ANCA相关性血管炎患者临床特征及相关危险因素分析

Clinical Characteristics and Analysis of Risk Factors with ANCA-Associated Vasculitides

摘要: 目的：描述抗中性粒细胞胞浆抗体(antineutrophil cytoplasimc antibody, ANCA)相关血管炎(ANCA-associated vasculitis, AAV)患者的临床特征，分析影响生存率的相关危险性因素。方法：回顾分析了2010年至2019年在遂宁市中心医院新确诊为AAV患者的基本资料、临床特征、生存结局，并对相关危险因素进行统计学分析。结果：纳入AAV患者115例，显微镜下多血管炎(microscopic polyangiitis, MPA) 107例(93%)，肉芽肿性多血管炎(granulomatosis with polyangiitis, GPA) 8例(7.0%)，平均年龄63.7 ± 13.9 (14~89)岁，大于60岁的老年患者占72.2% (83例)。AAV相关症状发作到明确诊断的中位时间为4 (1~60)个月，平均BVAS为16.5 ± 6.7 (6~35)分，肾脏损害占86.1%，呼吸道损害占53.1%。死亡患者有46例，死亡原因为严重感染有25例(占54.3%)，尿毒症并发症有7例(占15.2%)，心血管不良事件有4件(占8.7%)，血管炎活跃有6例(占13.0%)，颅内出血有1例(占2.2%)，不明原因有3例(占6.5%)。患者1年生存率为71.3%，5年生存率为60.8%。Kaplan-Meier生存曲线分析显示60岁以上患者生存率低(P = 0.042)；血肌酐 > 400 umol/L的患者生存率低(P = 0.005)；血小板计数 > 250 × 109/L患者的生存率低(P = 0.003)。进一步COX分析显示年龄(HR = 1.041, 95%CI = 1.014~1.068, P = 0.003)、血肌酐水平(HR = 1.085, 95%CI = 1.008~1.253, P = 0.002)、血小板(HR = 1.004, 95%CI = 1.001~1.007, P = 0.006)是影响患者生存率的独立危险因素。结论：老年人、血肌酐 > 400 umol/L、血小板 > 250 × 10⁹/L是影响AAV患者生存率的独立危险因素。

Abstract: Objective: To describe the clinical characteristics of patients with antineutrophil cytoplasmic anti-body (ANCA)-associated vasculitis (ANCA-associated vasculitis, AAV), and analyze the related risk factors affecting survival. Methods: The basic data, clinical characteristics, and survival outcomes of patients newly diagnosed with AAV in Suining Central Hospital from 2010 to 2019 were retrospec-tively analyzed, and related risk factors were statistically analyzed. Results: A total of 115 patients with AAV were included, 107 patients (93%) with microscopic polyangiitis (MPA) and 8 patients (7.0%) with granulomatosis with polyangiitis (GPA), with an average age of 63.7 ± 13.9 (14~89) years old, and elderly patients over 60 years old accounted for 72.2% (83 cases). The median time from onset of AAV-related symptoms to definite diagnosis was 4 (1~60) months, the mean BVAS was 16.5 ± 6.7 (6~35) points, kidney damage accounted for 86.1%, and respiratory damage accounted for 53.1%. There were 46 deaths, among which 25 (54.3%) died of severe infection, 7 (15.2%) ure-mic complications, 4 (8.7%) adverse cardiovascular events, and 6 (13.0%) active vasculitis, 1 (2.2%) intracranial hemorrhage, and 3 (6.5%) cases of unknown cause. The 1-year survival rate was 71.3% and the 5-year survival rate was 60.8%. Kaplan-Meier survival curve analysis showed that the survival rate of patients over 60 years old was low (P = 0.042); the survival rate of patients with serum creatinine > 400 umol/L was low (P = 0.005); the survival rate of patients with platelet count > 250 × 109/L was low (P = 0.003). Further COX analysis showed that age (HR = 1.041, 95%CI = 1.014~1.068, P = 0.003), serum creatinine level (HR = 1.085, 95%CI = 1.008~1.253, P = 0.002), platelets (HR = 1.004, 95%CI = 1.001~1.007, P = 0.006) was an independent risk factor affecting the survival rate of patients. Conclusion: The elderly, serum creatinine > 400 umol/L, platelet > 250 × 10⁹/L are independent risk factors affecting the survival rate of AAV patients.