Watts R A, Gonzalez-Gay M A, Lane S E, Garcia-Porrua C, Bentham G, Scott D G
Department of Rheumatology, Norfolk and Norwich Hospital, Norwich, UK.
Ann Rheum Dis. 2001 Feb;60(2):170-2. doi: 10.1136/ard.60.2.170.
The aetiopathogenesis of the primary systemic vasculitides (PSV) is unknown but includes both environmental and genetic factors. The development of classification criteria/definitions for PSV allows comparison of the epidemiology between different regions.
The same methods and the American College of Rheumatology (1990) criteria or Chapel Hill definitions were used to compare the epidemiology of Wegener's granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, and polyarteritis nodosa in Norwich (east England population 413 500) and Lugo (northwest Spain population 204 100). Patients with PSV were identified between 1 January 1988 and 31 December 1998.
Overall, the incidence of PSV in adults was almost equal in Norwich (18.9/million) and Spain (18.3/million). The incidence of Wegener's granulomatosis in Norwich (10.6/million) was greater than in Spain (4.9/million). There was a marked age-specific increase in incidence in Norwich with a peak age 65-74 years (52.9/million), but a virtually equal age distribution between ages 45 and 74 in Lugo (34.1/million). There was no significant increase with time in either population, or evidence of cyclical changes in incidence.
These data support the suggestion that environmental factors may be important in the pathogenesis of PSV.
原发性系统性血管炎（PSV）的病因发病机制尚不清楚，但包括环境和遗传因素。PSV分类标准/定义的制定有助于比较不同地区之间的流行病学情况。
采用相同方法及美国风湿病学会（1990年）标准或查珀尔希尔定义，比较诺里奇（英格兰东部，人口413500）和卢戈（西班牙西北部，人口204100）地区韦格纳肉芽肿、变应性肉芽肿性血管炎、显微镜下多血管炎和结节性多动脉炎的流行病学情况。在1988年1月1日至1998年12月31日期间确定PSV患者。
总体而言，诺里奇地区（18.9/百万）和西班牙（18.3/百万）成人PSV发病率几乎相等。诺里奇地区韦格纳肉芽肿发病率（10.6/百万）高于西班牙（4.9/百万）。诺里奇地区发病率随年龄有显著增加，65 - 74岁达到峰值（52.9/百万），而卢戈地区45至74岁年龄分布几乎相等（34.1/百万）。两地区发病率均未随时间显著增加，也没有发病率周期性变化的证据。
这些数据支持环境因素可能在PSV发病机制中起重要作用这一观点。
