Use of Eculizumab in Atypical Hemolytic Uremic Syndrome, Complicating Systemic Lupus Erythematosus
Use of Eculizumab in Atypical Hemolytic Uremic Syndrome, Complicating Systemic Lupus Erythematosus
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DOI：
10.1097/rhu.0000000000000423
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发表时间：
2016-09-01
影响因子：
3.4
通讯作者：
Liarski, Vladimir M.
中科院分区：
文献类型：
--
作者：
Bermea, Rene S.;Sharma, Niharika;Liarski, Vladimir M.
非典型溶血性尿毒综合征的特征是血小板减少、微血管病性溶血性贫血和终末器官损伤。在这份报告中，我们描述了两名系统性红斑狼疮患者，他们的表现与非典型溶血性尿毒综合征一致，并发急性肾损伤，常规治疗无效。这两名患者均表现出对eculizumab（一种针对补体蛋白C5的单克隆抗体）的应答，血小板计数稳定。在1年的随访中，他们的血液病保持在缓解状态，没有复发。
Atypical hemolytic uremic syndrome is characterized by the presence of thrombocytopenia, microangiopathic hemolytic anemia, and end-organ injury. In this report, we describe two patients with systemic lupus erythematosus who presented with findings compatible with atypical hemolytic uremic syndrome, complicated by acute kidney injury that was refractory to conventional therapies. Both patients exhibited a response to eculizumab, a monoclonal antibody to complement protein C5, with stabilization of their platelet count. On 1-year follow-up from their initial presentation, their hematologic disease remained in remission without recurrence.