Complement Factor H-Antibody-Associated Hemolytic Uremic Syndrome: Pathogenesis, Clinical Presentation, and Treatment
Complement Factor H-Antibody-Associated Hemolytic Uremic Syndrome: Pathogenesis, Clinical Presentation, and Treatment
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DOI：
10.1055/s-0034-1375297
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发表时间：
2014-06-01
影响因子：
5.7
通讯作者：
Jozsi, Mihaly
中科院分区：
文献类型：
--
作者：
Hofer, Johannes;Giner, Thomas;Jozsi, Mihaly
血浆中主要针对补体因子H抗体（CFH-Ab）的循环自身抗体的存在表征非典型溶血性尿毒症综合征（阿胡斯）的自身免疫形式。阿胡斯的这种获得性形式定义了阿胡斯患者的独特亚组，其需要部分不同于遗传定义形式的诊断和治疗方法。导致CFH-Ab产生和疾病发作的机制尚不完全清楚，但CFH-Ab HUS似乎是继发于遗传易感性和环境因素的组合。这种特异性阿胡斯实体的早期诊断是重要的，因为及时诱导血浆置换和伴随的免疫抑制导致有利的结果。然而，关于儿童临床特征和结局的信息有限。在这里，我们回顾了CFH-Ab HUS的生物学和临床特征的文献，并讨论了治疗方案。
The presence of circulating autoantibodies, primarily to complement factor H antibodies (CFH-Abs) in plasma characterizes the autoimmune form of atypical hemolytic uremic syndrome (aHUS). This acquired form of aHUS defines a distinct subgroup of aHUS patients, which requires diagnostic and treatment approaches in part different from those of the genetically defined forms. The mechanisms leading to CFH-Ab production and disease onset are not completely understood, but CFH-Ab HUS seems to be secondary to a combination of genetic predisposition and environmental factors. Early diagnosis of this specific aHUS entity is important, as prompt induction of plasma exchange and concomitant immunosuppression leads to a favorable outcome. Nevertheless, information on clinical features and outcome in children is limited. Here, we review the literature on the biological and clinical features of CFH-Ab HUS and discuss therapeutic options.