目的分析抗中性粒细胞胞质抗体(ANCA)相关小血管炎(AASV)患者的临床表现、诊断、分类、实验室特征、治疗及转归;探讨ANCA滴度判断AASV患者病情活动的价值及滴度高低对治疗及转归的影响,以提高对该类疾病的认识。方法选取2010年1月—2014年12月新疆医科大学第一附属医院ANCA阳性且经临床表现、实验室检查确诊为AASV的患者117例,将其按照ANCA滴度分为低滴度组(≤100 RU/ml,n=61)和高滴度组(>100RU/ml,n=56),观察两组患者临床表现、实验室指标、治疗方案及转归。结果 117例患者中110例(94.0%)诊断为显微镜下多血管炎(MPA),仅有7例(6.0%)诊断为肉芽肿性多血管炎(GPA)。低滴度组伯明翰血管炎活动性评分(BVAS)为(13.7±0.9)分,低于高滴度组的(18.3±0.8)分(t=3.890,P<0.001)。两组乏力、发热、关节痛、肌肉痛发生率比较,差异均无统计学意义(P>0.05);高滴度组体质量下降发生率高于低滴度组(P<0.05)。两组血尿、高血压发生率比较,差异无统计学意义(P>0.05);高滴度组蛋白尿、血肌酐升高发生率高于低滴度组(P<0.05)。两组呼吸系统、消化系统、心血管系统、眼耳鼻喉、神经系统受累发生率比较,差异均无统计学意义(P>0.05);高滴度组皮肤改变发生率高于低滴度组(P=0.027)。两组血红蛋白、血小板计数、C反应蛋白(CRP)、C_3、C_4水平比较,差异均无统计学意义(P>0.05);高滴度组白细胞计数、红细胞沉降率均高于低滴度组(P<0.05)。高滴度组强化治疗方案使用率(41.1%,23/56)高于低滴度组(19.7%,12/61)(χ~2=6.377,P=0.012)。两组患者改善率、终末期肾脏病(ESRD)发生率比较,差异均无统计学意义(P>0.05);高滴度组病死率(32.1%,18/56)高于低滴度组(13.6%,8/61)(P<0.05)。低滴度组平均生存时间为44.7个月〔SE=3.572,95%CI(37.703,51.706)〕,高滴度组平均生存时间为30.6个月〔SE=3.191,95%CI(24.390,36.899)〕。KaplanMeier生存曲线显示,低滴度组1年生存率为93.4%,5年生存率为75.5%;高滴度组1年生存率为59.9%,5年生存率为54.9%,高滴度组患者生存率低于低滴度组(χ~2=4.565,P=0.033)。结论 AASV患者ANCA高滴度者在病情活动度、肾脏受累及病死率方面较低滴度者更为严重,提示ANCA滴度在辅助疾病诊断、反映病情活动、指导治疗及判断预后方面有一定意义,应予以重视。 Objective To analyze the clinical manifestations, diagnosis, classification, laboratory characteristics, treatment and outcome of anti-neutrophil cytoplasmic antibodies (ANCA) -related vasculitis (AASV). To investigate the value of ANCA titers in judging the severity of AASV patients’ The impact of titers on treatment and prognosis in order to improve awareness of such diseases. Methods From January 2010 to December 2014, 117 patients with ANCA positive and clinically diagnosed as AASV in the First Affiliated Hospital of Xinjiang Medical University were enrolled. According to the ANCA titer, 117 patients were divided into low titer group (≤ 100 RU / ml, n = 56). The clinical manifestations, laboratory parameters, treatment regimens and outcomes of the two groups were observed. Results Of the 117 patients, 110 (94.0%) were diagnosed with microscopic polyangitis (MPA) and only 7 (6.0%) were diagnosed as granulomatous polyangiitis (GPA). The BVAS score was (13.7 ± 0.9) in the low-titer group and (18.3 ± 0.8) in the high titer group (t = 3.890, P <0.001). There was no significant difference in the incidence of fatigue, fever, arthralgia and muscle ache between the two groups (P> 0.05). The incidence of body weight loss in high titer group was higher than that in low titer group (P <0.05). There was no significant difference between the two groups in the incidence of hematuria and hypertension (P> 0.05). The incidence of high-titer histoneuria and serum creatinine was higher than that of the low-titer group (P <0.05). There was no significant difference in the incidence of respiratory system, digestive system, cardiovascular system, ocular ENT, and nervous system involvement between the two groups (P> 0.05). The incidence of skin changes in high titer group was higher than that in low titer group P = 0.027). There was no significant difference in hemoglobin, platelet count, CRP, C_3 and C_4 between the two groups (P> 0.05). The white blood cell count and the erythrocyte sedimentation rate in the high titer group were higher than those in the low titer group P <0.05). The use of intensive treatment regimen in high-titer group (41.1%, 23/56) was higher than in low titer group (19.7%, 12/61) (χ ~ 2 = 6.377, P = 0.012). There was no significant difference between the two groups in the rate of improvement and the incidence of end-stage renal disease (ESRD) (P> 0.05). The mortality in high titer group (32.1%, 18/56) 13.6%, 8/61) (P <0.05). The mean survival time in low titer group was 44.7 months (SE = 3.572, 95% CI, 37.703, 51.706), and the average survival time in high titer group was 30.6 months (SE = 3.191, 95% CI, 24.390, 36.899) ]. Kaplan-Meier survival curves showed that the 1-year survival rate was 93.4% in low-titer group and 75.5% in 5-year low-titer group. The 1-year survival rate in high titer group was 59.9%, and the 5-year survival rate was 54.9% The survival rate was lower than the low titer group (χ ~ 2 = 4.565, P = 0.033). Conclusion Patients with high ANCA ATSV titer in the activity of the disease, renal involvement and lower mortality were more serious, suggesting that ANCA titer in the diagnosis of auxiliary diseases, reflecting the activities of the disease, guiding the treatment and prognosis of a certain significance , Should be taken seriously.