<p>The complement system is a&#xa0;key component of the innate immune system and plays a&#xa0;decisive role in defence against pathogens, the elimination of cellular debris and the activation of immune mediators; however, dysregulation can lead to an overactivation of this system, which can subsequently have the sequelae of severe organ dysfunction, often starting with damage of endothelial cells. The therapeutic use of complement inhibitors in nephrology was established as a form of complement-mediated thrombotic microangiopathy (TMA), particularly by the treatment of atypical hemolytic uremic syndrome (aHUS). As the understanding of the pathophysiological importance of the complement system in various kidney diseases has advanced, targeted therapies have emerged. Notably, in 2025 the oral factor&#xa0;B inhibitor iptacopan was approved as the first specific treatment for C3 glomerulopathy. Additional indications, such as IgA nephropathy and lupus nephritis, are currently being evaluated in clinical trials. This review outlines recent progress in the development and clinical application of novel complement inhibitors. It presents the mechanisms of action and summarizes the outcomes from key studies.</p>

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Neue Komplementinhibitoren

  • Jessica Kaufeld

摘要

The complement system is a key component of the innate immune system and plays a decisive role in defence against pathogens, the elimination of cellular debris and the activation of immune mediators; however, dysregulation can lead to an overactivation of this system, which can subsequently have the sequelae of severe organ dysfunction, often starting with damage of endothelial cells. The therapeutic use of complement inhibitors in nephrology was established as a form of complement-mediated thrombotic microangiopathy (TMA), particularly by the treatment of atypical hemolytic uremic syndrome (aHUS). As the understanding of the pathophysiological importance of the complement system in various kidney diseases has advanced, targeted therapies have emerged. Notably, in 2025 the oral factor B inhibitor iptacopan was approved as the first specific treatment for C3 glomerulopathy. Additional indications, such as IgA nephropathy and lupus nephritis, are currently being evaluated in clinical trials. This review outlines recent progress in the development and clinical application of novel complement inhibitors. It presents the mechanisms of action and summarizes the outcomes from key studies.