Complement C5-inhibiting therapy for the thrombotic microangiopathies: accumulating evidence, but not a panacea.
Complement C5-inhibiting therapy for the thrombotic microangiopathies: accumulating evidence, but not a panacea.
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DOI：
10.1093/ckj/sfx081
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发表时间：
2017-10
影响因子：
4.6
通讯作者：
Kavanagh D
中科院分区：
文献类型：
--
作者：
Brocklebank V;Kavanagh D
血栓性微血管病（TMA）以严重内皮损伤引起的器官损伤为特征，可表现在多种疾病中。在补体介导的非典型溶血性尿毒综合征（aHUS）中，补体的原发性缺陷，如突变或自身抗体导致替代途径的过度激活，通常在暴露于环境触发因素后易导致疾病的发展。aHUS发病机制的阐明导致补体抑制剂eculizumab成功引入临床实践。在其他tma中，虽然可以看到补体活化，但其在发病机制中的作用仍有待于一项介入性试验的证实。尽管除了补体介导的aHUS外，许多关于tma的病例报告暗示了疗效，但发表偏倚、并发治疗以及在某些情况下疾病的自限性使得更广泛的解释变得困难。在本文中，我们将回顾补体抑制在补体介导的aHUS和其他tma中作用的证据。
Thrombotic microangiopathy (TMA), characterized by organ injury occurring consequent to severe endothelial damage, can manifest in a diverse range of diseases. In complement-mediated atypical haemolytic uraemic syndrome (aHUS) a primary defect in complement, such as a mutation or autoantibody leading to over activation of the alternative pathway, predisposes to the development of disease, usually following exposure to an environmental trigger. The elucidation of the pathogenesis of aHUS resulted in the successful introduction of the complement inhibitor eculizumab into clinical practice. In other TMAs, although complement activation may be seen, its role in the pathogenesis remains to be confirmed by an interventional trial. Although many case reports in TMAs other than complement-mediated aHUS hint at efficacy, publication bias, concurrent therapies and in some cases the self-limiting nature of disease make broader interpretation difficult. In this article, we will review the evidence for the role of complement inhibition in complement-mediated aHUS and other TMAs.
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DOI：
10.1111/j.1600-0897.2009.00731.x
发表时间：
2009-10-01
影响因子：
3.6
作者：
Ari, Elif;Yilmaz, Yusuf;Ozener, Cetin
通讯作者：
Ozener, Cetin
影响因子：
6.1
作者：
Ahlenstiel-Grunow, Thurid;Hachmeister, Svenja;Pape, Lars
通讯作者：
Pape, Lars
影响因子：
11.8
作者：
Becker, S;Fusco, G;Feinberg, J
通讯作者：
Feinberg, J
影响因子：
3.4
作者：
Bermea, Rene S.;Sharma, Niharika;Liarski, Vladimir M.
通讯作者：
Liarski, Vladimir M.
影响因子：
4.4
作者：
Blanc, Caroline;Togarsimalemath, Shambhuprasad Kotresh;Dragon-Durey, Marie-Agnes
通讯作者：
Dragon-Durey, Marie-Agnes