This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
Systemic scleroderma is an 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 is caused by the body’s production of too much collagen, which normally strengthens and supports connective tissues. 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.
There are three types of systemic scleroderma, defined by the tissues affected in the disorder.
Treatment depends on the symptoms that are present and the organs that are affected in the disease, and may include immunosupressive therapy.
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