Kalmi Galith, Nguyen Yann, Amarger Stephanie, Aubineau Magali, Bibes Beatrice, Blanchard-Delaunay Claire, Boccon-Gibod Isabelle, Bouillet Laurence, Coppo Paul, Dalmas Marie-Caroline, Debord-Peguet Sophie, Defendi Federica, Demoreuil Claire, Du-Thanh Aurélie, Gayet Stephane, Hadjadj Jerôme, Jeandel Pierre-Yves, Launay David, Ly Kim Heang, Avoy Chloé Mc, Niault Mathilde, Ollivier Yann, Pelletier Fabien, Porneuf Marc, Roos-Weil Damien, Fain Olivier, Gobert Delphine
Internal Medicine Department, Sorbonne Université, AP-HP, Hôpital Saint Antoine, Paris, France.
Internal Medicine Department, Nord-Université Paris Cité, AP-HP, Hôpital Beaujon, Clichy-sous-Bois, France.
J Allergy Clin Immunol Pract. 2024 Jan;12(1):212-222. doi: 10.1016/j.jaip.2023.10.017. Epub 2023 Oct 14.
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.
获得性C1抑制物（C1-INH）缺乏所致血管性水肿（AE）（AAE-C1-INH）与C1-INH过度消耗或抗C1-INH抗体有关，且常与淋巴增殖性综合征或单克隆丙种球蛋白病相关。这种情况下预防性治疗的护理标准尚未确立。利妥昔单抗可能对预防发作有效，尤其是在淋巴造血系统疾病得到控制时，但相关数据较少。
评估利妥昔单抗治疗AAE-C1-INH的疗效。
在法国进行了一项回顾性多中心研究，纳入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。
55例AAE-C1-INH患者纳入研究，其中23例有抗C1-INH抗体。39例患者确诊为淋巴系统恶性肿瘤，9例为单克隆丙种球蛋白病。7例无相关疾病。30例患者单独接受利妥昔单抗治疗或联合化疗（n = 25）。在51例有随访资料的患者中，中位随访3.9年（四分位间距，1.5 - 7.7年）后，34例患者临床缓解，17例患者有活动性AE。3例患者死亡。抗C1-INH抗体的存在与AE缓解概率较低相关（风险比，0.29 [95% CI，0.12 - 0.67]；P = .004）。淋巴瘤患者（风险比，0.27 [95% CI，0.09 - 0.80]；P = .019）以及接受利妥昔单抗联合化疗的患者（风险比，0.31 [95% CI，0.12 - 0.79]；P = .014）复发频率较低。
利妥昔单抗是AE的一种有效且耐受性良好的治疗选择，尤其是在淋巴系统恶性肿瘤且未检测到抗C1-INH抗体的情况下。
