Pediatric Rheumatology Division, Duke University Children's Health Center, 2301 Erwin Road, Durham, NC, 27710, USA,
Curr Allergy Asthma Rep. 2014 Jul;14(7):448. doi: 10.1007/s11882-014-0448-2.
The complement system is a major, multifunctional part of innate immunity and serves as a bridge between the innate and adaptive immune systems. It consists of more than 30 distinct proteins that interact with one another in a specific sequence. There are three pathways of complement activation: the classical, the lectin, and the alternative pathways. The three pathways are initiated by distinct mechanisms, but they all generate the same core set of effector molecules. Inherited complete deficiencies in complement components are generally very rare and predispose to infections and autoimmune disease. One of the better described associations is between deficiencies in early classical pathway components and the development of systemic lupus erythematosus. The goal of this review will be to discuss the associations between and the causal mechanisms of complement deficiencies and systemic lupus erythematosus.
补体系统是先天免疫系统的一个主要的、多功能部分，是先天免疫系统和适应性免疫系统之间的桥梁。它由 30 多种不同的蛋白质组成，这些蛋白质相互作用形成特定的序列。补体激活有三条途径：经典途径、凝集素途径和替代途径。三条途径的启动机制不同，但都产生相同的核心效应分子。遗传性补体成分完全缺乏通常非常罕见，易导致感染和自身免疫性疾病。其中一个描述得较好的关联是早期经典途径成分缺乏与系统性红斑狼疮的发生之间的关联。本篇综述的目的将是讨论补体缺乏与系统性红斑狼疮之间的关联和因果机制。
