PDF(Pubmed)
Abstract：
C3 glomerulopathy is a rare disease, characterized by an abnormal activation of the complement\'s alternative pathway that leads to the accumulation of the C3 component in the kidney. The disease recurs in more than half of kidney transplant recipients, with a significant impact on graft survival. Recurrence of the primary disease represents the second cause of graft loss after organ rejection. In C3 glomerulopathy, there are several risk factors which can promote a recurrence during transplantation, such as delayed graft function, infection and monoclonal gammopathy. All these events can trigger the alternative complement pathway. In this review, we summarize the impact of C3 glomerulopathy on kidney grafts and present the latest treatment options. The most widely used treatments for the disease include corticosteroids and mycophenolate mofetil, which are already used chronically by kidney transplant recipients; thus, additional treatments for C3 glomerulopathy are required. Currently, several studies using anti-complement drugs (i.e., eculizumab, Ravalizumab, avacopan) for C3 glomerulopathy in kidney transplant patients are ongoing with encouraging results.
摘要：
C3肾小球病是一种罕见的疾病，以补体替代途径的异常激活为特征，导致C3成分在肾脏中的积累。这种疾病在一半以上的肾移植受者中复发，对移植物存活有重大影响。原发疾病的复发是器官排斥后移植物丢失的第二个原因。在C3肾小球病中，有几个风险因素可以促进移植期间的复发，例如延迟的移植物功能，感染和单克隆丙种球蛋白病。所有这些事件都可以触发替代补体途径。在这次审查中,我们总结了C3肾小球病对肾移植物的影响,并提出了最新的治疗方案.该疾病最广泛使用的治疗方法包括皮质类固醇和霉酚酸酯，肾移植受者已经长期使用；因此，C3肾小球病需要额外的治疗。目前,使用抗补体药物的几项研究(即，依库珠单抗,Ravalizumab,avacopan）用于肾移植患者的C3肾小球病正在进行中，结果令人鼓舞。
