

‘Immune’ = protection against

I had symptoms for years, probably ten or fifteen years, before I was diagnosed.

Person with scleroderma


What symptoms does scleroderma cause?

Symptoms of scleroderma can include hardening or tightening of the skin, acid reflux or indigestion, diarrhoea, fatigue and many more. Your symptoms will depend on which parts of your body are affected and they may change over time. It is very unusual for people with scleroderma to have all symptoms at the same time. People have different combinations of symptoms, and the severity of symptoms differs from person to person.3, 5


Why do people get scleroderma?

There is no simple answer to this question. No one really knows why some people get scleroderma, while others don’t.6

We know that scleroderma is not an infection, like chicken pox or flu. You can’t catch it from someone else who has it.

We also know that genetics and genes may play a role.5, 6, 10, 11 If you have a close family member with scleroderma, you are at a very slightly higher risk of developing scleroderma than everyone else. But scleroderma is not a disease that you can pass directly to your children or inherit from your parents.5

It seems more likely that some people have a genetic ‘switch’ for scleroderma that is triggered by something. At the moment, most of the triggers are not known but sometimes there is a clue.6

For example, it’s likely that events that are very stressful on the body (like a physical injury, exposure to toxic chemicals, or mental stress) can alert your immune system and also change how your genes work. This could be the trigger for scleroderma in some people.5-­7, 12, 13

There isn’t a way to find out who will get scleroderma, or to know how it happened in those who have been diagnosed. Scientists are looking in to how scleroderma works, so we might know more in the future.


What could happen if I have scleroderma?

People are affected by scleroderma in different ways, so it can be difficult to predict how it will progress.5 Because you have connective tissue all through your body, scleroderma can affect any area.

Organs that are often affected by scleroderma include:6, 14-­16

  • The skin (9 out of 10 people)
  • The digestive system (9 out of 10 people)
  • The lungs (may be affected in 4 to 8 people out of 10)
  • Less commonly, the kidneys or heart are affected (1 in 10 people)

We know that around 1 in 4 people develop noticeable lung disease within 3 years of their scleroderma diagnosis, which is why your doctor will monitor you so that any lung symptoms can be identified early. 14, 17

A scientific study recently found that nearly half of all organs that were affected by scleroderma showed signs within 2 years of the start of the disease.18 This might give your doctor some idea of how your scleroderma might progress and which organs will be affected.

The most important thing is to report any changes in symptoms to your doctor or nurse. They will be able to help you discuss your options and address your concerns. They will also be able to make a plan with you for the appointments and tests you will need over the next few months so that you know what to expect.

While the symptoms can affect how you live your life, they don’t have to rule it.

There are many things you can do yourself to help manage your symptoms. Some of these are listed in the symptoms section of this website.

Your doctor or nurse will be able to help you manage your symptoms. They have the knowledge and experience to address any questions you have (or at least point you in the right direction), so don’t be afraid to ask.

Other people with scleroderma, just like you, may also have some great ideas and advice based on their own experience. There are recordings of people with scleroderma talking about their condition in the patient stories section on this website.

There is good evidence that people with scleroderma learn ways to adapt and manage their disease so that they can continue living their lives. A scientific survey of 1,902 people with all types and severities of scleroderma found that people who had lived with the disease for more than 11 years didn’t feel as negative about it as those who started having symptoms less than 5 years ago. Dr Frantz from the Rheumatology Department at the Cochin Hospital, Paris, and her team of colleagues from Europe and the United States, surveyed people with scleroderma in 60 different countries. They concluded that those living with the disease for longer probably found ways to adapt and work around many of their symptoms.19

Did you know?

This large international survey was completed by more than 1900 people with scleroderma from 60 countries. People were asked by their patient association or by their doctor if they would like to complete a online survey about which symptoms of scleroderma they had, how long they had them, and what they understood and felt about their disease and its impact on their lives.

References

  1. Derrett-­Smith EC, Denton CP. Systemic sclerosis: clinical features and management. Medicine 2010;38(2):109-­15.​

  2. Careta MF, Romiti R. Localized scleroderma: clinical spectrum and therapeutic update. An Bras Dermatol 2015;90(1):62-­73.

  3. Denton CP, Khanna D. Systemic sclerosis. 2017. The Lancet. In Press. Available at: http://thelancet.com/journals/lancet/article/PIIS0140-6736(17)30933-9/fulltext

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

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

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

  7. Pattanaik D et al. Pathogenesis of systemic sclerosis. Front. Immunol 2015;6:272.

  8. MacLeod AS. The innate immune system in acute and chronic wounds. Adv Wound Care 2016;5(2):65-­78.

  9. Strbo, N et al. Innate and adaptive immune responses in wound epithelialization. Adv Wound Care 2014;3(7):492-­501.

  10. Ramos PS et al. Genetics of systemic sclerosis: recent advances. Curr Opin Rheumatol. 2015;27:521-­29.

  11. Abraham D and Varga J. Scleroderma: from cell and molecular mechanisms to disease models. Trends Immunol. 2005;26(11):587-­95.

  12. Rubio-­Rivas M et al. Occupational and environmental scleroderma. Systemic review and meta-­analysis. Clin Rheumatol. 2017;36(3):569-­82.

  13. Chen Y. et al. Investigation of stressful life events in patients with systemic sclerosis. J Zhejiang Univ Sci B 2008;9(11):853-­6.

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

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

  16. 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.

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

  18. Jaeger VK 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.

  19. Frantz C et al. Impaired quality of life in systemic sclerosis and patient perception of the disease: a large international survey. Semin Arthritis Rheum. 2016;46(1):115-­23.

     

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