肉芽肿性多血管炎 (granulomatosis with polyangiitis, GPA)(以前称为韦格纳肉芽肿)的发病率和患病率因国家/地区而异。据报道,在美国患病率为 3/100,000,然而这一数字很可能被低估,因为该研究是基于医院的出院数据分析。[1]Cotch MF, Hoffman GS, Yerg DE, et al. The epidemiology of Wegener's granulomatosis. Estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources. Arthritis Rheum. 1996 Jan;39(1):87-92.http://www.ncbi.nlm.nih.gov/pubmed/8546743?tool=bestpractice.com 据报道,斯堪的纳维亚半岛的患病率更高,其中瑞典的患病率为 160/100 万。[2]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
GPA 可发生于任何年龄。在大多数病例中,平均发病年龄为 40-60 岁,性别分布基本相等。该病在白种人中最常见,但也可见于其他种族和民族的人群。斯堪的纳维亚半岛的研究表明发病率上升,但这可能(至少部分)是由于诊断率的提高。纬度靠北地区和纬度靠南地区的发病率似乎有所升高。[3]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[4]Knight A, Ekbom A, Brandt L, et al. Increasing incidence of Wegener's granulomatosis in Sweden, 1975-2001. J Rheumatol. 2006 Oct;33(10):2060-3.http://www.ncbi.nlm.nih.gov/pubmed/16960922?tool=bestpractice.com