Prevalence of spontaneous scleroderma in the US is 0.026%, compared with 1.6% in families with systemic sclerosis.[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
The prevalence of systemic sclerosis varies widely, from 30 cases per million adults (New Zealand) to 443 cases per million adults (Canada), depending on the geographic location. More than one half of the patients with systemic sclerosis have the limited cutaneous systemic sclerosis (lcSSc) type, while approximately 40% of patients have the diffuse cutaneous systemic sclerosis (dcSSc) type.[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
The point prevalence in south-east Norway was found to be 9.9/100,000, and the estimated prevalences were 6.9/100,000 for lcSSc and 1.8/100,000 for dcSSc.[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
The prevalence in northern Europe is thought to be lower than that in southern Europe.[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