C3 glomerulopathy: a new classification
C3 glomerulopathy: a new classification
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DOI：
10.1038/nrneph.2010.85
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发表时间：
2010-08-01
影响因子：
41.5
通讯作者：
Pickering, Matthew C.
中科院分区：
文献类型：
--
作者：
Fakhouri, Fadi;Fremeaux-Bacchi, Veronique;Pickering, Matthew C.
肾小球炎症的几种不同的病理模式与补体系统的异常调节有关，特别是与补体系统的旁路途径的失调有关。然而，这些病症的病理学发现是在缺乏大量免疫球蛋白的情况下，补体 C3 (C3) 沉积在肾小球内。这一发现提醒我们和其他人注意个体患者可能存在遗传性和获得性补体失调。本文总结了我们目前对补体系统失调与肾小球炎症之间关系的理解。在这里，我们建议以 C3 孤立沉积为特征的肾小球病理可有效地通过术语 C3 肾小球病进行分类。我们认为，这种分类将提醒病理学家和肾脏病学家注意筛查补体调节中获得性和遗传性异常的重要性。将来，它可能有助于识别可能受益于补体系统治疗抑制的个体。
Several distinct pathological patterns of glomerular inflammation are associated with abnormal regulation of the complement system, specifically, with dysregulation of the alternative pathway of the complement system. However, these conditions share the pathological finding of complement C3 (C3) deposited within the glomerulus in the absence of substantial immunoglobulin. This finding has alerted us and others to the possible presence of genetic and acquired complement dysregulation in individual patients. This article summarizes our current understanding of the relationship between dysregulation of the complement system and glomerular inflammation. Here, we suggest that glomerular pathologies that are characterized by the isolated deposition of C3 could usefully be classified by the term C3 glomerulopathy. In our view, this classification would alert the pathologist and nephrologist to the importance of screening for acquired and genetic abnormalities in complement regulation. In the future, it could help to identify individuals who might benefit from therapeutic inhibition of the complement system.