目的: 探究血浆置换治疗新月体型IgA肾病(IgA nephropathy, IgAN)的有效性。方法: 选择2012年1月—2020年9月在北京大学第一医院肾内科等国内9家医院经肾活检确诊为原发性新月体型IgAN的患者病例资料进行回顾性分析，收集患者基线的临床、病理资料及治疗方案信息。为了尽可能减少基线特征中潜在混杂因素的影响，研究采用倾向性评分最近邻匹配法1 ∶1匹配血浆置换组和常规强化免疫抑制治疗组患者临床及病理信息。研究以终末期肾病(end-stage kidney disease, ESKD)为主要结局，采用Kaplan-Meier方法比较两组患者肾生存差异。结果: 共纳入95例新月体型IgA肾病伴急性肾病(acute kidney disease, AKD)患者，其中37例患者接受了血浆置换治疗，58例患者接受常规强化免疫抑制治疗。整体人群肾活检时估算肾小球滤过率(estimated glomerular filtration rate, eGFR)[M(P₂₅, P₇₅)]12.77 (7.28, 21.29) mL/(min·1.73 m²)，24-h尿蛋白定量5.9 (4.0, 8.9) g，新月体百分比为64.71%(54.55%, 73.68%)。倾向性评分匹配共23对患者，中位随访时间7 (1, 26) 个月，共29例(63.0%)患者进入终末期肾病, 其中血浆置换治疗组16例(69.6%)，常规强化免疫抑制治疗组13例(56.5%)。两组患者基线eGFR[14.30(9.31~17.58) mL/(min·1.73 m²) vs. 11.45(5.59~20.79) mL/(min·1.73 m²)]、24-h尿蛋白定量[(7.4±3.4) g vs. (6.6±3.8) g]、新月体百分比(64.49%±13.23% vs. 66.41%±12.65%)、肾活检后应用激素治疗比例[23(100.0%) vs. 21(91.3%)] 比较，差异均无统计学意义(P均>0.05)。Kaplan-Meier生存分析结果示生存率组间比较，差异无统计学意义(Log-rank检验，P=0.933)。结论: 在常规强化免疫抑制治疗的基础上加用血浆置换治疗未能进一步改善新月体型IgA肾病预后。

Objective: To evaluate the efficacy of plasma exchange therapy on crescentic IgA nephropathy (IgAN). Methods: A retrospective analysis was performed in a cohort of patients with crescentic IgAN from January 2012 to September 2020 at 9 sites across China. Clinical and pathological data, as well as therapeutic regimens, were collected. In order to minimize the effect of potential confounders in baseline characteristics, propensity score matching using a 1 ∶1 ratio nearest neighbor algorithm was performed between the adjunctive plasma exchange therapy group and the intensive immunosuppressive therapy group. The primary outcome was end-stage of kidney disease (ESKD). Kaplan-Meier method was used to compare the difference in renal survival between the two groups. Results: A total of 95 crescentic IgAN patients with acute kidney disease were included in this study, including 37 (38.9%) patients receiving adjunctive plasma exchange therapy, and 58 (61.1%) patients receiving intensive immunosuppressive therapy. In the whole cohort, the baseline eGFR was 12.77 (7.28, 21.29) mL/(min·1.73 m²), 24-hour urinary protein quantification was 5.9 (4.0, 8.9) g, and crescent percentage was 64.71% (54.55%, 73.68%). In the study, 23 patients in each group were matched after propensity score matching The median follow-up time was 7 (1, 26) months. As a whole, 29 patients (63.0%) reached ESKD, including 16 patients (69.6%) in the adjunctive plasma exchange therapy group and 13 (56.5%) patients in the intensive immunosuppressive therapy group.. There were no stastical difference between the two groups in terms of baseline eGFR [14.30 (9.31, 17.58) mL/(min·1.73 m²) vs. 11.45 (5.59, 20.79) mL/(min·1.73 m²)], 24-hour urinary protein (7.4±3.4) g vs. (6.6±3.8) g, crescent percentage 64.49%±13.23% vs. 66.41%±12.65% and the proportion of patients received steroid therapy[23 (100.0%) vs. 21 (91.3%)] (All P>0.05). Kaplan-Meier survival analysis demonstrated that there was no significant difference in renal survival rate between the two groups (Log-rank test, P=0.933). Conclusion: The adjunctive plasma exchange therapy in addition to conventional intense immunosuppressive therapy did not additionally improve the prognosis of crescentic IgA nephropathy.