Norwich School of Medicine, University of East Anglia, Norwich Research Park, Norwich NR4 7TJ, UK.
Rheumatology (Oxford). 2012 May;51(5):926-31. doi: 10.1093/rheumatology/ker454. Epub 2012 Jan 17.
Granulomatosis with polyangiitis (Wegener's) (GPA) and microscopic polyangiitis (MPA) are uncommon and have unknown aetiology. The aim of the study was to investigate the epidemiology of GPA and MPA in a stable, well-defined population looking for differences in the pattern of occurrence, which might suggest a different aetiology.
Since 1988, we have maintained a prospective register of all patients with systemic vasculitis attending the Norfolk and Norwich University Hospital. Patients presenting with new-onset GPA and MPA as defined by the European Medicines Agency algorithm and registered with general practitioners in the former Norwich Health Authority area between 1988 and 2010 were identified. The population in 2008 was estimated to be 459 000 (221 000 males).
One hundred and eleven GPA and 58 MPA incident cases were identified during 1988-2010. The overall annual incidence of GPA and MPA was 11.3/million and 5.9/million, respectively. There was evidence of a cyclical pattern of occurrence with a periodicity of 7.6 years for GPA with a peak incidence of 28.3/million in 2005 and the lowest in 2002 (2.2/million). Other lesser peaks occurred in 1990 and 1996. While the peak incidence of MPA was in 2008 (15.2/million), there was no convincing evidence of periodicity. The incidence of cANCA/PR3- or pANCA/MPO-positive vasculitis showed a similar pattern to GPA and MPA, respectively.
This study lends support to the notion that the aetiology of GPA and MPA may be distinct conditions with different aetiologies. The cyclical incidence of GPA is possibly an indication for the influence of infection.
肉芽肿性多血管炎（韦格纳氏）（GPA）和显微镜下多血管炎（MPA）较为罕见，病因不明。本研究旨在调查一个稳定、明确的人群中 GPA 和 MPA 的流行病学，寻找发病模式的差异，这可能提示不同的病因。
自 1988 年以来，我们一直在维护一个在诺福克和诺维奇大学医院就诊的所有系统性血管炎患者的前瞻性登记。1988 年至 2010 年间，在诺福克前卫生局辖区内由欧洲药品管理局算法定义的新发病 GPA 和 MPA 并在全科医生处登记的患者被确定。2008 年的人口估计为 459000（221000 名男性）。
1988-2010 年间共发现 111 例 GPA 和 58 例 MPA 病例。GPA 和 MPA 的年发病率分别为 11.3/百万和 5.9/百万。发病存在周期性，GPA 为 7.6 年，2005 年发病率最高，为 28.3/百万，2002 年最低，为 2.2/百万。其他较小的高峰发生在 1990 年和 1996 年。虽然 MPA 的高峰发病年龄为 2008 年（15.2/百万），但没有明显的周期性证据。cANCA/PR3-或 pANCA/MPO 阳性血管炎的发病率与 GPA 和 MPA 分别相似。
本研究支持 GPA 和 MPA 的病因可能是不同的，具有不同的病因。GPA 的周期性发病率可能表明感染的影响。
