Scleroderma

Scleroderma is a chronic connective tissue disease. The word “scleroderma” comes from two Greek words: “sclero” meaning hard, and “derma” meaning skin. Hardening of the skin is one of the most visible manifestations of this disease which is classified as one of the autoimmune rheumatic diseases. The disease often affects the internal organs with life-threatening consequences. Depending on the subtype of illness, scleroderma can damage the lungs, kidneys and gastrointestinal tract with grave results. In some cases, the joints and muscles are affected, resulting in a loss of mobility.

Scleroderma is actually a group of connective tissue disorders with similar symptoms. They are associated with thickened, hardened skin, fibrosis, inflammation, blood vessel degeneration, and tissue damage. Scleroderma may be localized or systemic.  Localized scleroderma affects the skin and, in some cases, the muscle beneath it. Systemic scleroderma can affect specific areas of the body or be widespread and cause dysfunction in organs throughout the body.

The symptoms and severity of scleroderma vary from one person to another and the course of the disease is often unpredictable. The number of women affected with scleroderma is disproportionately high with some estimates suggesting as many as four out of every five patients being female. The disease most often strikes between the ages of 20 and 50; however, children and those above age 50 across all ethnic groups are also affected. Scleroderma is relatively rare and its prevalence is somewhat difficult to determine. It is estimated that there are about 240 cases for every million people, with about 19 new cases per million people each year.

Currently, there is no way to prevent scleroderma and there is no cure. There are a number of treatments available to address the various conditions associated with scleroderma. There are different classes of drugs currently approved, either in the US or Europe, to treat various conditions. Presently, most treatments act to slow the progression of the disease and limit damage rather than truly arresting the disease. In addition, some of the drugs currently in use can have serious side effects.

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