A prevalência de esclerodermia espontânea nos EUA é de 0.026%, em comparação com 1.6% em famílias com esclerose sistêmica.[8]Englert H, Roberts-Thomson PJ, Byth K, et al. Familial scleroderma: nature, nurture or both? Intern Med J. 2008 Apr;38(4):235-42.
http://www.ncbi.nlm.nih.gov/pubmed/18298553?tool=bestpractice.com
[9]Arnett FC, Cho M, Chatterjee S, et al. Familial occurrence frequencies and relative risks for systemic sclerosis (scleroderma) in three United States cohorts. Arthritis Rheum. 2001 Jun;44(6):1359-62.
https://onlinelibrary.wiley.com/doi/epdf/10.1002/1529-0131%28200106%2944%3A6%3C1359%3A%3AAID-ART228%3E3.0.CO%3B2-S
http://www.ncbi.nlm.nih.gov/pubmed/11407695?tool=bestpractice.com
A prevalência de esclerose sistêmica varia amplamente, de 30 casos por milhão em adultos (Nova Zelândia) a 443 casos por milhão em adultos (Canadá), dependendo da localização geográfica. Mais da metade dos pacientes com esclerose sistêmica apresenta esclerose sistêmica cutânea limitada (ESCL), enquanto aproximadamente 40% dos pacientes apresentam esclerose sistêmica cutânea difusa (ESCD).[10]Mayes MD, Lacey JV Jr., Beebe-Dimmer J, et al. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum. 2003 Aug;48(8):2246-55.
https://onlinelibrary.wiley.com/doi/epdf/10.1002/art.11073
http://www.ncbi.nlm.nih.gov/pubmed/12905479?tool=bestpractice.com
[11]Bernatsky S, Joseph L, Pineau CA, et al. Scleroderma prevalence: demographic variations in a population-based sample. Arthritis Rheum. 2009 Mar 15;61(3):400-4
http://onlinelibrary.wiley.com/doi/10.1002/art.24339/full
http://www.ncbi.nlm.nih.gov/pubmed/19248123?tool=bestpractice.com
[12]Chifflot H, Fautrel B, Sordet C, et al. Incidence and prevalence of systemic sclerosis: a systematic literature review. Semin Arthritis Rheum. 2008 Feb;37(4):223-35.
http://www.ncbi.nlm.nih.gov/pubmed/17692364?tool=bestpractice.com
Constatou-se que a taxa de prevalência pontual no sudeste da Noruega foi de 9.9/100,000 e as prevalências estimadas foram de 6.9/100,000 para ESCL e 1.8/100,000 para ESCD.[13]Hoffmann-Vold AM, Midtvedt Ø, Molberg Ø, et al. Prevalence of systemic sclerosis in south-east Norway. Rheumatology (Oxford). 2012 Sep;51(9):1600-5.
http://rheumatology.oxfordjournals.org/content/51/9/1600.long
http://www.ncbi.nlm.nih.gov/pubmed/22539477?tool=bestpractice.com
Acredita-se que a prevalência no norte da Europa seja menor que no sul da Europa.[13]Hoffmann-Vold AM, Midtvedt Ø, Molberg Ø, et al. Prevalence of systemic sclerosis in south-east Norway. Rheumatology (Oxford). 2012 Sep;51(9):1600-5.
http://rheumatology.oxfordjournals.org/content/51/9/1600.long
http://www.ncbi.nlm.nih.gov/pubmed/22539477?tool=bestpractice.com