Abstract: Objective To analyze the clinical features of patients with lupus nephritis positive for antineutrophil cytoplasmic antibodies (ANCA) and explore the clinical implications of ANCA detection. Methods Totally 261 patients with lupus nephritis were enrolled in this study, including 53 ANCA-positive and 208 ANCA-negative ones. The clinical data of the patients pertaining to the disease history, physical examination, laboratory examinations and pathological inspection were retrospectively analyzed. Results Compared with patients negative for ANCA, the ANCA-positive patients had significantly higher incidence of serositis (75.5%), acute renal failure (64.2%), myocarditis (30.2%), neuropsychiatric involvement (26.4%)and lung homorrhage(7.5%)(P<0.05). Significant differences were also found between the two groups in SLE disease active index (SLE-DAI), number of the diagnostic criteria, erythrocyte sedimentation rate (ESR), anemia, anti-Sm antibodies, and serum complement C3. Most patients positive for ANCA(67.9%) had type Ⅳ lupus nephritis with more crescent formation, renal tubular atrophy, hyaline thrombi, and higher mortality rate as well than the negative patients. Conclusion ANCA detection may benefit the estimation of the disease severity and prognostic evaluation of lupus nephritis.