Abstract

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.

摘要

补体系统是一种多功能系统，通过三种不同的途径代表先天免疫反应中针对病原体的第一线宿主防御。其功能受损，包括缺乏或过度失调的激活，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的，也可能是后天获得的。尽管目前有许多诊断工具可用，从传统的（例如基于溶血性或 ELISA 的检测）到创新的工具（例如下一代测序技术），但这些疾病通常未被识别。至于治疗方面，基于使用靶向药物的策略现在很普遍。本综述的目的是提供补体系统病理生理学的最新概述，