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A variety of new treatments are currently being investigated for the treatment of systemic scleroderma in hopes of better controlling symptoms and slowing down the progression of the disease. Examples include:

• Interferons
• Tumor necrosis factor alpha blockers
• Halofuginone
• Plasmapharesis
• Photopheresis
• Autologous stem cell transplantation

Interferons

Interferons are a naturally occurring group of proteins that act as chemical messengers between cells of the immune system. Three types of interferons (alpha, beta, and gamma) have been discovered so far each of which plays an important role in immune-medicated reactions. Currently, interferons are used as immunotherapy for the treatment of some types of cancers and some autoimmune disease.

Recombinant gamma-interferon (e.g., Actimmune) represents a potentially effective therapy for scleroderma because it inhibits excessive production of collagen and may reduce fibrosis of organs in scleroderma. There is some evidence that it may also improve skin softening. In one study, the 5-year survival rate of patients with diffuse scleroderma who were treated with recombinant gamma-interferon was about 85%.

Tumor Necrosis Factor-Alpha Blockers

Drugs that block the action of tumor necrosis factor-alpha (TNF-alpha), a potent immune system mediator of chronic inflammation, are currently being used to treat a variety of chronic inflammatory diseases including Crohn's disease, psoriatic arthritis, and rheumatoid arthritis with good success. Because anti TNF-alpha blocking drugs can reduce or modify the chronic inflammatory response, there is significant interest in evaluating these drugs for the treatment of other chronic inflammatory conditions such as scleroderma. Examples of anti TNF-alpha blockers include:

• infliximab (Remicade)
• etanercept (Enbrel)
• adalimumab (Humira)

Halofuginone

Halofuginone is a drug that specifically inhibits collagen type I synthesis and is, therefore, a potentially effective treatment for systemic scleroderma. In March, 2000 the U.S. Food and Drug Administration (FDA) designated halofuginone as an "orphan drug" - a drug specifically developed to treat a rare disease that affects less than 200,000 people in the United States. Orphan drug designation also provides companies that are developing the drug with financial incentives (e.g., tax breaks) to develop and test the drug and bring it to market. Halofuginone has been shown to reduce collagen synthesis and fibrosis in animal models and shows promise as an antifibrotic drug for the treatment of systemic scleroderma.

Plasmapharesis

Plasmapheresis, also called "plasma exchange", involves removing blood from the patient and separating the plasma (fluid component of blood) from the blood cells. The plasma, containing immunologically active substances, is then discarded and is replaced with artificial plasma (fluid containing salts and proteins). The artificial plasma together with the blood cells is then returned to the patient's body. Plasmapheresis is performed with a continuous flow machine which removes the patient's blood from one arm and, after the plasma has been separated and replaced with artificial plasma, returns the "new" blood through the other arm. Several small studies have reported that regular plasmapharesis treatments may be effective for the treatment of patients with CREST syndrome, particularly in the early stages of the disease. One small study also reported that plasmapharesis appeared to slow down the progression of severe systemic scleroderma.

Photopheresis