What causes scleroderma?

Updated: Nov 13, 2018
  • Author: Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon); Chief Editor: Herbert S Diamond, MD  more...
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Answer

The exact etiology of systemic sclerosis is not known. Systemic sclerosis is not inherited, although a genetic predisposition plays an important role in its development. Environmental factors (eg, triggers or accelerators) may contribute to the development of systemic sclerosis in the proper genetic background. [15, 16, 17, 18, 19] These include the following:

  • Silica exposure

  • Solvent exposure (vinyl chloride, trichloroethylene, epoxy resins, benzene, carbon tetrachloride)

  • Radiation exposure or radiotherapy

Case reports describe the development of scleroderma in patients treated with the immune checkpoint inhibitor pembrolizumab for metastatic melanoma. [20]  

Cytomegalovirus, human herpesvirus 5, and parvovirus B19 have been proposed as viral accelerating factors. However, evidence of their involvement is inconclusive. [21]


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