Case report: Short-term eculizumab use in atypical HUS associated with Lemierre's syndrome and post-infectious glomerulonephritis.
Case report: Short-term eculizumab use in atypical HUS associated with Lemierre's syndrome and post-infectious glomerulonephritis.
复制标题
病例报告：短期eculizumab在非典型HUS中使用与Lemierre综合征和感染后肾小球肾炎相关的非典型HUS。
DOI：
10.3389/fmed.2023.1167806
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发表时间：
2023
影响因子：
3.9
通讯作者：
中科院分区：
文献类型：
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作者：
非典型溶血性尿毒综合征（阿胡斯）是由遗传异常、感染、自身免疫性疾病、药物和恶性肿瘤引起的罕见疾病。抗C5单克隆抗体依库珠单抗是治疗由补体旁路途径的遗传缺陷引起的阿胡斯的主要药物。然而，依库珠单抗在非遗传形式的阿胡斯中的效用和治疗停止的时间仍然存在争议。在这里，我们报告了成功的短期使用依库珠单抗与阿胡斯由于罕见的感染性和自身免疫性病因：Lemierre综合征和感染后肾小球肾炎，分别在两个年轻的成年患者。在长期随访期间，2例患者均迅速停用依库珠单抗，无阿胡斯复发。考虑到其有利的安全性特征与适当的脑膜炎球菌预防，依库珠单抗可以被认为是非遗传性阿胡斯的治疗选择。
Atypical hemolytic uremic syndrome (aHUS) is a rare disease caused by genetic abnormalities, infections, autoimmune diseases, drugs, and malignancies. Anti-C5 monoclonal antibody eculizumab is the mainstay of treatment of aHUS caused by the genetic defects of the alternative complement pathway. However, the utility of eculizumab in non-genetic forms of aHUS and the timing of treatment discontinuation remain controversial. Here, we report successful short-term eculizumab use in two young adult patients with aHUS due to rare infectious and autoimmune etiologies: Lemierre's syndrome and post-infectious glomerulonephritis, respectively. Eculizumab was rapidly discontinued in both patients with no aHUS recurrence during long-term follow-up. Considering its favorable safety profile with appropriate meningococcal prophylaxis, eculizumab can be considered as a treatment option for non-genetic aHUS.