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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Systemic scleroderma


Other Names for this Disease

  • Progressive systemic sclerosis
  • Scleroderma, systemic
  • Systemic sclerosis
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Cause

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What causes systemic scleroderma?

The exact, underlying cause of systemic sclerosis is unknown. The cause appears to involve some injury to the cells that line blood vessels, resulting in excessive activation of dermal connective tissue cells, called fibroblasts. Fibroblasts normally produce collagen and other proteins.[1] Build-up of collagen in the skin and other organs causes the signs and symptoms of the condition.[2]

It is suspected that scleroderma may develop from a variety of factors, which may include:
  • Abnormal immune or inflammatory activity[3]
  • Genetic susceptibility: while no specific genes are thought to cause scleroderma, certain genes (or combination of genes) may increase a person's risk to be affected. However, the condition is not passed directly from parents to children.[3]
  • Environmental triggers: suspected triggers may include infections; injury; drugs (e.g. vitamin K, cocaine, penicillamine, appetite suppressants and some chemotherapeutic agents); and chemicals (e.g. silica, organic solvents, pesticides, aliphatic hydrocarbons and epoxy resin).[1][3]
  • Hormones: because women develop scleroderma more often than men, researchers suspect that hormones may play a role. However, the role of female hormones has not been proven.[3]

Widespread scleroderma can also occur in association with other autoimmune diseases, including systemic lupus erythematosus and polymyositis.[2]

Last updated: 4/9/2014

References
  1. Vanessa Ngan. Systemic sclerosis. DermNet NZ. December 29, 2013; http://dermnetnz.org/immune/systemic-sclerosis.html. Accessed 4/9/2014.
  2. Scleroderma. MedlinePlus. February 21, 2013; http://www.nlm.nih.gov/medlineplus/ency/article/000429.htm. Accessed 4/9/2014.
  3. Handout on Health: Scleroderma. NIAMS. August, 2012; http://www.niams.nih.gov/Health_Info/Scleroderma/default.asp#4. Accessed 4/9/2014.


Other Names for this Disease
  • Progressive systemic sclerosis
  • Scleroderma, systemic
  • Systemic sclerosis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.