What is the incidence of CREST syndrome in the US?

Updated: Oct 05, 2020
  • Author: Jeanie C Yoon, MD; Chief Editor: Dirk M Elston, MD  more...
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The incidence of systemic sclerosis approximates 2.7-19.3 new cases per million adults per year. The prevalence is 253-286 cases per million persons. [33] The highest prevalence has been reported in a Choctaw Native American Group in Oklahoma (660 cases per million, based on 14 cases). [34] The apparent increase in both incidence and prevalence over the past 50 years is most likely an artifact of better classification, earlier diagnosis, and improved survival. Some serum antibody studies suggest that CREST syndrome may account for 22-25% of all cases of systemic sclerosis; however, epidemiologic studies specifically looking at CREST syndrome are lacking. [33, 34, 35, 36]

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