Diseases / Research

Scleroderma

Fred Hutch is developing innovative treatments for Scleroderma, and our scientists made a major breakthrough when they discovered that fetal cells in a mother's bloodstream—a condition called microchimerism—are associated with scleroderma development.

Fast Facts

  • Scleroderma is a chronic autoimmune disease, in which the body’s immune system mistakenly tries to destroy healthy cells.
  • Scleroderma's hallmark feature 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 damages multiple body parts, including the digestive tract, lungs, kidneys and joints.
  • 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 scleroderma's cause is unknown, genetic and environmental factors are thought to play a role. Scleroderma does not often run in families, but there may be certain gene variants that increase la person's risk for the disease.

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Prevention & Causes

Investigating scleroderma's mother-child link — Dr. J. Lee Nelson and colleagues made a major breakthrough when they discovered that fetal cells in a mother's bloodstream—a condition called microchimerism—are associated with the mother's development of scleroderma.

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 particularly important. 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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Treatment & Prognosis

 

Using stem cell transplants to treat scleroderma – Our researchers helped make hematopoietic stem-cell transplantation — the standard treatment for leukemia and other blood cancers — an established treatment for severe scleroderma.

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 >

Making transplants less toxic – Because systemic sclerosis patients do not always tolerate conventional transplants well, the Hutchinson Center is developing lower-intensity transplantation regimens, known as or non-myeloablative or "mini" stem-cell transplants. This treatment involves lower doses of chemotherapy and radiation, generally reducing toxic side effects. Learn more >

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