Objectives There are limited real-life data regarding the efficacy and safety of rituximab (RTX) as a remission maintenance agent in microscopic polyangiitis (MPA) and granulomatosis-with-polyangiitis (GPA). We aimed to estimate the incidence and risk factors for relapses, as well for serious adverse events (SAEs) in MPA/GPA patients during RTX maintenance. Methods A retrospective cohort of newly diagnosed/relapsing GPA/MPA patients who received RTX maintenance (≥1 RTX cycle, ≥6 months follow-up) following complete remission (BVAS version-3 = 0 plus prednisolone ≤7.5 mg/day) with induction regimens. SAEs included serious infections, COronaVIrus-Disease 2019 (COVID-19)–associated hospitalizations, deaths, cardiovascular events, malignancies and hypogammaglobulinemia. The incidence rates (IRs) and relapse-free survival were estimated through Kaplan–Meier plots. Cox regression was conducted to investigate factors associated with the time-to-relapse. Results A total of 101 patients were included: 48% females, 69% GPA, 53% newly diagnosed, median age 63 years. During follow-up (294.5 patient-years, median: 3 RTX cycles), 30 relapses (57% major) occurred among 24 patients (24%, IR 10.2/100 patient-years). Kidney involvement (adjusted hazard ratio/aHR: 0.20; 95% CI: 0.06–0.74, P = 0.016), prior induction with RTX plus CYC (vs RTX monotherapy: aHR = 0.02; 95% CI: 0.001–0.43, P = 0.012) and shorter time interval until complete remission (aHR = 1.07; 95% CI: 1.01–1.14, P = 0.023) were associated with decreased relapse risk. We recorded 17 serious infections (IR 5.8/100 patient-years), 11 COVID-19–associated hospitalizations (IR 3.7/100 patient-years), 4 malignancies (IR 1.4/100 patient-years), 6 cardiovascular events (IR 2/100 patient-years) and 10 deaths (IR 3.4/100 patient-years). Conclusion In this real-world study, relapses during RTX maintenance occurred in approximately 1 out of 4 patients. Kidney involvement, induction with RTX plus CYC, and earlier achievement of complete remission were associated with lower relapse risk. The serious infections rate was consistent with previous reports, whereas an increased rate of COVID-19–associated hospitalizations was observed.

中文翻译：

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ANCA 相关血管炎利妥昔单抗维持治疗期间的复发和严重不良事件：一项多中心回顾性研究


目的 关于利妥昔单抗 （RTX） 作为缓解维持剂治疗显微镜下多血管炎 （MPA） 和肉芽肿伴多血管炎 （GPA） 的疗效和安全性的真实数据有限。我们旨在估计 RTX 维持期间 MPA/GPA 患者复发的发生率和风险因素，以及严重不良事件 （SAE）。方法 回顾性队列新诊断/复发的 GPA/MPA 患者，这些患者在完全缓解后接受 RTX 维持治疗 （≥1 个 RTX 周期，≥ 6 个月随访） （BVAS 版本 3 = 0 加泼尼松龙≤7.5 mg/天） 采用诱导方案。SAE 包括严重感染、2019 年 COronaVIrus 病 （COVID-19） 相关住院、死亡、心血管事件、恶性肿瘤和低丙种球蛋白血症。通过 Kaplan-Meier 图估计发病率 （IRs） 和无复发生存期。进行 Cox 回归以研究与复发时间相关的因素。结果 共纳入 101 例患者： 女性 48%，GPA 69%，新诊断 53%，中位年龄 63 岁。在随访期间 （294.5 患者年，中位数：3 个 RTX 周期），24 例患者 （24%，IR 10.2/100 患者年） 发生了 30 次复发 （57% 严重）。肾脏受累（调整后的风险比/aHR：0.20;95% CI：0.06-0.74，P = 0.016）、先前使用 RTX 加 CYC 诱导（与 RTX 单药治疗相比：aHR = 0.02;95% CI：0.001-0.43，P = 0.012）和较短的完全缓解时间间隔（aHR = 1.07;95% CI：1.01-1.14，P = 0.023）与复发风险降低相关。我们记录了 17 例严重感染 （IR 5.8/100 患者年）、11 例 COVID-19 相关住院 （IR 3.7/100 患者年）、4 例恶性肿瘤 （IR 1.4/100 患者年）、6 例心血管事件 （IR 2/100 患者年） 和 10 例死亡 （IR 3.4/100 患者年）。结论在这项真实世界研究中，大约每 4 名患者中就有 1 名在 RTX 维持期间复发。肾脏受累、RTX 加 CYC 诱导和早期达到完全缓解与较低的复发风险相关。严重感染率与之前的报告一致，而观察到与 COVID-19 相关的住院率增加。