Causes

The disease process leading to scleroderma appears to occur as an autoimmune response, in which a defective immune system attacks or sets conditions that damage the body itself. In scleroderma, this response produces inflammation and an overproduction of collagen, the tough fibrous protein that helps construct connective tissues, such as tendons, bones, and ligaments as well as scar tissue. Most likely this disease process is regulated by a number of genetic defects with environmental factors playing the role of trigger. Research published in 2005 also revealed that the growth of new blood vessels (angiogenesis) is abnormal or impaired in people with scleroderma, particularly those with pulmonary vascular disease.

Inflammatory Response and Autoimmunity

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The Normal Immune System Response. The inflammatory process is a byproduct of the body's immune system, which fights infection and heals wounds and injuries:

• When an injury or an infection occurs, white blood cells are mobilized to rid the body of any foreign proteins, such as a virus.
• The masses of blood cells gather at the injured or infected site to perform various functions. They include selecting and destroying harmful substances, scavenging and ingesting the debris, and healing any injuries to the tissue by forming clots and scar tissue.
• In the process, the surrounding area becomes inflamed and some healthy tissue is injured.
• Under normal conditions, the immune system has other factors that control and limit this inflammatory process.