Introduction to Scleroderma

Risk Factors for Scleroderma

Researchers believe that scleroderma is a form of "autoimmune disease" that affects the body's connective tissue. The immune system, which normally protects the body against infection, becomes overactive in patients with scleroderma resulting in overproduction of collagen leading to excessive production of connective tissue or "scar" tissue in the body.

Although the exact cause of scleroderma remains unclear, there are certain conditions that appear to qualify as risk factors. A "risk factor" is anything that increases a person's likelihood of getting a disease or condition. Risk factors for scleroderma include:

• Female gender - scleroderma occurs approximately 3 times more frequently in women than in men.

• Infections - some researchers suspect that infections may "trigger" the process leading to the development of scleroderma. Studies are ongoing to learn more about the potential role of persistent viral and bacterial infections as a possible cause of scleroderma.

• Chemical exposure - environmental or occupational exposure to certain toxic chemicals such as epoxy resins, benzene and vinyl chloride, and silica dust.

• Genetic predisposition - genetic factors may play a role in scleroderma and some patients may be genetically predisposed to the development of this disease. Scleroderma, however, is not thought to be an inherited disease that is passed on from parents to their offspring.

• Microchimerism - the presence of a small number of circulating cells transferred from one individual to another. This transfer of low numbers of cells takes place naturally during pregnancy between the mother and the fetus and is known as fetomaternal microchimerism. Recent studies have demonstrated an increased number of "microchimeric" cells in the blood and tissue of patients with scleroderma. Because the incidence of scleroderma is higher following a woman's child bearing years, one theory that has been proposed suggests that left-over fetal circulating cells during pregnancy may trigger an autoimmune response.

• BCG and tetanus vaccination - morphea-like lesions have been reported to occur in rare cases after bacillus Calmette-Guerin (BCG) and tetanus vaccinations.

• Vitamin K injection - in rare cases, injection of vitamin K has been associated with the development of scleroderma.

• Radiation therapy - localized scleroderma is a rare side-effect of radiation therapy for cancer

• Vibration injury - a possible association between Raynaud's phenomenon and workers exposed to hand-arm vibration has been suggested by some reports in the medical literature.

• Appetite suppressants - use of appetite suppressants such as L-tryptophan has been associated with a syndrome referred to as eosinophilic fasciitis. This condition is similar to scleroderma but involves inflammation of the fascia instead of the skin and is not accompanied by Raynaud's phenomenon. It is believed to be due to a contaminant in some appetite suppressant formulations. Rapeseed oil has also been associated with a scleroderma-like syndrome.