Scleroderma

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Scleroderma (systemic sclerosis): Fast Facts

• Scleroderma is a chronic autoimmune disease, in which the body’s immune system mistakenly targets healthy cells for destruction.
• The hallmark feature of scleroderma is hardening of the skin. Scleroderma can range from a localized disease that primarily affects the skin to a more serious illness, known as systemic sclerosis, that causes damage to multiple parts of the body, including the joints, digestive tract, lungs and kidneys.
• Like many other autoimmune diseases, scleroderma occurs much more frequently in women than men, with about four times as many women as men developing the disease.
• Although the cause of scleroderma is unknown, genetic and environmental factors are thought to play a role in the disease. Scleroderma does not often run in families, but there may be certain gene variants that increase likelihood of the disease.

Learn more about autoimmune diseases at the Seattle Cancer Care Alliance.

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Scleroderma: Some of our Key Research

Using transplantation to treat severe autoimmune diseases
Dr. Richard Nash and colleagues are leading clinical trials to examine the feasibility of high-dose chemotherapy and stem-cell transplantation — the standard treatment for leukemia and other blood cancers — in treating severe forms of autoimmune diseases, including multiple sclerosis, systemic lupus erythematosus and scleroderma (also known as systemic sclerosis). Their early results have been promising, prompting larger studies.

With transplantation, it may be possible to remove the reactive cells that trigger the immune system to attack the body. In such a transplantation, a patient's stem cells are collected, and cells that react against the patient's own tissue are removed. Next, the patient undergoes high-dose chemotherapy and takes drugs to suppress their immune system. The patient then receives an infusion of the stem cells that were collected before treatment, with the goal of rebuilding a new, healthier immune system. Learn more. »

Because systemic sclerosis patients do not always tolerate conventional transplants well, Dr. V.K. Gadi is studying the effectiveness of employing a lower-intensity transplantation regimen, known as a "mini" or non-myeloablative stem-cell transplant. This radically different treatment, pioneered at the Hutchinson Center, involves lower doses of chemotherapy and radiation, generally reducing toxic side effects.

Investigating mother-child cell exchange and scleroderma's origins
Dr. J. Lee Nelson and colleagues made a major breakthrough when they discovered in 1998 that trace fetal cells persisting in a mother's bloodstream years after pregnancy--a condition called microchimerism--are associated with the mother's development of scleroderma.

One of the observations that led Nelson to test the hypothesis that fetal cells are involved in scleroderma was the clinical similarities between scleroderma and chronic graft-versus-host disease, which can occur after transplantations as a result of an incompatibility of the donor's immune cells and the patient's tissues. Nelson reasoned that a similar process might be involved with rogue fetal cells in women with scleroderma. Fetal cells generally share only part of their genes with the mother, so they are partly genetically foreign.

Nelson's team has shown that there are more fetal cells in women with scleroderma than in healthy women, and that some of the fetal cells are immune system cells. They have also found that among the cell-surface proteins that form the identification system of the immune system, molecules expressed on the cell surface—called human leukocyte antigens (HLA-DRB1)—were of particular importance. Certain relationships of these molecules on the persisting fetal cells and the mother's cells were associated with an increased risk of subsequent scleroderma in mothers studied. Learn more. »

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Scleroderma: More Resources

• Advances in disease treatment in recent years have come out of clinical trials that test innovative medical approaches on patients. Find out more about clinical trials on scleroderma. »

• The Hutchinson Center conducts ongoing studies that depend on participation of healthy volunteers. Learn more about getting involved in research studies. » 

• The Hutchinson Center is on a mission to eliminate cancer and related diseases as causes of human suffering and death, and you can help. Make a gift today. »

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