Rudbeck Laboratory C5:3, Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden.
Centre of Biomaterials Chemistry, Linnaeus University, Kalmar, Sweden.
Front Immunol. 2018 Oct 24;9:2237. doi: 10.3389/fimmu.2018.02237. eCollection 2018.
Complement system aberrations have been identified as pathophysiological mechanisms in a number of diseases and pathological conditions either directly or indirectly. Examples of such conditions include infections, inflammation, autoimmune disease, as well as allogeneic and xenogenic transplantation. Both prospective and retrospective studies have demonstrated significant complement-related differences between patient groups and controls. However, due to the low degree of specificity and sensitivity of some of the assays used, it is not always possible to make predictions regarding the complement status of individual patients. Today, there are three main indications for determination of a patient's complement status: (1) complement deficiencies (acquired or inherited); (2) disorders with aberrant complement activation; and (3) C1 inhibitor deficiencies (acquired or inherited). An additional indication is to monitor patients on complement-regulating drugs, an indication which may be expected to increase in the near future since there is now a number of such drugs either under development, already in clinical trials or in clinical use. Available techniques to study complement include quantification of: (1) individual components; (2) activation products, (3) function, and (4) autoantibodies to complement proteins. In this review, we summarize the appropriate indications, techniques, and interpretations of basic serological complement analyses, exemplified by a number of clinical disorders.
补体系统异常被认为是许多疾病和病理状况的病理生理机制，无论是直接还是间接的。这种情况的例子包括感染、炎症、自身免疫性疾病以及同种异体和异种移植。前瞻性和回顾性研究都表明，患者组和对照组之间存在显著的补体相关性差异。然而，由于一些使用的检测方法特异性和敏感性较低，并不总是能够对个体患者的补体状态做出预测。今天，确定患者补体状态有三个主要指征：(1)补体缺乏（获得性或遗传性）；(2)补体异常激活的疾病；和(3)C1 抑制剂缺乏（获得性或遗传性）。另一个指征是监测接受补体调节药物治疗的患者，由于目前有许多此类药物正在开发中、已经进入临床试验或临床使用，预计这种指征在不久的将来会增加。用于研究补体的现有技术包括：(1) 单个成分的定量；(2) 激活产物；(3) 功能；和(4) 补体蛋白的自身抗体。在这篇综述中，我们总结了基本血清学补体分析的适当指征、技术和解释，并用一些临床疾病举例说明。
