Eculizumab discontinuation in a patient with atypical hemolytic uremic syndrome after ChAdOx1 nCoV-19 vaccination.

Marisa Roldão, Francisco Ferrer, Karina Lopes
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Abstract

Eculizumab has proven to be effective in patients with atypical hemolytic uremic syndrome (aHUS) in clinical trials and in the real world, but the optimal duration of therapy remains unknown. Standard maintenance treatment is often life-long, but the possibility of discontinuation has not yet been systematically tested. We describe a case of aHUS after ChAdOx1 nCoV-19 vaccination in a patient with homozygous CFHR3/CFHR1 gene deletion who discontinued eculizumab maintenance therapy 24 weeks after achieving disease remission. We report the safety of discontinuing eculizumab treatment with the aim of minimizing the risk of adverse reactions, reducing the risk of meningitis, improving quality of life, and reducing the considerable treatment costs.

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1例非典型溶血性尿毒症患者在接种ChAdOx1 nCoV-19疫苗后停用依珠单抗
在临床试验和现实世界中,Eculizumab已被证明对非典型溶血性尿毒症综合征(aHUS)患者有效,但最佳治疗持续时间尚不清楚。标准的维持治疗通常是终身的,但停止治疗的可能性尚未得到系统的测试。我们描述了一例ChAdOx1 nCoV-19疫苗接种后aHUS的病例,该患者患有纯合子CFHR3/CFHR1基因缺失,在疾病缓解24周后停止了eculizumab维持治疗。我们报告停止eculizumab治疗的安全性,目的是尽量减少不良反应的风险,降低脑膜炎的风险,提高生活质量,并减少可观的治疗费用。
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