Kaufeld Jessica, Lovric Svjetlana
Klinik für Nieren- und Hochdruckerkrankungen, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland.
Inn Med (Heidelb). 2024 Dec;65(12):1216-1224. doi: 10.1007/s00108-024-01812-1. Epub 2024 Nov 18.
Complement-mediated kidney diseases encompass a complex group of diseases that are primarily caused by dysregulation of the complement system. The complement system is a crucial component of the innate immune system consisting of soluble and membrane-bound proteins. The complement system is essential for the defence against pathogens and homeostasis but its uncontrolled activation can lead to an exaggerated cellular response to immunogenic, inflammatory and metabolic stimuli. For example, glomerular deposition of immune complexes can activate the complement system and contribute to the progression of kidney diseases. The most well-known complement-mediated kidney diseases are atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy, which falls under the group of membranoproliferative glomerulonephritis (MPGN). Diagnosing these diseases requires detailed investigations with respect to triggering factors, including genetic analyses and the measurement of specific complement factors. New therapeutic approaches with drugs targeting complement system activation are now offering promising treatment options. These medications fundamentally differ from other immunosuppressive drugs and specifically target the pathological processes within the complement system. This article provides an overview of the mechanisms of complement regulation, the pathophysiological foundations of complement-mediated kidney diseases and the latest diagnostic and therapeutic advances. The goal is to provide a deeper understanding of these complex conditions and to emphasize the importance of an interdisciplinary approach in treatment and research.
补体介导的肾脏疾病包括一组复杂的疾病,主要由补体系统失调引起。补体系统是固有免疫系统的重要组成部分,由可溶性和膜结合蛋白组成。补体系统对于抵御病原体和维持体内平衡至关重要,但其不受控制的激活会导致细胞对免疫原性、炎症和代谢刺激产生过度反应。例如,免疫复合物在肾小球的沉积可激活补体系统并促进肾脏疾病的进展。最著名的补体介导的肾脏疾病是非典型溶血性尿毒症综合征(aHUS)和C3肾小球病,后者属于膜增生性肾小球肾炎(MPGN)。诊断这些疾病需要对触发因素进行详细调查,包括基因分析和特定补体因子的测量。针对补体系统激活的药物的新治疗方法现在提供了有前景的治疗选择。这些药物与其他免疫抑制药物有根本区别,专门针对补体系统内的病理过程。本文概述了补体调节机制、补体介导的肾脏疾病的病理生理基础以及最新的诊断和治疗进展。目的是更深入地了解这些复杂病症,并强调跨学科方法在治疗和研究中的重要性。