Systemic lupus erythematosus (SLE) is characterized by excessive production of various autoantibodies, which play important roles in the pathogenesis of SLE. Apart from classical autoantibodies such as anti-double stranded DNA antibody (anti-dsDNA), anti-Smith antibody (anti-Sm), and anti-phospholipid antibody (APL), recent studies focus on some novel autoantibodies including anti-complement (C) 1q antibody (anti-C1q), which is closely correlated with lupus nephritis; anti-N-methyl-D-asparate receptor antibody (NMDAR), which mediates neuropsychiatric manifestations in SLE to some extent; anti-galectin, which is involved in secondary anti-phospholipid syndrome (APS) in SLE; and anti-Müllerian hormone (AMH), which represents a more specific biomarker for subclinical ovarian damage caused by the disease itself or cytotoxic drugs in SLE patients. Correlation of these autoantibodies with disease activity and organ involvement of SLE may help to evaluate disease severity, efficacy of treatment, and long-term prognosis. Furthermore, combined measurement of a variety of autoantibodies is supposed to be more valuable in estimating disease activity and severity.