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Scleroderma

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Description

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

Alternative Names

Systemic Sclerosis

Other Treatments

Interferons. Interferons include agents that are used in hepatitis. Such drugs have helped reduce liver scarring. Early research is suggesting interferon gamma (e.g., Actimmune) may reduce scarring in systemic sclerosis. In one early study, five year survival was 85% for patients with diffuse systemic sclerosis who took interferon gamma. In addition, skin softening improved in 40%.

Tumor-Necrosis Factor Modifiers. Tumor-necrosis factor (TNF) modifiers are major breakthroughs in the treatment of rheumatoid arthritis. They are genetically engineered to interfere with specific components 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).

Halofuginone. Halofuginone, a drug that inhibits the synthesis of collagen, is showing some promise in preventing scarring. The agent blocks production of certain collagen types involved in cell proliferation.

Minocycline. Very small studies are reporting gradual and progressive improvement in some patients who take low doses of the minocycline. Although this drug is an antibiotic, in low doses it has anti-inflammatory characteristics that may help inhibit skin symptoms.

Investigative Procedures

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

Autologous Stem-Cell Transplantation. Researchers are investigating possible benefit using transplantation of the patient's own stem cells (called an autologous transplantation). (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 four years. Results of a major study evaluating stem-cell transplants in severe scleroderma are expected in 2005.

Of note, there are significant risks with the procedure. The procedure is also not at all foolproof:

Transplantation does not work in all patients.
It cannot reverse all damage that may have been done during the disease process.
And, importantly 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.

Alternative Treatments and Diet

Some patients avoid high-fiber diets (which include fruits and vegetables) and so their diets may lack sufficient nutrients. Supplements, then, may be needed, but patients would do best to 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 (e.g., selenium, beta-carotene, vitamin C, vitamin E, and methionine). Studies have not reported much benefit from such supplements. In general, however, there have not been long-term trials and some may have been started to late in the course of the disease to have much effect.

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

Warnings on Alternative and So-Called Natural Remedies

Alternative or natural remedies are not regulated and their quality is not publicly controlled. In addition, any substance that can affect the body's chemistry can, like any drug, produce side effects that may be harmful. Even if studies report positive benefits from herbal remedies, the compounds used in such studies are, in most cases, not what are being marketed to the public. There have been a number of reported cases of serious and even lethal side effects from herbal products. In addition, some so-called natural remedies were found to contain standard prescription medication.

The following warnings are of particular importance for people with scleroderma:

Chinese Herbal Remedies. A 2002 study reported that a woman with CREST syndrome had developed slowly progressive kidney disease after taking Chinese herbs. Studies suggest that up to 30% of herbal patent remedies imported from China having been laced with potent pharmaceuticals such as phenacetin and steroids. And one study reported a significant percentage of such remedies containing toxic metals.

High-Dose Antioxidants. Some studies are now suggesting that excessive use of 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. And, 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 blunted 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 a number of inflammatory problems. Recently, there is evidence 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, there is an increased risk for bleeding at high doses and 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. It should be further noted that not all brands have any effect at all. In a 2002 study one-third of 26 brands tested did not contain enough active ingredients to provide any benefits at all.

The following website is building a database of natural remedy brands that it tests and rates. Not all are yet available (www.consumerlab.com). The Food and Drug Administration has a program called MEDWATCH for people to report adverse reactions to untested substances, such as herbal remedies and vitamins (800-332-1088).

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