What is scleroderma?
Scleroderma is a little-known and incurable autoimmune disease of as yet unknown cause that involves four main abnormalities: dysregulation of the immune system associated with the presence of very specific autoantibodies, inflammation, microvascular damage, and fibrosis.
Nicknamed “the disease that mummifies” scleroderma is diagnosed in thousands of Quebecers and 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.
Scleroderma affects approximately 22,000 Canadians and five to six times more women than men are affected by the disease. First symptoms usually appear in the early forties but can also occur in children and people of all ages. Scleroderma is not contagious.
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.
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.