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Scleroderma

Description

An in-depth report on the causes, diagnosis, treatment, and prevention of scleroderma.

Alternative Names

Systemic sclerosis

Risk Factors

Scleroderma is uncommon -- it afflicts 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 more rare.

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 i s very low (less than 1%).

Genetics. Preliminary research suggests that patients with certain gene variations may be more susceptible to scleroderma than those who do not carry these variations.

Ethnicity. Limited data on risk by ethnic group in the United States suggests that the risk from highest to lowest is the following: Choctaw Native Americans (highest), African-Americans, Hispanics, Caucasians, Japanese Americans.

African-Americans have a higher rate of diffuse scleroderma, lung involvement, and a worse prognosis than Caucasians. Other studies also found lower survival rates among Japanese Americans. A 2003 study further reported that even though African-Americans with scleroderma tended to have more severe problems, they received poorer care than Caucasian patients at major medical centers.

Genetic factors affect population groups differently. Studies are finding, for instance, that ethnic groups differ in the number of specific scleroderma-related antibodies they produce. Caucasians, for instance, have a higher rate of anti-centromere antibodies, which are associated with limited disease, while African-American patients have higher rates of autoantibodies and genetic factors that are associated with a more severe condition.

Geography. There appears to be certain geographic clusters of scleroderma, or specific types of scleroderma related to geography. This may suggest an infectious or genetic factor at work, but the reasons are largely unknown. The following are some examples:

• Studies reported significantly higher than average scleroderma mortality rates in male patients (both African-American and Caucasian) who live in two specific regions of the Southeast: one cluster around Coffee, Tennessee, and two others near Northampton, North Carolina.
• A cluster of scleroderma cases has been observed in South Boston, Massachusetts.
• There is a higher incidence of scleroderma cases among Choctaw Native Americans in Oklahoma. In the case of the Choctaw Native Americans, the cluster appears to have a strong genetic component. In most cases of geographic clusters, however, the disease is unlikely to be inherited, and family members of patients are not at higher risk.

• Review Date: 12/14/2006
• Reviewed By: Harvey Simon, MD, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital

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