Romano Roberta, Giardino Giuliana, Cirillo Emilia, Prencipe Rosaria, Pignata Claudio
Department of Translational Medical Sciences - Section of Pediatrics, Federico II University of Naples, Naples, Italy.
Int Rev Immunol. 2021;40(3):159-170. doi: 10.1080/08830185.2020.1833877. Epub 2020 Oct 16.
The complement system is a multi-functional system representing the first line host defense against pathogens in innate immune response, through three different pathways. Impairment of its function, consisting in deficiency or excessive deregulated activation, may lead to severe systemic infections or autoimmune disorders. These diseases may be inherited or acquired. Despite many diagnostic tools are currently available, ranging from traditional, such as hemolytic or ELISA based assays, to innovative ones, like next generation sequencing techniques, these diseases are often not recognized. As for therapeutic aspects, strategies based on the use of targeted drugs are now widespread. The aim of this review is to present an updated overview of complement system pathophysiology, clinical implications of its dysfunction and to summarize diagnostic and therapeutic approaches.
补体系统是一个多功能系统，通过三种不同途径在先天性免疫应答中代表宿主抵御病原体的第一道防线。其功能受损，包括缺陷或过度失调的激活，可能导致严重的全身感染或自身免疫性疾病。这些疾病可能是遗传性的或后天获得的。尽管目前有许多诊断工具，从传统的如基于溶血或酶联免疫吸附测定的检测方法到创新的如新一代测序技术，但这些疾病往往未被识别。至于治疗方面，基于使用靶向药物的策略目前已广泛应用。本综述的目的是提供补体系统病理生理学的最新概述、其功能障碍的临床意义，并总结诊断和治疗方法。
