Objectives The incidence of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) shows disparate results due to variable classification criteria and heterogeneous population series. We aimed to estimate the incidence of AAV in a well-defined population with standardized classification criteria. Methods This was a population-based study of AAV patients diagnosed from January 2000 to December 2023 in Cantabria, northern Spain. Patients were classified according to ACR/EULAR 2022 into granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), or unclassified vasculitis if the criteria were not met. Eosinophilic granulomatosis with polyangiitis patients were not included. The annual incidence rates were estimated by cases over 1 000 000 (106) (95% CI) including overall AVV, type of AAV, sex and year of diagnosis. A literature review was also performed. Results We included 152 patients [80M/72F; mean age 70.6 (13.18) years]. They were classified as MPA (67; 44%), GPA (64; 42.2%), and unclassified vasculitis (21; 13.8%). Annual incidence was 13.4 (95% CI: 10, 16.8)/106 [male 14.5 (95% CI: 10.5, 18.5); female 12.1 (95% CI: 8.7, 15.6)]. The Annual incidence of MPA was 5.9 (95% CI: 4, 7.8)/106 and GPA 5.6 (95% CI: 3.9, 7.3)/106. The mean annual incidence increased from 6.1 (95% CI: 4.5, 7.7)/106 to 16.5 (595% CI: .6, 27.4)/106 in the last 3 years, particularly in GPA from 2.3 (95% CI: 0.3, 4.9)/106 to 8.2 (95% CI: 2, 14.5)/106. The prevalence of AAV was 184.7 (95% CI: 181, 188)/106. Conclusion During a 20-year period we found that the incidence of AAV (GPA and MPA) in northern Spain was higher than in southern Spain, but lower than northern European countries. An increase in the incidence was observed in the last years.

中文翻译：

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西班牙北部 ANCA 血管炎的流行病学


目的 由于不同的分类标准和异质性人群系列，抗中性粒细胞胞浆抗体 （ANCA） 相关血管炎 （AAV） 的发病率显示出不同的结果。我们旨在使用标准化分类标准估计定义明确的人群中 AAV 的发生率。方法 这是一项针对 2000 年 1 月至 2023 年 12 月在西班牙北部坎塔布里亚诊断的 AAV 患者的人群研究。如果不满足标准，根据 ACR/EULAR 2022 将患者分为肉芽肿性多血管炎 （GPA） 、显微镜下多血管炎 （MPA） 或未分类的血管炎。嗜酸性肉芽肿性多血管炎患者未被纳入。年发病率通过超过 1 000 000 （106） （95% CI） 的病例估计，包括总体 AVV、AAV 类型、性别和诊断年份。还进行了文献综述。结果我们纳入了 152 例患者 [80M/72F;平均年龄 70.6 （13.18） 岁]。他们被分为 MPA （67; 44%）、GPA （64; 42.2%） 和未分类的血管炎 （21; 13.8%）。年发病率为 13.4 （95% CI： 10， 16.8）/106 [男性 14.5 （95% CI： 10.5， 18.5）;女性 12.1 （95% CI： 8.7， 15.6）]。MPA 的年发病率为 5.9 （95% CI： 4， 7.8）/106 和 GPA 5.6 （95% CI： 3.9， 7.3）/106。在过去 3 年中，平均年发病率从 6.1 （95% CI： 4.5， 7.7）/106 增加到 16.5 （595% CI： .6， 27.4）/106，尤其是 GPA 从 2.3 （95% CI： 0.3， 4.9）/106 增加到 8.2 （95% CI： 2， 14.5）/106。AAV 的患病率为 184.7 （95% CI： 181， 188）/106。结论 在 20 年期间，我们发现西班牙北部的 AAV （GPA 和 MPA） 发病率高于西班牙南部，但低于北欧国家。在过去几年中观察到发病率增加。