Abstract: Objective To study the significance of antineutrophil cytoplasmic antibodies （ANCA） and its relationship with systemic lupus erythematosus （SLE）. Methods Sixty patients with SLE and sixty normal controls were enrolled in this study, and divided into ANCA-positive and ANCA-negative groups based on ELISA results. The data on patients' general state of health, clinical manifestations and laboratory examination results were collected by questionnaire or medical records. Results The positive rate of ANCA was significantly higher in SLE group than in normal controls （33.3% vs 3.3%, P < 0.001）, and in active SLE patients than in non-active SLE patients（84.62% vs 19.15%, P < 0.01）. Compared with patients negative for ANCA, the ANCA-positive patients had a significantly higher incidence of nervous system disorders, myocarditis, renal damage and serositis, decrease of serum complement level, increase of serum IgG level, and a higher positive rate of anti-dsDNA antibody, anti-Sm antibody and γ-globulin （all P < 0.05）. The SLE disease activity index and ESR value were significantly higher in ANCA-positive patients than those in ANCA-negative patients （24.40 ± 10.16 vs 11.30 ± 6.07, 84.45 ± 29.03 vs 47.07 ± 26.31, respectively, both P < 0.05）. The patients with lupus nephritis had a significantly higher positive rate of ANCA than that without nephritis （46.7% vs 21.9%, P < 0.05）. Conclusion ANCA may serve as a parameter for the assessment of disease activity of SLE and differentiation of SLE with lupus nephritis from those without.