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Scleroderma

Description

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

Alternative Names

Systemic sclerosis

Other Treatments

Interferons. Interferons include drugs that are used in hepatitis. Such drugs have helped reduce liver scarring. Early research is suggesting interferon gamma (for example, Actimmune) may reduce scarring in scleroderma. In one early study, 5-year survival was 85% for patients with diffuse systemic sclerosis who took interferon gamma. In addition, 40% of patients said their skin got softer. It should be noted, however, that interferon alpha appears to trigger the development of scleroderma in some people with hepatitis.

Tumor-Necrosis Factor Modifiers. Tumor-necrosis factor (TNF) modifiers are major breakthroughs in the treatment of rheumatoid arthritis. They interfere with specific parts of TNF, a powerful immune factor. Researchers believe they should be tested in other inflammatory conditions, including scleroderma. The current agents include infliximab (Remicade), etanercept (Enbrel), and adalimumab (Humira).

Halofuginon e. Halofuginone, a drug that slows down the synthesis of collagen, is showing some promise in preventing scarring. The drug blocks production of certain collagen types involved in cell production.

Minocycline. Although this drug is an antibiotic, in low doses it has anti-inflammatory characteristics that may help slow down skin symptoms. Small studies suggested it provides slow and progressive symptom improvement, but other studies reported that minocycline is not effective for systemic scleroderma.

Investigative Procedures

Blood Exchange (Plasmapheresis). Plasmapheresis is a process in which the liquid part of the blood, called plasma, is separated from blood cells. The procedure involves first withdrawing blood from the patient. The plasma, which contains the active immune factors, is discarded and replaced with other fluids. The blood is then returned to the patient. In a small 2001 study, this procedure appeared to slow down the course of severe progressive scleroderma. Other studies are underway.

Autologous Stem-Cell Transplantation. Researchers are investigating a possible benefit using transplantation of the patient's own stem cells (an autologous transplant). (Patients with autoimmune diseases cannot be given cells from donors.) Stem cells are the early forms for all blood cells in the body (including red, white, and immune cells). The transplant procedures introduce normal white blood cells that replace the abnormal autoimmune cells. The procedure has improved or stabilized systemic scleroderma in some patients, with remissions lasting up to 4 years. Initial results of ASTIS, a major study evaluating stem-cell transplants and high-dose immunosuppressive therapy in severe scleroderma, indicate that this combination has significant benefits with few toxic side effects. Additional research will compare stem cell transplants to monthly cyclophosphamide therapy.

There are significant risks with stem cell transplants:

• Transplantation does not work in all patients.
• It cannot reverse all damage that may have been done during the disease process.
• The treatment carries significant dangers. The mortality rate from the procedure itself is 10% in scleroderma patients, which is higher than in other patient groups who are given transplants.

Because the procedure has serious side effects, experts suggest that the best candidates would be those at high risk for complications from scleroderma. In general, such patients would have diffuse scleroderma whose first symptoms occurred within the previous three years and who have evidence of at least mild abnormalities in the heart, lungs, or kidney. In general, patients with advanced scleroderma would not be the best candidates, because their condition is usually stable. In such cases, the risks of the procedure would outweigh the risks from the disease.

A Phase I trial of reduced-intensity stem cell transplant showed the procedure is safe and appears effective. A reduced-intensity stem cell transplant does not destroy the bone marrow, and therefore avoids many of the risks associated with the full-strength procedure. In this trial of 10 patients, survival rate was 90% (9 out of 10 people). Seven out of 10 people (70%) showed no progression of the disease during follow-up examination. Patients' skin improved significantly, and their heart, lung, and kidney functions remained stable during the 12-month study.

Intermittent pneumatic compression (IPC) pump therapy . During this therapy, a band wrapped around the arm is inflated and deflated. This helps stimulate blood flow. Experts are studying the use of IPC pump therapy for patients with ulcers on their arms. Medications usually do not work for such ulcers, and amputation is usually the only option for such patients. However, a small pilot study has shown that IPC pump therapy for an average of 5 hours per day healed 96% of ulcers.

Intravenous immunoglobulin (IVIg). Animal studies have found that administration of IVIg, an agent that modifies the immune system, may reduce the severity of scleroderma and other autoimmune diseases. Early studies in patients with scleroderma found it can improve skin fibrosis.

Alternative Treatments and Diet

Some patients avoid high-fiber diets (which include fruits and vegetables) and so their diets may lack enough nutrients. Supplements may be needed, but patients should consult someone experienced in dietary conditions associated with scleroderma. Because of reports that oxygen-free radicals may play a role in the development of sclerosis, some researchers recommend taking antioxidant supplements (for example, selenium, beta-carotene, vitamin C, vitamin E, and methionine). Though studies have not reported much benefit from such supplements, there have not been long-term trials, and some studies may have been started too late in the course of the disease to have much effect.

Because of the difficulty of the disease, many patients are tempted to try high-dose supplements or other alternative treatments. It is very important to note that this approach is not without its dangers.

Herbs and Supplements Generally, manufacturers of herbal remedies and dietary supplements do not need FDA approval to sell their products. Just like a drug, herbs and supplements can affect the body's chemistry, and therefore have the potential to produce side effects that may be harmful. There have been numerous reported cases of serious and even deadly side effects from herbal products. Always check with your doctor before using any herbal remedies or dietary supplements. The following warnings are of particular importance for people with scleroderma: Chinese Herbal Remedies. A 2002 study reported that a woman with CREST syndrome developed slowly progressive kidney disease after taking Chinese herbs. Studies suggest that up to 30% of herbal remedies imported from China contain strong pharmaceuticals such as phenacetin and steroids. One study reported a large percentage of such remedies contain toxic metals. High-Dose Antioxidants. Some studies are now suggesting that using too much antioxidant supplements may interfere with other nutrients, or convert into pro-oxidants and become harmful. Of particular concern are studies that have found an increase in lung cancer and overall mortality rate among smokers who took beta-carotene supplements. Even more worrisome, in people with existing cancer, high doses of antioxidant vitamins, such as vitamins A, C, E, or beta carotene, may actually protect cancer cells (just as they do healthy cells). One small study found that high doses of antioxidants, including vitamins C and E, interfered with cholesterol-lowering drugs and reduced their effect. This study also supports other evidence that high doses of vitamin C may speed up atherosclerosis. Comfrey. Comfrey is an herbal remedy used for several inflammatory problems. Recent evidence indicates that comfrey can be toxic to the liver, and animal studies have reported a possible cancer risk. It is banned in Canada and other countries but is widely available in the US. Gingko. Although the risks for gingko appear to be low, the risk for bleeding goes up after taking it at high doses. There is also a risk of interaction with high doses of vitamin E anti-clotting medications. Commercial gingko preparations have also been reported to contain colchicine, an agent that can be harmful in pregnant women and people with kidney or liver problems. Moreover, some brands have no effect at all. In a 2002 study, one-third of 26 brands tested did not contain enough active ingredients to provide any benefit.

• 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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