Division of Rheumatology, NYU Grossman School of Medicine, New York, New York.
Division of Rheumatology, NYU Grossman School of Medicine, New York, New York.
Transl Res. 2022 Jul;245:1-17. doi: 10.1016/j.trsl.2022.02.001. Epub 2022 Feb 11.
The complement system plays crucial roles in homeostasis and host defense against microbes. Deficiency of early complement cascade components has been associated with increased susceptibility to systemic lupus erythematosus (SLE), whereas excessive complement consumption is a hallmark of this disease. Although enhanced classical pathway activation by immune complexes was initially thought to be the main contributor to lupus nephritis (LN) pathogenesis, an increasing body of evidence has suggested the alternative and the lectin pathways are also involved. Therapeutic agents targeting complement activation have been used in LN patients and clinical trials are ongoing. We review the mechanisms by which complement system dysregulation contributes to renal injury in SLE and summarize the latest evidence on the use of anticomplement agents to manage this condition.
补体系统在维持体内平衡和宿主防御微生物方面起着至关重要的作用。早期补体级联成分的缺乏与系统性红斑狼疮 (SLE) 的易感性增加有关，而补体的过度消耗是这种疾病的标志。尽管最初认为免疫复合物增强的经典途径激活是狼疮肾炎 (LN) 发病机制的主要原因，但越来越多的证据表明替代途径和凝集素途径也参与其中。针对补体激活的治疗药物已在 LN 患者中使用，并且正在进行临床试验。我们综述了补体系统失调导致 SLE 肾脏损伤的机制，并总结了最新的关于使用抗补体药物来治疗这种疾病的证据。
