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Scleroderma: Rare autoimmune disease affects thousands

Posted Dec 02 2009 12:04am
CLEVELAND -- Scleroderma, a chronic autoimmune disorder affects about 12,000 Ohioans.

Autoimmune diseases are among the top ten causes of death for women. While you may have heard of Lupus, or Multiple Sclerosis, you may not be familiar with one that affects 300,000 Americans. It's called Scleroderma.

Scleroderma is a rare autoimmune disease that causes the body to attack itself and produce too much collagen. The most common sign of the disease is thickening of the skin. But the disease can affect several other parts of the body as well.

There's no known cause or cure and Scleroderma can be deadly. The more severe form can lead to hardening of the internal organs.

There are two types of Scleroderma. The systemic form affects the internal organs while the localized form mainly affects a local area of the skin.

Dr. Soumya Chatterjee, a rheumatologist at the Cleveland Clinic says about 1,900 patients from all over the country come to the Clinic for treatment.

Dr. Chatterjee explains that systemic Scleroderma is divided into into two forms: Limited (formerly known as CREST) and diffuse.

CREST is an acronym for extended symptoms of Limited Scleroderma:

•Calcinosis is abnormal calcium deposits in the skin.
•Raynaud's Phenomenom describes color changes to the hands and feet when exposed to cold or stress. Fingers and toes may turn red, white and then purple as blood vessels spasm.
•Esophageal Dysmotility is difficulty swallowing.
•Sclerodactyly is when the skin tightens on the fingers.
•Telengectasias is red spots on the skin.

The limited form of Scleroderma tends to have less severe organ involvement. The diffuse form is usually more aggressive.

There are many treatments available for various symptoms of Scleroderma but currently no cure. Research is underway looking for ways to slow the progression of the disease.
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