Systemic lupus erythematosus (SLE) is an autoimmune disease. Its pathogenesis is not entirely clear, and its present therapeutic method and effect are not accurate. In recent years, studies on B-cell activating factor (BAFF), a new family member of tumor necrosis factor, and its relationship with the incidence and clinical treatment of SLE have been reported. The paper summarizes the relevant researches from the following aspects: research process of BAFF/a proliferation-inducing ligand (APRIL) system; the role of BAFF/APRIL system in the pathogenesis of SLE; relationship between BAFF/APRIL system and different clinical phenotypes of SLE and research process of this system being a biomarker of disease activity; the relationship between BAFF/APRIL system and targeted therapy of SLE. The paper provides references for the further study of BAFF/APRIL system and SLE.