Neuropsychiatric systemic lupus erythematosus (NPSLE) is a serious complication of systemic lupus erythematosus (SLE) involving damage to the nervous system, most prevalent among young women and no effective treatment having been found. The imbalance between the formation and degradation of neutrophil extracellular traps (NETs) leads to disturbances in immune function, thus leading to autoimmune diseases. In this review，the pathogenesis of NPSLE is expounded from aspects involving the blood-brain barrier, cerebrovascular pathologies, the serum and cerebrospinal fluid, cytokines, inflammatory responses and the complement system. In addition, we further explore the roles of NETs formation in NPSLE.