Scleroderma or Systemic Sclerosis
Sclero from the Greek means hard and derma from the Latin means skin. Hardened skin is the visible manifestation of the disease. It is classified as a chronic, autoimmune, connective tissue disease.
Some researchers are of the opinion that a malfunction in the autoimmune system may be responsible for excess collagen production. Collagen is the main structural protein found in connective tissue which is a normal component of skin, blood vessels and the internal organs. This excess build up of collagen in the skin creates a hardened external skin appearance (localized scleroderma) while excess collagen causes fibrosis in the heart, blood vessels, lungs and muscles lining the GI tract (Systemic scleroderma or systemic sclerosis).
Symptoms may be visible such as when the skin is involved or invisible when internal organs are impacted. The hallmark of scleroderma is fibrosis (collagen fibrils), which is a process similar to scar tissue formation generally resulting from inflammation.
There are over 300,000 scleroderma patients in the USA, one third of which suffer from the systemic form of the disease.80% of scleroderma patients are women and can be found in every age group but are predominately found in the 25 to 55 age range.
Scleroderma is: NOT contagious, NOT infectious, NOT cancerous nor is it likely to be inherited even though it has been found that members of the same family exhibiting other autoimmune diseases are more susceptible to developing scleroderma.
The degree of severity is highly variable ranging from mild to life threatening. Presently the cause is unknown. Environmental factors may trigger or exacerbate the disease such as exposure to paint thinners, silica, certain drugs and pvc found in plastics. There is no cure nor are there any medications available to slow down disease progression which tends to follow three main avenues; the overproduction of collagen which is the basic component of scar tissue, an autoimmune process and blood vessel damage.
Four types of localized scleroderma exist and can be differentiated by the size of the skin patch, thickness, position and/or color: morphea, generalized morphea, linear scleroderma and “en coup de sabre”. Two types of systemic sclerosis have been noted: diffuse cutaneous scleroderma and limited scleroderma. Organs generally involved include the skin, lungs, kidneys, heart, gastrointestinal tract and the musculoskeletal system. Some of the more prominent features of scleroderma include; skin changes, raynaud’s phenomenon, and internal organ involvement.
In the case of the type 1 diabetic, glucose instability (brittleness) may be caused by scleroderma or exacerbated by this condition.
As we saw in the case of patients exhibiting gastroparesis, variations in glucose levels result from mal-absorption and delayed gastric emptying. These are common symptoms in systemic scleroderma patients as well. Likewise, small blood vessels are damaged and become narrowed and overly sensitive to cold temperatures resulting in Raynaud’s phenomena. Since scleroderma impacts both the gastrointestinal tract as well as the blood vessels of the circulatory system, uncontrollable and unpredictable glucose variability may result.
Even though scleroderma is not curable many of the symptoms can be improved with medication and lifestyle changes.
Symptoms include: dry cough, atherosclerosis, heart attack, stroke, peripheral vascular disease, high blood pressure, kidney failure, digestive problems.
Diagnosis – medical history, lab findings, skin biopsy – no single test can prove scleroderma’s presence. Chest x-rays, MRI and CT scan are used to assess damage to muscles and internal organs.
Treatment is focused on alleviating symptoms and reducing further complications as the disease progresses. Exercise has proven beneficial. Chemicals found in green tea may guard against fibrotic changes. Vitamin E studies suggest it may be useful in disease management. Gotu kola, a tropical herb, curcumin and vitamin D are being studied as a treatment for inflammatory and autoimmune diseases including scleroderma. There appears to be an intimate relationship between fibrotic changes and the presence of inflammatory T cells. Targeting T cells has shown some promise of improvement.
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