Welch T R
Division of Nephrology and Hypertension, Children's Hospital Research Foundation and Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA.
Nephron. 2001 Jul;88(3):199-204. doi: 10.1159/000045990.
The complement system has long been recognized as having a role in immune glomerular disease. This review provides an update on this association, some strategies for the clinical testing of complement in disease, and a brief commentary on current research directions. Evidence of complement activation in glomerulonephritis comes from characteristic patterns of a decrease in the serum concentrations of specific components, some of which are virtually diagnostic of certain nephritides. These patterns are often accompanied by the presence of complement components in the glomeruli and the detection of complement breakdown products in the circulation. In certain diseases, circulating complement-activating substances can be detected. Although there are over 20 complement proteins, clinical analysis is most often directed at C3 and C4, with occasional measurement of B and C5. Recently, a variety of mechanisms for complement-induced injury has been recognized. These mechanisms go far beyond simple passive lysis of erythrocytes, the earliest functional effect of complement studied. The role of such mechanisms in renal disease is just beginning to be studied. Local synthesis of complement components in the kidney may play a role both in host defense and in the promotion of interstitial inflammation and scarring. Such mechanisms will likely be defined more precisely with the availability of animals with specific complement deficiencies. Ultimately, an understanding of the role of complement in renal disease may permit specific targeted inhibition of one or more complement functions as a form of therapy.
补体系统在免疫性肾小球疾病中的作用早已得到认可。本综述对这种关联进行了更新，介绍了疾病中补体临床检测的一些策略，并对当前的研究方向作了简要评论。肾小球肾炎中补体激活的证据来自特定成分血清浓度降低的特征性模式，其中一些实际上可用于诊断某些肾病。这些模式通常伴随着肾小球中补体成分的存在以及循环中补体裂解产物的检测。在某些疾病中，可检测到循环中的补体激活物质。尽管有20多种补体蛋白，但临床分析大多针对C3和C4，偶尔也会检测B和C5。最近，人们认识到了多种补体诱导损伤的机制。这些机制远远超出了补体最早被研究的功能效应——红细胞的简单被动溶解。此类机制在肾脏疾病中的作用才刚刚开始研究。肾脏中补体成分的局部合成可能在宿主防御以及促进间质炎症和瘢痕形成中都发挥作用。随着具有特定补体缺陷的动物的出现，此类机制可能会得到更精确的定义。最终，了解补体在肾脏疾病中的作用可能会使针对一种或多种补体功能的特异性靶向抑制成为一种治疗形式。
