Abstract: Systemic lupus erythematosus (SLE) is an autoimmune disease with complex pathogenesis and diverse clinical manifestations. In recent years, as theoretical and technological innovations in the postgenomic era, specifically advances in gene detection techniques, over 30 diseasecausing genes for SLE have been identified and the concept of “Monogenic lupus” has been proposed. This review will focus on the pathogenesis, clinical manifestations and genetic diagnosis of these genes, so as to raise awareness of the genetic contribution to SLE and advance further research.