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What is Scleroderma?

Scleroderma (systemic sclerosis or SSC) is a little known and incurable autoimmune disease, a condition in which a person’s immune system turns against itself by producing antibodies that attack its own tissues (i.e. autoantibodies*).

Scleroderma affects approximately 2,300 people living in BC and 22,000 Canadians. Scleroderma is characterized by an overproduction of collagen and damage to the blood vessels that causes excessive scarring within the various organs.  This imbalance leads to a hardening of the skin (fibrosis) and an alteration of the affected organs. Moreover, in most cases, scleroderma limits the motor skills and dexterity of those affected and causes great physical and psychological suffering since it produces disfiguring appearance-related changes.

The skin, digestive system, heart, lungs, and kidneys are the organs most commonly affected by the potentially serious complications of scleroderma, which leads to significant decrease in quality of life and life expectancy.

 

Fast Facts about Scleroderma:

  • 2.7 million worldwide have scleroderma which is about 4 in 10,000 people.
  • Scleroderma is not contagious.
  • It is still unknown what exactly causes this autoimmune disease.
  • The disease is most commonly diagnosed between ages 30-50, but it can also strike children and people of all age groups.
  • Scleroderma is five to six times more common in women then men.
  • Both limited and diffuse scleroderma are associated with a significant reduction in life expectancy. For people who have mostly skin involvement and no major organs involved, the long-term outlook is more favourable.
  • Since each case is unique, no two patients will follow the same course of the disease.
  • This disease’s name is derived from the Greek words “skleros” (hard) and “derma” (skin) and is characterized by progressive skin hardening.

 

* Autoantibodies are antibodies produced by the immune system and directed against the body’s own cells.  Antibodies usually play a protective role against pathogens (which can cause a disease).  However, in the event of a dysfunction of the immune system, autoantibodies may appear and react with antigens (any substance recognized by the body as foreign, and which elicits an immune response aimed at eliminating it) contained in healthy tissues or organs of the body: it is autoimmunity.  Certain diseases such as scleroderma, called autoimmune disease, are responsible for pathological autoimmunity.