Whatmough Steven, Fernandez Sophie, Sweeney Niamh, Howell Laura, Dhaygude Ajay
Renal Department, Royal Preston Hospital, Preston, UK.
Faculty of Health and Wellbeing, University of Central Lancashire, Preston, UK.
Clin Kidney J. 2019 Feb;12(1):42-48. doi: 10.1093/ckj/sfy084. Epub 2018 Sep 11.
Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is a multisystem autoimmune disorder associated with significant morbidity and mortality. Approximately 80-90% of patients have circulating ANCAs. Long-term outcomes appear to be improving. This retrospective study analyses the incidence and patient outcomes over a period of 23 years at a single tertiary centre.
Outcomes of patients diagnosed with AAV between 1 January 1988 and 31 December 2010 were collected retrospectively. Data including patient demographics, age of diagnosis, dates of starting renal replacement therapy, death and biochemistry results were collected. Patients were divided into two cohorts (1988-99 and 2000-10) and analysed using Stata software (StataCorp, College Station, TX, USA).
A complete dataset was obtained for 273 patients. Of these patients, 101 were diagnosed between 1988 and 1999 while 172 were diagnosed between 2000 and 2010. The number of patients diagnosed with AAV increased from 2.2/million in 1988 to 10.3/million in 2010. A higher proportion of patients (56.4%) in the earlier cohort presented with creatinine >500 μmol/L compared with the later cohort (30.2%; P < 0.001). Overall patient survival improved significantly between the two cohorts. Cohort 1 had a median survival of 59 months compared with 125 months for Cohort 2 (P = 0.003).
This study shows that AAV is being diagnosed at an earlier stage, resulting in improved outcomes. This may be because of improvements in the management of AAV and chronic kidney disease.
抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）是一种多系统自身免疫性疾病，与显著的发病率和死亡率相关。约80 - 90%的患者循环血液中存在ANCA。长期预后似乎正在改善。这项回顾性研究分析了在一个单一的三级中心23年间AAV的发病率及患者预后情况。
回顾性收集1988年1月1日至2010年12月31日期间诊断为AAV的患者的预后数据。收集的数据包括患者人口统计学信息、诊断年龄、开始肾脏替代治疗的日期、死亡情况及生化检查结果。患者被分为两个队列（1988 - 1999年和2000 - 2010年），并使用Stata软件（美国德克萨斯州大学站市StataCorp公司）进行分析。
获得了273例患者的完整数据集。其中，101例在1988年至1999年间被诊断，172例在2000年至2010年间被诊断。被诊断为AAV的患者数量从1988年的2.2/百万增加到2010年的10.3/百万。与后一个队列（30.2%）相比，前一个队列中肌酐>500μmol/L的患者比例更高（56.4%；P < 0.001）。两个队列之间患者的总体生存率有显著提高。队列1的中位生存期为59个月，而队列2为125个月（P = 0.003）。
本研究表明，AAV的诊断时间提前，预后得到改善。这可能是由于AAV和慢性肾脏病管理方面的改善。
