Complement analysis 2016: Clinical indications, laboratory diagnostics and quality control
Complement analysis 2016: Clinical indications, laboratory diagnostics and quality control
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DOI：
10.1016/j.imbio.2016.06.008
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发表时间：
2016-11-01
期刊：
影响因子：
2.8
通讯作者：
Kirschfink, Michael
中科院分区：
文献类型：
--
作者：
Prohaszka, Zoltan;Nilsson, Bo;Kirschfink, Michael
近年来，体液和活检的补体分析，远远超出了C3和C4，大大提高了我们对疾病过程的理解。这种扩展的补体分析允许更精确的鉴别诊断和补体靶向治疗的关键监测。这些变化是对补体参与多种疾病的理解不断加深的结果。为了理解正确的补体分析的重要性，重要的是要了解它在疾病中的作用。从历史上看，这是缺乏补体，表现在严重感染，指出。从那时起，补体已被连接到各种炎症性疾病，如自身免疫性疾病和遗传性血管性水肿。虽然补体在肾移植物排斥反应中的作用已被认识较久，但补体的显著影响。在某些肾病中，现在已经导致了一些罕见肾脏疾病的重新分类，并增加了补体分析在诊断中的作用。更出乎意料的是，补体也与神经、眼科和皮肤病有关。由于这种程度的参与，在一些不同的和有影响力的健康问题，适当的补体测试显然是重要的;然而，补体系统的分析在实验室之间差异很大。除了一些蛋白质，如C3和C4，既没有良好的表征标准制剂，也没有校准的测定。这对于评估经典、替代或凝集素途径功能的试验的实验室间变化尤其如此。此外，需要对补体活化产物的测量进行标准化，这对于确定体内是否发生临床相关的补体活化非常关键。最后，针对补体蛋白（例如抗C1 q）、C3和C4转化酶（C3和C4肾炎因子）或调节蛋白（例如抗Clinhibitor、抗H因子）的自身抗体在定义基于补体失调的自身免疫过程和疾病中是重要的。为了提高补体实验室分析的质量，国际补体学会（ICS）和国际免疫学会联合会（IUIS）成立了一个标准化委员会，为现代补体分析提供指导方针，并为国际测试项目的发展提供标准。(C)2016 Elsevier GmbH. All rights reserved.
In recent years, complement analysis of body fluids and biopsies, going far beyond C3 and C4, has significantly enhanced our understanding of the disease process. Such expanded complement analysis allows for a more precise differential diagnosis and for critical monitoring of complement-targeted therapy. These changes are a result of the growing understanding of the involvement of complement in a diverse set of disorders. To appreciate the importance of proper complement analysis, it is important to understand the role it plays in disease. Historically, it was the absence of complement as manifested in severe infection that was noted. Since then complement has been connected to a variety of inflammatory disorders, such as autoimmune diseases and hereditary angioedema. While the role of complement in the rejection of renal grafts has been known longer, the significant impact of complement. In certain nephropathies has now led to the reclassification of some rare kidney diseases and an increased role for complement analysis in diagnosis. Even more unexpected is that complement has also been implicated in neural, ophtalmological and dermatological disorders. With this level of involvement in some varied and impactful health issues proper complement testing is clearly important; however, analysis of the complement system varies widely among laboratories. Except for a few proteins, such as C3 and C4, there are neither well-characterized standard preparations nor calibrated assays available. This is especially true for the inter-laboratory variation of tests which assess classical, alternative, or lectin pathway function. In addition, there is a need for the standardization of the measurement of complement activation products that are so critical in determining whether clinically relevant complement activation has occurred in vivo. Finally, autoantibodies to complement proteins (e.g. anti-C1q), C3 and C4 convertases (C3 and C4 nephritic factor) or to regulatory proteins (e.g. anti-Clinhibitor, anti-factor H) are important in defining autoimmune processes and diseases based on complement dysregulation. To improve the quality of complement laboratory analysis a standardization commmittee of the International Complement Society (ICS) and the International Union of Immunological Societies (IUIS) was formed to provide guidelines for modern complement analysis and standards for the development of international testing programs. (C) 2016 Elsevier GmbH. All rights reserved.