Abstract：
Angioedema (AE) due to acquired C1-inhibitor (C1-INH) deficiency (AAE-C1-INH) is related to excessive consumption of C1-INH or to anti-C1-INH antibodies, and is frequently associated with lymphoproliferative syndromes or monoclonal gammopathies. Standard of care for prophylactic treatment in this condition is not established. Rituximab may be effective to prevent attacks, especially if the lymphoid hemopathy is controlled, but data are scarce.
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.
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.
摘要：
背景：获得性C1抑制剂（C1-INH）缺乏症（AAE-C1-INH）引起的血管性水肿（AE）与过量消耗C1-INH或抗C1-INH抗体（Ab）有关，并经常与淋巴增生综合征或单克隆免疫球蛋白病有关。在这种情况下预防性治疗的护理标准尚未建立。利妥昔单抗可能有效预防发作，特别是如果淋巴血液病得到控制，但是数据很少。
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。
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。
