Objective To describe the clinical and serological features of patients with antineutrophil cytoplasmic antibody(ANCA)-associated vasculitis(AAV)in eastern China based on data from a hospital-based study.Indicators that can predict disease activity are explored.Methods Two hundred and fifty-six AAV patients diagnosed during January 1,2009 to December 31,2020 in Zhongshan Hospital,Fudan University were enrolled.The clinical features were retrospectively analyzed,SPSS 25.0 was used to analyze the data,t-test was applied to compare the differences between the laboratory indicators of the active group and the inactive group.Multivariate logistic regression analysis was used to explore the biological markers related to the disease activity of AAV.Receiver operating characteristic curve(ROC)was used to analyze the diagnostic efficacy of different indicators.Results Among 256 AAV patients,193 were MPA,48 were GPA and 15 were EGPA.The mean age at diagnosis of patients was(58.0±15.7)years,with MPA patients were about 10 years older than patients with GPA and EGPA.MPA and EGPA were positive for MPO-ANCA(88.6％and 40.0％,respectively),while GPA was mainly positive PR3-ANCA(83.3％).For all patients,the lungs and kidneys were the most commonly affected(68.4％and 60.9％,respectively),the era-nose-throat(ENT)were commonly affected(60.4％)in GPA,while skin(60.0％)and nervous system(46.7％)were commonly being affected in EGPA patients.The results of multivariate regression analysis showed that MPO-ANCA＞66.15 U/ml[OR(95％CI)=2.757(1.023,9.461),P=0.001],ESR＞69.5 mm/1 h[OR(95％CI)=3.962(1.014,9.461),P=0.002],and C4＞0.283 g/L[OR(95％CI)=9.252(3.366,15.791),P＜0.001]were independent risk factors for active disease.Diagnostic tests showed that C4 had the highest diagnostic efficacy in terms of sensitivity(82.4％),negative predictive value(58.3％)and area under the receiver operating characteristic(AUC=0.854).Combined test analysis suggested that C4 could significantly improve the sensitivity or specificity of ESR and MPO-ANCA.Conclusion C4 can serve as a potential biological marker for evaluating the disease activity of AAV patients.
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