Department of Nephrology, General University Hospital and First Faculty of Medicine, Charles University in Prague, Prague, Czech Republic.
ERA-EDTA Registry, Department of Medical Informatics, Academic Medical Center, Amsterdam, the Netherlands.
Am J Kidney Dis. 2015 Oct;66(4):613-20. doi: 10.1053/j.ajkd.2015.03.025. Epub 2015 May 12.
This study describes the incidence and outcomes of European patients requiring renal replacement therapy (RRT) for kidney failure due to antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
Cohort study.
SETTING & PARTICIPANTS: 12 renal registries providing individual RRT patient data to the European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) Registry in 1993-2012 participated.
Cause of primary kidney disease: AAV (ie, granulomatosis with polyangiitis [Wegener] and microscopic polyangiitis) versus 3 separate matched control groups without AAV: (1) primary glomerulonephritis, (2) diabetes mellitus, and (3) disease other than diabetes mellitus as the cause of primary kidney disease, including glomerulonephritis (termed "nondiabetes").
Incidence, causes of death, and survival.
ERA-EDTA primary renal disease codes.
2,511 patients with AAV (1,755, granulomatosis with polyangiitis; 756, microscopic polyangiitis) were identified, representing an incidence of 1.05 per million population (pmp) for granulomatosis with polyangiitis (predominating in Northern Europe) and 0.45 pmp for microscopic polyangiitis (prevailing in Southern Europe). Kidney transplantation was performed in 558 (22.2%) patients with vasculitis. The 10-year probability for survival on RRT after day 91 was 32.5% (95% CI, 29.9%-35.1%) in patients with vasculitis. Survival on RRT after day 91 did not differ between AAV and matched nondiabetes patients. Patient and transplant survival after kidney transplantation, adjusted for time period and country, was better in AAV than in matched nondiabetes patients (HRs of 0.81 [95% CI, 0.67-0.99] and 0.82 [95% CI, 0.69-0.96], respectively).
No data for extrarenal manifestations, treatment, and relapses.
Geographical differences in the incidence of RRT for kidney failure due to granulomatosis with polyangiitis and microscopic polyangiitis copied their distribution in the general population. Overall survival on RRT after day 91 for patients with AAV was similar to that for patients with nondiabetes diagnoses. Our results suggest that patients with AAV are suitable candidates for kidney transplantation with favorable survival outcomes.
本研究描述了欧洲抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）导致的肾衰竭患者接受肾脏替代治疗（RRT）的发病率和结局。
队列研究。
1993 年至 2012 年，12 个肾脏登记处向欧洲肾脏协会-欧洲透析与移植协会（ERA-EDTA）登记处提供了个体 RRT 患者数据，参与了研究。
主要肾脏疾病的病因：AAV（即肉芽肿性多血管炎[韦格纳]和显微镜下多血管炎）与 3 个单独的无 AAV 对照组相比：（1）原发性肾小球肾炎，（2）糖尿病，和（3）除糖尿病以外的疾病是原发性肾脏疾病的病因，包括肾小球肾炎（称为“非糖尿病”）。
发病率、死因和生存率。
ERA-EDTA 原发性肾脏疾病代码。
共确定了 2511 例 AAV（1755 例，肉芽肿性多血管炎；756 例，显微镜下多血管炎）患者，肉芽肿性多血管炎的发病率为每百万人口 1.05 例（pmp）（在北欧占主导地位），显微镜下多血管炎的发病率为 0.45 pmp（在南欧占主导地位）。血管炎患者中有 558 例（22.2%）接受了肾移植。血管炎患者第 91 天后开始 RRT 的 10 年生存率为 32.5%（95%CI，29.9%-35.1%）。第 91 天后 RRT 的生存率在 AAV 和匹配的非糖尿病患者之间没有差异。调整了时间和国家因素后，肾移植后患者和移植的生存率在 AAV 患者中优于匹配的非糖尿病患者（HR 分别为 0.81（95%CI，0.67-0.99）和 0.82（95%CI，0.69-0.96））。
没有关于肾外表现、治疗和复发的数据。
肉芽肿性多血管炎和显微镜下多血管炎导致的肾衰竭患者接受 RRT 的发病率的地理差异与它们在普通人群中的分布相吻合。AAV 患者第 91 天后开始 RRT 的总体生存率与非糖尿病患者相似。我们的结果表明，AAV 患者是适合进行肾移植的候选者，具有良好的生存结果。
