随着时间进展,结节性多动脉炎 (PAN) 已不常见,主要是因为有效的乙肝病毒 (HBV) 免疫计划、改良的乙肝病毒血液筛查技术以及对于血管炎定义和分类的重大改变。 在 1994 年教会山共识会议 (CHCC) 定义之前,显微镜下多血管炎被纳入 PAN 发病率和患病率。 在一项比较欧洲三个地区 PAN 发病率的研究中,特别强调了显微镜下多血管炎的影响:与 CHCC 定义的 0-0.9 /100万发病率相比,美国风湿病协会 (ACR) 的标准是 4.4-9.7/100万。[10]Watts RA, Lane SE, Scott DG, et al. Epidemiology of vasculitis in Europe. Ann Rheum Dis. 2001 Dec;60(12):1156-7.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1753455/pdf/v060p01156a.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/11760724?tool=bestpractice.com
根据美国风湿病协会 (ACR) 的标准,欧洲和美国结节性多动脉炎的发病率为每年 2-9/100万。[11]Watts RA, Scott DG. Epidemiology of vasculitis. In: Bridges L, Ball G, eds. Vasculitis. Oxford, UK: Oxford University Press; 2008:7-22. 据报道,某些人群发病率较高:科威特的发病率为每年 16/100万(根据 CHCC 定义)[12]el-Reshaid K, Kapoor MM, el-Reshaid W, et al. The spectrum of renal disease associated with microscopic polyangiitis and classic polyarteritis nodosa in Kuwait. Nephrol Dial Transplant. 1997;12:1874-1882.http://ndt.oxfordjournals.org/cgi/reprint/12/9/1874.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/9306338?tool=bestpractice.com ,阿拉斯加的发病率为每年 77/100 万,阿拉斯加是乙型肝炎病毒感染流行区(这是一项早于 ACR 标准和 CHCC 定义的研究)。[13]McMahon BJ, Heyward WL, Templin DW, et al. Hepatitis B-associated polyarteritis nodosa in Alaskan Eskimos: clinical and epidemiologic features and long-term follow-up. Hepatology. 1989;9:97-101.http://www.ncbi.nlm.nih.gov/pubmed/2562798?tool=bestpractice.com 根据 ACR 标准,西欧国家结节性多动脉炎的患病率是 31-33/100万。[14]Koldingsnes W, Nossent H. Epidemiology of Wegener's granulomatosis in northern Norway. Arthritis Rheum. 2000 Nov;43(11):2481-7.https://onlinelibrary.wiley.com/doi/pdf/10.1002/1529-0131%28200011%2943%3A11%3C2481%3A%3AAID-ANR15%3E3.0.CO%3B2-6http://www.ncbi.nlm.nih.gov/pubmed/11083271?tool=bestpractice.com[15]Mahr A, Guillevin L, Poissonnet M, et al. Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum. 2004;51:92-99.http://www.ncbi.nlm.nih.gov/pubmed/14872461?tool=bestpractice.com[16]Mohammad AJ, Jacobsson LT, Mahr AD, et al. Prevalence of Wegener's granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden. Rheumatology (Oxford). 2007 Aug;46(8):1329-37.https://academic.oup.com/rheumatology/article/46/8/1329/1786473http://www.ncbi.nlm.nih.gov/pubmed/17553910?tool=bestpractice.com 根据 CHCC 定义,德国结节性多动脉炎的患病率是 2-9/100 万。[17]Reinhold-Keller E, Zeidler A, Gutfleisch J, et al. Giant cell arteritis is more prevalent in urban than in rural populations: results of an epidemiological study of primary systemic vasculitides in Germany. Rheumatology (Oxford). 2000;39:1396-1402.http://rheumatology.oxfordjournals.org/cgi/content/full/39/12/1396http://www.ncbi.nlm.nih.gov/pubmed/11136884?tool=bestpractice.com 目前尚无美国患病率数据。
结节性多动脉炎可发生于任何年龄,最常见于 40-60 岁。 尽管大部分病例系列报告表明男性更易患上结节性多动脉炎,但是并没有明显的性别差异。[15]Mahr A, Guillevin L, Poissonnet M, et al. Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum. 2004;51:92-99.http://www.ncbi.nlm.nih.gov/pubmed/14872461?tool=bestpractice.com[18]Guillevin L, Lhote F, Gayraud M, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome: a prospective study in 342 patients. Medicine (Baltimore). 1996 Jan;75(1):17-28.http://www.ncbi.nlm.nih.gov/pubmed/8569467?tool=bestpractice.com[19]Guillevin L, Mahr A, Callard P, et al. Hepatitis B virus-associated polyarteritis nodosa: clinical characteristics, outcome, and impact of treatment in 115 patients. Medicine (Baltimore). 2005;84:313-322.http://www.ncbi.nlm.nih.gov/pubmed/16148731?tool=bestpractice.com 然而,瑞典近期的患病率估计得出了相反的结果,发现 2/3 的患者为女性。[16]Mohammad AJ, Jacobsson LT, Mahr AD, et al. Prevalence of Wegener's granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden. Rheumatology (Oxford). 2007 Aug;46(8):1329-37.https://academic.oup.com/rheumatology/article/46/8/1329/1786473http://www.ncbi.nlm.nih.gov/pubmed/17553910?tool=bestpractice.com 在巴黎多种族人群中,具有欧洲血统的人群结节性多动脉炎患病率较高。[15]Mahr A, Guillevin L, Poissonnet M, et al. Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum. 2004;51:92-99.http://www.ncbi.nlm.nih.gov/pubmed/14872461?tool=bestpractice.com