DESCRIPTION
Scleroderma, also known as systemic sclerosis, is an immune system disorder affecting the connective tissue. It is often classified with arthritis or rheumatological disorders because of the way it affects the connective tissue. A gene, called connective tissue growth factor (CTGF), has been found to be more common in people with scleroderma than in those unaffected by the disease. Environmental factors are thought to trigger the disease. These factors could be chemical agents in the home and work environment. Scleroderma is not a common disease.
Scleroderma causes damage to the cells lining the small arteries, producing rough scar-like tissue on the skin. Localized scleroderma affects only the skin on the hands and face, and generally does not spread any further. Systemic scleroderma can affect larger areas of the skin, and can also spread to the internal organs of the body.
The first symptom of scleroderma is Raynaud's Syndrome. This occurs due to the damage of the small arteries, particularly in the toes and fingers. Decreased blood circulation causes tingling or numbness in these extremities. The digits can also undergo color change, becoming pale, then bluish, and gradually returning to red as the chronic symptom of the syndrome passes and the body returns to normal. Raynaud's Syndrome is more common than scleroderma, so its occurrence does not necessarily indicate scleroderma.
All cases of scleroderma are characterized by changes in the skin. Scarring may appear on the hands. The skin becomes thick or hard. This condition may spread to the feet, face, elbows, knees and other areas of the body. The disease may progress to a certain point and not get any worse. It may lessen in severity and go away on its own.
If the disease continues to progress, however, the skin will continue to tighten and lose its ability to stretch. This can cause the underlying bones and tissue, particularly the toes and fingers, to lose their ability to move.
In some cases internal organ are affected. This occurs most frequently in the upper digestive tract. Tightening of tissue in the esophagus causes loss of the ability to contract those muscles normally, making it difficult to swallow. Heartburn and gastroesophageal reflux disorder can result. Many patients with systemic scleroderma also have stomach disorders and intestinal disorders which can lead to anemia.
The lungs may also be affected by systemic scleroderma. This can lead to pulmonary fibrosis and/or pulmonary hypertension. Systemic scleroderma with involvement of the lung can put the person at higher risk for lung cancer. Symptoms of lung involvement are shortness of breath and difficulty performing physical activities.
Other sites of involvement can be the kidneys and the heart. Kidney involvement frequently has lead to death, but management of hypertension has reduced that risk. Involvement of the heart tissue can lead to scarring, risking irregular heart beats, pericarditis and heart failure.
Scleroderma and its complications can have a great impact on one's life. Depression is common in many patients, especially because of changes in appearance, especially if the skin on the face is involved. Pain is common in over half the people affected by scleroderma. In most cases the disease progresses to a point and gets no worse and in some cases spontaneously heals. There are no specific tests or ways to quickly and certainly diagnose scleroderma. A clinical pattern called the "CREST" syndrome - standing for calcinosis cutitis, Raynaud's Syndrome, esophageal involvement, sclerodactyly and telangiectasia is most commonly used for diagnosis.
TREATMENT
There are no medical cures for scleroderma. Drugs such as immuno-suppressants are frequently prescribed to treat the symptoms of the disorder.
Many complementary measures are successful in treating scleroderma. As a first measure, the home and work environment should be checked for possible exposure to chemical agents. If silicon breast implants are in place, they should be removed. A detoxification program should be undertaken to rid the body of any stored-up toxins.
PABA, para-aminobenzoic acid has an antifibrotic action. PABA is often administered as KABA, potassium para-aminobenzoic acid. KABA at the rate of 12 grams per day gradually softens the skin and restores flexibility. Treatment might need to be continued indefinitely.
Vitamin E (200-1200 IU per day), along with having antifibrotic effects of its own, reduces the oxidative effects of scleroderma. Vitamin E can also be applied topically.
Vitamin D2 (10,000-12,000 IU per day) inhibits fidroblast growth. The bioactive equivalent of Vitamin D2, calcitrol, might be substituted.
Gamma-linoleic acid increases capillary blood flow and should be considered as a therapeutic supplement. Evening Primrose Oil is a good source of gamma-linoleic acid.
Estriol, one of the three main estrogens produced by the body is useful in post-menopausal women who've contracted the disease. Bromelain at 160mg per day has been shown to help in some cases. Zinc and copper deficiencies have been noted in scleroderma patients, and moderate supplementation of both minerals should accompany any scleroderma treatment protocol.
SOURCES
Klippel, John H.. Primer On the Rheumatic Diseases 11ED. Atlanta, GA: Arthritis Foundation.
Valentini G, Black C (2002). "Systemic sclerosis". Best practice & research. Clinical rheumatology 16 (5): 807–16
Jimenez S, Koenig AS. Scleroderma. eMedicine.com. Accessed: May 22, 2006.
Preliminary criteria for the classification of systemic sclerosis (scleroderma)
Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee" (1980). Arthritis Rheum. 23 (5): 581
Gaby, M.D. A.R. "Natural Remedies for Scleroderma" 2006
Alternative Medicine Review Volume 11, Number 3
Scleroderma Dorland's Medical Dictionary
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