What is Scleroderma?
Scleroderma (systemic sclerosis or SSc) is a largely misunderstood chronic disease affecting about approximately 22,000 Canadian and five to six times more women than men are affected by this disease.
Scleroderma is an 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).
This disease is derived from the Greek words “skleros” (hard) and “derma” (skin) and is characterized by progressive skin hardening.
Nicknamed “the disease that mummifies” scleroderma is diagnosed in thousands of Canadians. It 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.
In scleroderma, a protein called collagen is made in larger than normal amounts. This protein is important in many different body functions, including making up skin and blood vessels. The increase in collagen causes thickening of the skin and can cause scars to form in different internal organs including the lungs, esophagus, and kidneys. The organs involved may be different for different patients.
In most cases, scleroderma patients have difficulty with coordinating small movements of the hands and fingers. For example, patients may have trouble doing up buttons and writing.
Fast Facts about Scleroderma:
- Scleroderma is found throughout the world. About 4 in 10,000 people have scleroderma.
- Scleroderma is 5 to 6 times more common in women than men.
- The disease is most commonly diagnosed between ages 30-50, but it can also strike children and people of all age groups.
- Scleroderma is not contagious.
- 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.
- It is still unknown what exactly causes this autoimmune disease.
- Since each case is unique, no two patients will follow the same course of the disease