Risk Factors

Scleroderma is uncommon and afflicts only about 150,000 Americans. The cause of scleroderma has not been determined and there are few specific risk factors. The incidence tends to be higher in certain groups, however.

Age. Systemic scleroderma usually develops between the ages of 35 and 55. Localized scleroderma is more common in children than adults, but is extremely rare even in the young age group. It occurs in between 0.2 and 0.4 per 100,000 people. Systemic scleroderma in children is even rarer.

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Gender. The incidence of scleroderma is three to eight times higher in women than in men. Of possible importance was a 2002 study reporting that the disease tended to be less severe in women who developed it in middle age after being pregnant. Women who had an earlier onset and did not have a history of pregnancy had a much higher rate of complications. This may reflect a different cause of the disease in these two groups. (It should be noted that pregnancy itself is not a risk factor for scleroderma.)

Family History. A family history is the strongest risk factor for scleroderma, but even among family members, the risk is very low (less than 1%).

Genetics. Preliminary research suggests that patients with certain gene variations, or genetic polymorphisms, may be more susceptible to scleroderma than those who do not carry the polymorphism. For example, a 2004 study showed that the IL-1alpha-889 polymorphism is more common in patients with scleroderma than in healthy people.