背景:白细胞介素-5(IL-5)抑制剂代表了嗜酸性肉芽肿性多血管炎(EGPA)的新疗法。这项研究评估了IL-5受体抑制剂benralizumab在欧洲EGPA患者队列中的有效性和安全性。
方法:这项回顾性队列研究包括来自欧洲EGPA研究组的28个欧洲转诊中心的EGPA患者,这些患者来自六个国家(意大利,法国,英国,俄罗斯,西班牙,和瑞士),他们在2019年1月1日至2022年9月30日之间接受了贝那利珠单抗作为任何治疗方案。我们评估了完全反应率,定义为无疾病活动(伯明翰血管炎活动评分[BVAS]为0)和泼尼松剂量高达4mg/天,与部分反应相反,定义为BVAS为0,泼尼松剂量大于4mg/天。活动性疾病表现,肺功能,糖皮质激素剂量的变化,在12个月的随访中还评估了安全性结局.
结果:纳入121例复发难治性EGPA患者(64[53%]女性和57[47%]男性;开始benralizumab治疗时的中位年龄54·1岁[IQR44·2-62·2])。15例报告完全缓解(12.4%,在第3,25个月时,121例患者的95%CI7·1-19·6(28·7%,19·5-39·4)在第6个月的87例患者中,32例(46·4%,34·3-58·8)在12个月时有69名患者;在另外43名患者中观察到部分反应(35·5%,27·0-44·8)患者在3个月,23(26·4%,17·6-37·0),第6个月和第13个月(18·8%,10·4-30·1)在12个月。BVAS从基线时的3·0(IQR2·0-8·0)降至第3个月和第6个月时的0·0(0·0-2·0),并在第12个月时降至0·0(0·0-1·0)。有全身表现的患者比例,活动性周围神经疾病,耳朵,鼻子,和喉咙受累,肺部受累减少,随着肺功能测试的改善。六名患者在完全反应后复发。口服泼尼松(或等效剂量)从基线时的10·0mg/天(5·0-12·5)减少到第3个月时的5·0mg/天(3·6-8·5)(p<0·01),在第6个月达到5·0毫克/天(2·5-6·3),在第12个月达到2·5毫克/天(0·0-5·0)(p<0·0001)。121例患者中有19例(16%)出现不良事件,16例(13%)停用贝那利珠单抗。
结论:这些数据表明,贝那利珠单抗在实际临床实践中可能是EGPA的有效治疗方法。需要进一步的临床试验来确认贝那利珠单抗在具有较高基线疾病活动性的患者中的疗效。
背景:无。
Interleukin-5 (IL-5) inhibitors represent novel therapies for eosinophilic granulomatosis with polyangiitis (EGPA). This study assessed the effectiveness and safety of the IL-5 receptor inhibitor benralizumab in a European cohort of patients with EGPA.
This retrospective cohort study included patients with EGPA from 28 European referral centres of the European EGPA Study Group across six countries (Italy, France, UK, Russia, Spain, and Switzerland) who received benralizumab as any line of treatment between Jan 1, 2019, and Sep 30, 2022. We assessed the rates of complete response, defined as no disease activity (Birmingham Vasculitis Activity Score [BVAS] of 0) and a prednisone dose of up to 4 mg/day, in contrast to partial response, defined as a BVAS of 0 and a prednisone dose greater than 4 mg/day. Active disease manifestations, pulmonary function, variation in glucocorticoid dose, and safety outcomes were also assessed over a 12-month follow-up.
121 patients with relapsing-refractory EGPA treated with benralizumab at the dose approved for eosinophilic asthma were included (64 [53%] women and 57 [47%] men; median age at the time of beginning benralizumab treatment 54·1 years [IQR 44·2-62·2]). Complete response was reported in 15 (12·4%, 95% CI 7·1-19·6) of 121 patients at month 3, 25 (28·7%, 19·5-39·4) of 87 patients at month 6, and 32 (46·4%, 34·3-58·8) of 69 patients at month 12; partial response was observed in an additional 43 (35·5%, 27·0-44·8) patients at month 3, 23 (26·4%, 17·6-37·0) at month 6, and 13 (18·8%, 10·4-30·1) at month 12. BVAS dropped from 3·0 (IQR 2·0-8·0) at baseline to 0·0 (0·0-2·0) at months 3 and 6, and to 0·0 (0·0-1·0) at month 12. The proportion of patients with systemic manifestations, active peripheral neurological disease, ear, nose, and throat involvement, and pulmonary involvement decreased, with an improvement in lung function tests. Six patients relapsed after having a complete response. The oral prednisone (or equivalent) dose decreased from 10·0 mg/day (5·0-12·5) at baseline to 5·0 mg/day (3·6-8·5) at month 3 (p<0·01), to 5·0 mg/day (2·5-6·3) at month 6, and to 2·5 mg/day (0·0-5·0) at month 12 (p<0·0001). 19 (16%) of 121 patients had adverse events and 16 (13%) discontinued benralizumab.
These data suggest that benralizumab could be an effective treatment for EGPA in real-life clinical practice. Further clinical trials are required to confirm the efficacy of benralizumab in patients with a higher baseline disease activity.
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