系统评价血浆置换联合激素或/和免疫抑制剂治疗对抗中性粒细胞胞浆抗体(ANCA)相关性血管炎性肾损害的临床疗效及预后。

计算机检索MEDLINE via OvidSP、Embase、中国生物医学文献数据库、知网、万方及维普数据库，检索时限为建库至2019年4月，搜集血浆置换联合激素或/和免疫抑制剂治疗ANCA相关性血管炎肾损害的随机对照试验(RCT)及队列研究的相关文献。总共纳入939例患者，其中标准治疗组(单独的激素或/和免疫抑制剂治疗)共503例患者，联合治疗组(标准治疗联合血浆置换治疗)共436例患者，应用Review Manager 5.3软件行荟萃分析。

(1)肾功能改善方面：联合治疗组血肌酐降低更明显，其差异有统计学意义(SMD＝－0.51，95% CI：－0.92～－0.09，P＝0.02)，亚组分析结果显示联合治疗组短期疗效及长期疗效和标准治疗组相比好转，且差异有统计学意义；联合治疗组血尿素氮降低不明显，其差异无统计学意义(SMD＝－0.25；95% CI：－0.37～ 0.87，P＝0.43)。(2)原发病控制方面：联合治疗组伯明翰血管炎活动性评分(BVAS)下降更明显，其差异有统计学意义(MD＝－1.51，95% CI：－2.38～ －0.64，P<0.05)；联合治疗组ANCA水平降低更明显，其差异有统计学意义(MD＝－27.68，95% CI：－42.17～－13.19，P<0.05)。(3)长期随访结局方面：联合治疗组发生死亡或终末期肾病的风险小于标准治疗组，其差异有统计学意义(RR＝0.67，95% CI：0.53～ 0.84，P＝0.0004)；联合治疗组脱离透析频率高于标准治疗组，其差异有统计学意义(RR＝1.32，95% CI：1.08～ 1.61，P＝0.007)。联合治疗组不良反应发生率和标准治疗组相当，不良反应主要包括肺部感染、肺结核、带状疱疹、胃肠道症状及肝损害，其差异无统计学意义(RR＝0.64，95%CI：0.39～ 1.05，P＝0.08)。

血浆置换联合激素或/和免疫抑制剂治疗可有效降低血清肌酐值、血清ANCA水平、伯明翰血管炎活动评分；减少终末期肾病的复合终点和全因死亡，提高疾病控制率，但随访过程中两组不良反应发生率相似。

To systematically evaluate the clinical efficacy and prognosis of plasma exchange combined with hormones and/or immunosuppressive agents in treating severe renal impairment of ANCA-associated vasculitis.

Computer search was made in MEDLINE via OvidSP, Embase, China Biomedical Literature Database, China National Knowledge Internet, Wanfang Data and CQVIP Database. The search period was from the establishment of the database to April 2019. Related literatures were collected from randomized controlled trials (RCT) and cohort studies on treatment of severe renal impairment in ANCA-associated vasculitis with plasma exchange combined with hormones and/or immunosuppressive agents. A total of 939 patients were included, including 503 patients in the standard treatment group (corticosteroids alone or combining immunosuppressive agents) and 436 patients in the combination treatment group (standard treatment combined with plasma exchange). Meta-analysis was performed with the software of Review Manager 5.3 version.

Regarding the improvement of renal function: the blood creatinine decreased more in the combination treatment group (SMD=-0.51, 95%CI: -0.92 to -0.09, P=0.02). Subgroup analysis results showed that the short-term and long-term efficacy of the combination treatment group improved with statistically significant difference compared with the standard treatment group. There was no significant decrease in urea nitrogen in the combination treatment group (SMD=-0.25, 95%CI: -0.37 to 0.87, P=0.43). In terms of primary disease control: Birmingham vasculitis activity score (BVAS) of the combination treatment group decreased more significantly (MD=-1.51, 95%CI: -2.38 to -0.64, P<0.05); The decrease of ANCA level in combination treatment group was more obvious (MD=-27.68, 95%CI: -42.17 to -13.19, P<0.05). In terms of long-term follow-up outcomes: the risk of death or end-stage renal disease in the combination treatment group was less than that in the standard treatment group (RR= 0.67, 95%CI: 0.53-0.84, P<0.05); The frequency of freedom from dialysis in the combination treatment group was higher than that in the standard treatment group (RR=1.32, 95%CI: 1.08-1.61, P<0.05). The incidence of adverse reactions in the combination treatment group was similar to that in the standard treatment group, and the adverse reactions mainly included pulmonary infection, tuberculosis, herpes zoster, gastrointestinal symptoms, and liver damage (OR=0.64, 95%CI: 0.39-1.05, P=0.08).

Plasma exchange combined with hormones and/or immunosuppressive agents could effectively reduce serum creatinine level, serum ANCA level, BVAS, and the composite end point and all-cause mortality of end-stage renal disease, so as to improve disease control rate, while the incidence of adverse reactions was similar between the two groups during the follow-up.