What Doctors Don't Tell YouQ:An acquaintance of mine has scleroderma. What are the latest research findings for this? Is there any hope of a cure? It is a horrible disease. M G, Merstam, Surrey……
A:Scleroderma, which means “hard” skin, is an autoimmune disease in which the skin becomes fibrotic swollen, thickened and hardened and ultimately scarred due to rampant overproduction of the protein collagen. Initially, the tightened skin may occur rapidly, create a burning sensation due to the increase in pressure in the skin and rubbing against the muscles and tendons below it. Characteristically, thick waxy patches come and go and, if large enough, restrict movement. This fibrosis, which follows the chronic inflammation of the skin in scleroderma, may also affect and ultimately damage arteries, joints and internal organs like the lungs, the heart muscle, the esophagus and the kidneys. The concern is especially for lung tissue inflammation, which often necessitates immediate treatment with drugs. Anyone suffering this disease often has a mask-like appearance.
Scleroderma is strikes three times as many women as men, usually between 30 and 60. It’s also relatively common, affecting 100,000 people in the US alone.
Scleroderma may also be accompanied by vascular symptoms called Raynaud’s syndrome, where blood flow to the extremities is turned on and off, the extremities turn white and then purple blue or red, and the sufferer experiences tingling, burning and pain. It may also affect the blood vessels around the nerves of the esophagus, causing problems swallowing food. The repeated stop-start aspect of blood flow produces toxic byproducts of oxygen, which ultimately damage tissue.
Medicine tends to treat this puzzling triad of illnesses by treating the symptoms of each. For the fibrotic skin, particularly when it involves the chest or abdomen, doctors tend to prescribe Cuprimine (D-penicillamine) and methotrexate, an all purpose, highly potent drug often used in conditions like arthritis which started life as chemotherapy for lymphomas and leukemia. Nevertheless, no drug has yet been shown to correct skin hardness that is already there, only to attempt to stop the progression of the disease. Oral steroids are also used if there is inflammatory muscle disease. If doctors even suspect that there is lung fibrosis, they are usually quick to prescribe cyclophosphamide (Cyproxin).
All such drugs have a remarkable litany of side effects, as they wear another hat as chemotherapy. Methotrexate, for instance, can decrease your resistance to infection, cause mouth ulcers and nausea, anemia, black stools, blurred vision, changes in skin coloration, convulsions, hair loss, infertility, intestinal inflammation, kidney failure, paralysis (including complete paralysis), intestinal ulcers, bleeding and vomiting blood. Cytoxan (cyclophosphamide), another anti-cancer drug, used for breast cancer, leukemias and lymphomas, can cause bladder damage, inflamed colon, decreased sperm count, impaired wound healing, new tumour growth, temporary hair loss, prolonged or temporary sterility in men, failure to menstruate and yellowing of eyes and skin. Penicillamine’s listing in the US Physicians’ Desk Reference starts off with a large box encouraging doctors who plan to use this drug to thoroughly familiarize themselves with its toxicity and special dosage considerations. “Penicillamine should never be used casually,” it says. No wonder. As a last-ditch remedy for arthritis that hasn’t responded to anything else, penicillamine can kill patients who go on to develop aplastic anemia, agranulocytosis, thrombocytopenia, and other conditions. Serious blood or kidney adverse reactions can occur at any time. And of course the side effects of steroids, well known and otherwise, have been covered in depth in WDDTY (see vol 7 no 2).
Thankfully, not all treatments involve such life-or-death medication. Doctors also recommend that patients with scleroderma have occupational and physical therapy to prevent or help permanent contracture of the fingers, and to wear gloves while performing many physical tasks like washing the dishes, to avoid breaking the skin.
For Raynaud’s phenomenon, medicine tends to use medications that expand the blood vessels. The most common vasodilators include drugs like Procardia, also used if there are pulmonary artery problems. Procardia can cause lightheadedess, mood changes, headache, nausea, swelling of arms, legs, hands and feet, tremors, wheezing, blurred vision, abdominal pain, chest congestion, insomnia, general chest pain, impotence, joint pain, muscle and bone inflammation, sexual difficulties, shortness of breath, and pins and needles. It may cause muscle and bone inflammation, which may lead one to mistake a drug side effect for an effect of the illness.
Besides drugs, those with Raynaud’s should preserve body heat by dressing in multiple layers of clothing, and wearing gloves, hats and scarves.
Although medicine usually draws a blank about the cause of this illness, abnormal metal status is the latest and most interesting theory, according to a breakthrough discovery by researchers at Johns Hopkins University in a study of 60 patients. Antony Rosen, a researcher based at Johns Hopkins, headed up a group that found that the toxic oxygen products caused by interrupted blood flow damage tissue, but only when there is an abnormal amount of iron, copper, zinc or other metals present in the body. When the body treats these molecules as foreign invaders, scleroderma results (J Experimental Med, 1997; 185: 71-80). This means that environmental exposure to metal may be an important trigger, says Rosen (Lancet, 1997; 349: 107). Rosen says his team are now trying to understand the factors responsible for abnormal cellular metal accumulation. It may well be that even abnormal processing of minerals or other nutrient imbalances are ultimately found to be the culprit.
Besides these drugs, a number of other nutrients have shown promise in treating this puzzling condition. In one small experimental study, patients with sclerosis and patches of scleroderma improved after taking vitamin E supplements (Cutis, 1973; 11: 54-62). University of California at Los Angeles assistant professor and WDDTY panelist Dr Melvyn Werbach recommends that patients take 800 IU of vitamin E 15 minutes before meals, increasing to 1600 IU if needed and then decreasing to a maintenance dosage. He also notes that you should avoid taking iron at the same time (Nutritional Influences on Illness: A Sourcebook of Clinical Research, Third Line Press, Tarzana, California, 1996).
Werbach has also found that omega-6 fatty acids can help. In one study, four patients suffering from scleroderma for up to 13 years given 1 gram of EPO three times a day reported relief of pain in the extremities, improvement of skin texture, telangiectasia (widening of capillaries and small veins, a condition of this illness) (Br J Clin Pract, Nov/Dec 1985).
The B vitamin PABA may also soften the skin, says Werbach. In one study, 90 per cent of a group of 224 patients given PABA experienced skin softening (some markedly so), while less than a fifth of those not given the B vitamin experienced mild to moderate improvement (Clin Exp Rheumatol, 1988; 6: 261-68) results that were repeated in another double-blind study (Arthritis Rheum, 1966; 9: 495). In another study of 135 patients whose scleroderma was spread throughout their bodies, every patient experienced softening of the sclerotic skin, so long as treatment was continued for three months. Some patients experienced complete resolution of symptoms and were able to discontinue treatment. The only failure was a patient whose affected skin, which was rockhard, covered most of his body. PABA has also been shown to help longevity in these patients. In one study, those receiving the B supplement live significantly longer, with more than three-quarters living at least 10 years (J Clinical Epidemiology, 1988; 41: 193-204).
Besides these nutritional remedies, Dr Werbach knows of a number of herbal remedies known to soften scleroderma.
The enzyme bromelain obtained from the pineapple has anecdotal evidence of being able to reverse many of the effects of scleroderma; in one case, a patient was able to clench her fist, eat normally and sleep in a reclining position (J Natl Med Assoc, 1964; 56: 272-3). Trademarked derived fractions of the herb Centella asiatica (known as Gota kola) have been tested experimentally; in one case using the total triterpenoid fraction of Centella asiatica (TTFCA), the herb worked 85 per cent of the time (Acta Diabetol Lat, 1972, 52: 141-50).
Another substance called piascldine, which is an extract from avocado and soy bean oils, normally preserved in an alcohol solution (made by Pharmascience in France), has good evidence of improving symptoms of scleroderma and Raynaud’s phenomenon. In one study of 50 patients, half showed improvement in symptoms of scleroderma. All the patients with Raynaud’s syndrome had better finger movement and ability to adapt to the cold (J Presse Med, 1970; 78: 1235-6). Ginkgo biloba, vitamin E, magnesium, niacin and the elimination of any allergies can also help Raynaud’s (see Proof! vol 1 no 3).
Maryon Stewart
Daniel Redwood DC
Tom Ferguson MD