较之系统性硬化症家庭中的患病率 1.6%,自发硬皮病在美国的患病率为 0.026%。[9]Englert H, Roberts-Thomson PJ, Byth K, et al. Familial scleroderma: nature, nurture or both? Intern Med J. 2008;38:235-242.http://www.ncbi.nlm.nih.gov/pubmed/18298553?tool=bestpractice.com[10]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;44:1359-1362.http://www3.interscience.wiley.com/cgi-bin/fulltext/83002209/HTMLSTARThttp://www.ncbi.nlm.nih.gov/pubmed/11407695?tool=bestpractice.com 系统性硬化症的患病率变化很大,从每百万成年人中有 30 例(新西兰)到每百万成年人中有 443 例(加拿大),这取决于地理位置。一半以上的系统性硬化症患者属于局限性皮肤系统性硬化症 (lcSSc) 型,而近 40% 的患者属于弥漫性皮肤系统性硬化症 (dcSSc) 型。[11]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;48:2246-2255.http://www3.interscience.wiley.com/cgi-bin/fulltext/104551157/HTMLSTARThttp://www.ncbi.nlm.nih.gov/pubmed/12905479?tool=bestpractice.com[12]Bernatsky S, Joseph L, Pineau CA, et al. Scleroderma prevalence: demographic variations in a population-based sample. Arthritis Rheum. 2009;61:400-404.http://onlinelibrary.wiley.com/doi/10.1002/art.24339/fullhttp://www.ncbi.nlm.nih.gov/pubmed/19248123?tool=bestpractice.com[13]Chifflot H, Fautrel B, Sordet C, et al. Incidence and prevalence of systemic sclerosis: a systematic literature review. Semin Arthritis Rheum. 2008;37:223-235.http://www.ncbi.nlm.nih.gov/pubmed/17692364?tool=bestpractice.com 经发现,挪威东南部的时点患病率为 9.9/100,000,而 lcSSc 和 dcSSc 的估计患病率分别为 6.9/100,000 和 1.8/100,000。[14]Hoffmann-Vold AM, Midtvedt Ø, Molberg Ø, et al. Prevalence of systemic sclerosis in south-east Norway. Rheumatology (Oxford). 2012;51:1600-1605.http://rheumatology.oxfordjournals.org/content/51/9/1600.longhttp://www.ncbi.nlm.nih.gov/pubmed/22539477?tool=bestpractice.com 据悉,北欧的患病率比欧洲南部低。[14]Hoffmann-Vold AM, Midtvedt Ø, Molberg Ø, et al. Prevalence of systemic sclerosis in south-east Norway. Rheumatology (Oxford). 2012;51:1600-1605.http://rheumatology.oxfordjournals.org/content/51/9/1600.longhttp://www.ncbi.nlm.nih.gov/pubmed/22539477?tool=bestpractice.com