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Other Names for this Disease
- Progressive systemic sclerosis
- Scleroderma, systemic
- Systemic sclerosis
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autoimmune disorder that affects the skin and internal organs. It is characterized by the buildup of scar tissue (fibrosis) in the skin and other organs. The fibrosis results from the excess production of a tough protein called collagen, which normally strengthens and supports connective tissues throughout the body. The signs and symptoms of systemic scleroderma usually begin with episodes of Raynaud's phenomenon, which can occur weeks to years before fibrosis. This may be followed by puffy or swollen hands before the skin becomes thickened and hard. Fibrosis can also affect internal organs and can lead to impairment or failure of the affected organs. The most commonly affected organs are the esophagus, heart, lungs, and kidneys.Systemic scleroderma is an
There are three types of systemic scleroderma, defined by the tissues affected in the disorder.
Last updated: 1/30/2013
- Systemic scleroderma. Genetics Home Reference (GHR). September 2011; http://ghr.nlm.nih.gov/condition/systemic-scleroderma. Accessed 1/30/2013.
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