Systemic lupus erythematosus (SLE) is a multisystem, autoimmune, connective-tissue disorder with a broad range of clinical presentations. SLE predominantly affects women, especially from ethnic groups with African and Asian ancestry. This disorder is a chronic illness that can be life threatening when major organs are affected, but more commonly results in debilitating condition and affects employment and fertility. This article summarizes the recent advances in our understanding of the genetics, epidemiology and pathogenesis of SLE. In addition, progress in the assessment and management of serious complications such as lupus nephritis and CNS lupus is reviewed. New therapeutic approaches, such as low-dose cyclophosphamide regimens, mycophenolate mofetil and biological agents are also discussed.