BACKGROUND More effective and safer treatments are needed for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. METHODS We conducted a randomized trial with a 2-by-2 factorial design to evaluate the use of plasma exchange and two regimens of oral glucocorticoids in patients with severe ANCA-associated vasculitis (defined by an estimated glomerular filtration rate of <50 ml per minute per 1.73 m2 of body-surface area or diffuse pulmonary hemorrhage). Patients were randomly assigned to undergo plasma exchange (seven plasma exchanges within 14 days after randomization) or no plasma exchange (control group). Patients were also randomly assigned to follow either a standard-dose regimen or a reduced-dose regimen of oral glucocorticoids. Patients were followed for up to 7 years for the primary composite outcome of death from any cause or end-stage kidney disease (ESKD). RESULTS Death from any cause or ESKD occurred in 100 of 352 patients (28.4%) in the plasma-exchange group and in 109 of 352 patients (31.0%) in the control group (hazard ratio, 0.86; 95% confidence interval [CI], 0.65 to 1.13; P = 0.27). The results were similar in subgroup analyses and in analyses of secondary outcomes. We also assessed the noninferiority of a reduced-dose regimen of glucocorticoids to a standard-dose regimen, using a noninferiority margin of 11 percentage points. Death from any cause or ESKD occurred in 92 of 330 patients (27.9%) in the reduced-dose group and in 83 of 325 patients (25.5%) in the standard-dose group (absolute risk difference, 2.3 percentage points; 90% CI, -3.4 to 8.0), which met the criterion for noninferiority. Serious infections at 1 year were less common in the reduced-dose group than in the standard-dose group (incidence rate ratio, 0.69; 95% CI, 0.52 to 0.93), but other secondary outcomes were similar in the two groups. CONCLUSIONS Among patients with severe ANCA-associated vasculitis, the use of plasma exchange did not reduce the incidence of death or ESKD. A reduced-dose regimen of glucocorticoids was noninferior to a standard-dose regimen with respect to death or ESKD. (Funded by the U.K. National Institute for Health Research and others; PEXIVAS Current Controlled Trials number, ISRCTN07757494; ClinicalTrials.gov number, NCT00987389.).

中文翻译：

---

严重ANCA相关性血管炎中的血浆置换和糖皮质激素。

背景技术抗中性粒细胞胞浆抗体（ANCA）相关血管炎需要更有效和更安全的治疗。方法我们采用2乘2析因设计进行了一项随机试验，以评估血浆置换和两种方案对严重ANCA相关性脉管炎（定义为肾小球滤过率<50 ml /分钟）的患者使用口服糖皮质激素的情况。每1.73平方米的体表面积或弥漫性肺出血）。随机分配患者进行血浆置换（随机分组后14天内7次血浆置换）或不进行血浆置换（对照组）。还随机分配患者遵循口服糖皮质激素的标准剂量方案或减量方案。对因任何原因或终末期肾脏疾病（ESKD）死亡的主要复合终点患者进行了长达7年的随访。结果血浆置换组352名患者中有100例（28.4％）死于任何原因或ESKD，对照组352名患者中109例（109％）死于任何原因（危险比，0.86； 95％置信区间[CI]） ，0.65至1.13； P = 0.27）。在亚组分析和次要结局分析中，结果相似。我们还以11个百分点的非劣效性边界评估了糖皮质激素减少剂量方案与标准剂量方案的非劣效性。降低剂量组的330例患者中有92例（27.9％）因任何原因或ESKD死亡，而标准剂量组325例的83例患者中有83例（25.5％）（绝对风险差异为2.3个百分点； CI为90％） ，-3.4至8.0），符合非自卑的标准。减量组在1年时发生严重感染的情况少于标准剂量组（发生率，0.69； 95％CI，0.52至0.93），但两组的其他继发结局相似。结论在严重的ANCA相关血管炎患者中，血浆置换的使用不能降低死亡或ESKD的发生率。就死亡或ESKD而言，糖皮质激素的减量方案不逊于标准剂量方案。（由英国国家卫生研究院等资助； PEXIVAS当前对照试验编号：ISRCTN07757494； ClinicalTrials.gov编号：NCT00987389。）。95％CI（0.52至0.93），但两组的其他次要结局相似。结论在严重的ANCA相关血管炎患者中，血浆置换的使用并不能减少死亡或ESKD的发生。就死亡或ESKD而言，糖皮质激素的减量方案不逊于标准剂量方案。（由英国国家卫生研究院等资助； PEXIVAS当前对照试验编号：ISRCTN07757494； ClinicalTrials.gov编号：NCT00987389。）。95％CI（0.52至0.93），但两组的其他次要结局相似。结论在严重的ANCA相关血管炎患者中，血浆置换的使用不能降低死亡或ESKD的发生率。就死亡或ESKD而言，糖皮质激素的减量方案不逊于标准剂量方案。（由英国国家卫生研究院等资助； PEXIVAS当前对照试验编号：ISRCTN07757494； ClinicalTrials.gov编号：NCT00987389。）。