SCLERODERMA

v  Scleroderma is an autoimmune condition of the connective tissue characterized by skin thickening, spontaneous scarring, blood vessel disease, and varying degrees of inflammation, associated with an overactive immune system. Autoimmune diseases are illnesses that occur when the body's tissues are attacked by its own immune system. Scleroderma is characterized by the formation of scar tissue (fibrosis) in the skin. This leads to thickness and firmness of involved skin. This may also occur in internal organs such as the kidney.

v  The cause of scleroderma is not known. Researchers have found some evidence that certain genes are important hereditary factors, but the environment seems to also play a role. The result is activation of the immune system in a susceptible individual, causing damage to the inner lining of tiny blood vessels and injury to tissues that result in scar tissue formation and the accumulation of excess collagen.

Antinuclear Antibody Test

v  in the blood, that have the capability of binding to certain structures within the nucleus of the cells. The nucleus is the innermost core within the body's cells and contains the DNA, the primary genetic material. ANAs are found in patients whose immune system may be predisposed to cause inflammation against their own body tissues. Antibodies that are directed against one's own tissues are referred to as autoantibodies. The propensity for the immune system to work against its own body is referred to as autoimmunity. ANAs suggest the possible presence of autoimmunity. Therefore, when they are detected in a patient's blood (referred to as a "positive" result), doctors will consider the possibility that an autoimmune illness exists in that patient. We normally have antibodies in our blood that repel invaders in our bodies, such as virus and bacteria microbes. Antinuclear antibodies (ANAs) are unusual antibodies, detectable

v  Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs. The disease can be either localized to the skin or involve other organs in addition to the skin. Symptoms may include areas of thickened skin, stiffness, feeling tired, and poor blood flow to the fingers or toes with cold exposure. One form of the condition, known as CREST syndrome, classically results in calcium deposits, Raynaud's syndrome, esophagealproblems, thickening of the skin of the fingers and toes, and areas of small dilated blood vessels.

Prognosis

v  The 5-year survival rate for scleroderma is about 85%, whereas the 10-year survival rate is less than 70%. This varies according to the subtype; for instance, persons with limited skin disease have a 10-year survival rate of 71%, whereas the outlook for patients with systemic scleroderma has generally improved over the years. Ten-year survival rates rose from 54% in 1972 to 66% in 2001 The major causes of death in persons with scleroderma are: pulmonary hypertension, pulmonary fibrosis and scleroderma renal crisis. People with scleroderma are also at a heightened risk for contracting cancers (especially liver, lung, haematologic and bladder cancers) and, perhaps, cardiovascular disease.