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Causes of Scleroderma

Scleroderma literally means "thick skin." Individuals with scleroderma can develop thickening of their skin, on the outside of their body, but also in the tissues within their body. The condition is uncommon, affecting approximately 300,000 people in the population.

The cause of scleroderma remains unclear, but it is thought to be a problem with the immune system, in which collagen (a protein found in connective tissue) is deposited in unusually high amounts throughout the body. This process can affect the blood vessels, in particular, but can also disrupt the GI tract.

Although scleroderma is usually managed by a rheumatologist, it sometimes comes to the attention of gastroenterologists due to its strong impact on the bowels. Treatment of scleroderma can be complex and difficult, and typically involves potent therapies to depress the immune system, such as steroids, methotrexate, and other drugs. 

Types of Scleroderma

Scleroderma is a progressive condition that presents in many different ways; it is not a "one size fits all" disorder. Rheumatologists divide scleroderma into different types, including localized disease (affecting certain areas of the body only) and systematic sclerosis (SSc).

SSc is a form of scleroderma that has skin and internal organ involvement, including the GI tract. Localized scleroderma does not affect internal organs.

However, GI involvement occurs in approximately 90-95% of patients with SSc and is the leading cause of morbidity and the third most common cause of mortality in SSc.

From here on in this article, scleroderma refers to SSc.

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