期刊
PEDIATRIC NEPHROLOGY
卷 -, 期 -, 页码 -出版社
SPRINGER
DOI: 10.1007/s00467-023-06120-8
关键词
Eculizumab; Avacopan; aHUS; C3 glomerulopathy; Complement; Kidney diseases; Children
The complement system plays a crucial role in kidney diseases, and complement inhibitors have shown potential in improving their outcomes. This article provides a summary of the main complement inhibitors used in nephrology and discusses their efficacy in different complement-mediated kidney diseases. These novel drugs have the potential to significantly improve the prognosis of kidney diseases.
Historically, the complement system (classical, lectin, alternative, and terminal pathways) is known to play a crucial role in the etiopathogenesis of many kidney diseases. Direct or indirect activation in these settings is revealed by consumption of complement proteins at the serum level and kidney tissue deposition seen by immunofluorescence and electron microscopy. The advent of eculizumab has shown that complement inhibitors may improve the natural history of certain kidney diseases. Since then, the number of available therapeutic molecules and experimental studies on complement inhibition has increased exponentially. In our narrative review, we give a summary of the main complement inhibitors that have completed phase II and phase III studies or are currently used in adult and pediatric nephrology. The relevant full-text works, abstracts, and ongoing trials (clinicaltrials.gov site) are discussed. Data and key clinical features are reported for eculizumab, ravulizumab, crovalimab, avacopan, danicopan, iptacopan, pegcetacoplan, and narsoplimab. Many of these molecules have been shown to be effective in reducing proteinuria and stabilizing kidney function in different complement-mediated kidney diseases. Thanks to their efficacy and target specificity, these novel drugs may radically improve the outcome of complement-mediated kidney diseases, contributing to an improvement in our understanding of their underlying pathophysiology.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据