补体参与了多种疾病的发生发展,在天然免疫和获得性免疫中起着重要的作用。随着研究水平的进步,研究者发现很多既往病因不清的疾病与补体系统的异常活化密切相关,如阵发性睡眠性血红白尿、C3肾小球病、不典型溶血尿毒综合征等。而对于补体系统的检测也从最初将其作为反映疾病活动度水平的指标,逐步成为很多疾病的临床诊断及治疗的靶点,更成为开发新型靶向药物的重要理论依据。
Complement system is involved in the pathogenesis of a group of diseases and also provides an important interface between the innate and adaptive immune response. Currently available tools provide an exciting opportunity to study this system, and some diseases were found to be resuhed from excessive and uncontrolled activation of the complement of which the pathogenesis used to be unclear, such as paroxysmal nocturnal hemoglobinuria (PNH), atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G), etc. Several complement inhibitors have been approved for clinical use, and additional complement inhibitory drugs are in development. The use of these drugs in patients will expand our understanding of the benefits and limitations of complement inhibition in human diseases.