Abstract: The pathogenesis of systemic lupus erythematosus is complex， and it is currently believed that genetic susceptibility，immune and environmental factors are involved. With the development of gene detection technology， more and more monogenic lupus has been found. The different genetic variants and the causal molecular pathway are associated with distinct clinical manifestations and have different suitable targeted therapies. This article aims to summarize the clinical characteristics and possible targets for therapies of monogenic lupus in children with different pathways， hoping to provide clues for clinicians to identify patients with monogenic lupus in the early stage and to provide individualized treatment as soon as possible.

Key words: child, monogenic, lupus, clinical manifestations, treatment

摘要： 系统性红斑狼疮的发病机制较为复杂，目前认为与遗传、环境及免疫等因素相关。随着基因检测技术的发展，越来越多的单基因狼疮被发现。根据致病基因/途径的不同，儿童单基因狼疮的临床表现也不同，有不同的靶向治疗方案。该文将总结不同通路儿童单基因狼疮的临床特点和可能的治疗靶点，为临床医师早期识别单基因狼疮患者提供帮助，并尽早进行个体化治疗。

关键词: 儿童, 单基因, 狼疮, 临床表现, 治疗