To evaluate efficacy of rituximab in AAE-C1-INH.
A retrospective multicenter study was carried out in France, including patients with AAE-C1-INH treated with rituximab between April 2005 and July 2019.
Fifty-five patients with AAE-C1-INH were included in the study, and 23 of them had an anti-C1-INH antibody. A lymphoid malignancy was identified in 39 patients, and a monoclonal gammopathy in 9. There was no associated condition in 7 cases. Thirty patients received rituximab alone or in association with chemotherapy (n = 25). Among 51 patients with available follow-up, 34 patients were in clinical remission and 17 patients had active AE after a median follow-up of 3.9 years (interquartile range, 1.5-7.7). Three patients died. The presence of anti-C1-INH antibodies was associated with a lower probability of AE remission (hazard ratio, 0.29 [95% CI, 0.12-0.67]; P = .004). Relapse was less frequent in patients with lymphoma (risk ratio, 0.27 [95% CI, 0.09-0.80]; P = .019) and in patients treated with rituximab and chemotherapy (risk ratio, 0.31 [95% CI, 0.12-0.79]; P = .014).
Rituximab is an efficient and well-tolerated therapeutic option in AE, especially in lymphoid malignancies and in the absence of detectable anti-C1-INH antibodies.
目的:评价利妥昔单抗治疗获得性C1抑制剂缺乏症血管性水肿的疗效。
方法:在法国进行了一项回顾性多中心研究,包括2005年4月至2019年7月期间接受利妥昔单抗治疗的AAE-C1-INH患者.
结果:本研究纳入了55例AAE-C1-INH患者,其中23人具有抗C1-INHAb。在39例患者中发现了淋巴恶性肿瘤;9例单克隆丙种球蛋白病。7例无相关情况。30例患者单独或联合化疗接受了利妥昔单抗(N=25)。在有随访的51例患者中,中位随访3.9年(IQR1.5-7.7)后,34例患者临床缓解,17例患者出现活动性AE。三名患者死亡。抗C1-INHAb的存在与血管性水肿缓解的较低概率相关(HR0.29(0.12-0.67),p=0.004)。淋巴瘤患者(风险比0.27(0.09-0.80)p=0.019)和利妥昔单抗和化疗患者(风险比0.31(0.12-0.79),复发频率较低,p=0.014)。
结论:利妥昔单抗是一种有效且耐受性良好的AE治疗选择,特别是在淋巴恶性肿瘤和缺乏可检测的抗C1-INHAb。