抗中性粒细胞胞质抗体相关性血管炎患者的临床特征及预后分析

目的 分析抗中性粒细胞胞质抗体（ANCA）相关性血管炎（AAV）患者的临床特征及预后因素。方法 回顾性分析我院2008年7月至2016年12月初诊的168例AAV患者的一般情况、临床表现及实验室指标，并对所有患者进行随访，中位随访时间为12（1，96）个月，终点事件为死亡。结果 168例患者中，显微镜下多血管炎（MPA）、肉芽肿性多血管炎（GPA）及嗜酸细胞性肉芽肿性多血管炎（EGPA）分别为152、14、2例；MPO-ANCA阳性145例（86.3%），PR3-ANCA阳性20例（11.9%）；49例患者死亡，91例患者存活，28例失访。与GPA患者相比，MPA患者间质性肺病50.7%（77/152）vs 14.3%（2/14）]及肾脏受累78.9%（120/152）vs 50.0%（7/14）]常见，估计的肾小球滤过率（eGFR）更低14.23（7.27，71.49）mL/（min·1.73 m²）vs 104.08（16.61，135.72）mL/（min·1.73 m²）]，差异均有统计学意义（P均<0.05）。与PR3-ANCA阳性组相比，MPO-ANCA阳性组患者年龄较大（64.01±10.62）岁vs（50.50±16.88）岁]，肾脏受累更多见77.9%（113/145）vs 50.0%（10/20）]，eGFR更低19.00（9.40，42.85）mL/（min·1.73 m²）vs 149.40（86.75，249.45）mL/（min·1.73 m²）]，差异均有统计学意义（P均<0.05）。与非死亡组相比，死亡组AAV患者年龄更大（67.45±10.61）岁vs（61.98±12.52）岁]，间质性肺病发病率更高59.2%（29/49）vs 41.8%（38/91）]，伯明翰系统性血管炎活动评分（BVAS）更高（18.53±8.02）分vs（13.68±5.98）分]，eGFR更低8.58（5.73，22.07）mL/（min·1.73 m²）vs 45.15（11.54，120.79）mL/（min·1.73 m²）]，血钠浓度137.00（134.00，140.00）mmol/L vs 139.00（136.00，141.00）mmol/L]及白蛋白水平（28.41±5.24）g/L vs（31.92±5.91）g/L]更低，血清D-二聚体水平更高2.84（1.20，6.28）mg/L vs 2.24（0.80，3.69）mg/L]，差异均有统计学意义（P均<0.05）。多因素Cox比例风险回归分析显示年龄、eGFR、血清白蛋白水平及BVAS是AAV患者死亡的独立影响因素（HR=1.058、0.987、0.932、1.086，P均<0.05）。结论 AAV患者临床表现以肾脏受累和肺受累多见，年龄、eGFR、血清白蛋白水平及BVAS是AAV患者死亡的独立影响因素，间质性肺病、血清D-二聚体水平高、低钠血症也可能与AAV预后有关。

Clinical characteristics and prognosis of patients with anti-neutrophil cytoplasmic antibody-associated vasculitis

Objective To analyze the clinical characteristics and prognosis of patients with anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV). Methods Clinical data (general information, clinical manifestation, and laboratory indexes) of 168 patients newly diagnosed as AAV between Jul. 2008 and Dec. 2016 were retrospectively analyzed. Patients were followed up for a median period of 12 months (range, 1-96 months). End-point event was death. Results There were 152 cases of microscopic polyangiitis (MPA), 14 cases of granulomatous polyangiitis (GPA) and 2 cases of eosinophilic granulomatosis with polyangiitis (EGPA). MPO-ANCA was positive in 145 (86.3%) patients and PR3-ANCA was positive in 20 (11.9%) patients. Forty-nine patients died, 91 patients survived, and 28 patients lost their visits. Interstitial lung disease and renal involvement were more common in MPA patients as compared with GPA patients (50.7%77/152]vs 14.3%2/14], 78.9%120/152]vs 50.0%7/14], all P<0.05). Estimated glomerular filtration rate (eGFR) in MPA patients was significantly lower than that in GPA patients (14.237.27, 71.49]mL/min·1.73 m²]vs 104.0816.61, 135.72]mL/min·1.73 m²], P<0.05). Compared with PR3-ANCA positive patients, MPO-ANCA positive patients was more elder (64.01±10.62]years vs50.50±16.88]years), had more renal involvement (77.9%113/145]vs 50.0%10/20]) and lower eGFR (19.009.40, 42.85] mL/min·1.73 m²]vs 149.4086.75, 249.45] mL/min·1.73 m²], all P<0.05). Compared with the survivals, the dead patients were older (67.45±10.61]years vs61.98±12.52]years), had higher incidence of interstitial lung disease (59.2%29/49]vs 41.8%38/91]), higher Birmingham vasculitis activity score (BVAS) (18.53±8.02 vs 13.68±5.98), lower eGFR (8.585.73, 22.07]mL/min·1.73 m²]vs 45.1511.54, 120.79]mL/min· 1.73 m²], lower blood sodium concentration (137.00134.00, 140.00]mmol/L vs 139.00136.00, 141.00] mmol/L), lower albumin level (28.41±5.24] g/L vs31.92±5.91]g/L), and higher serum D-dimer level (2.841.20, 6.28]mg/L vs 2.240.80, 3.69]mg/L) (all P<0.05). Multivariate Cox proportional regression analysis showed that age, eGFR, serum albumin level and BVAS were independent influence factors of death (hazard ratio=1.058, 0.987, 0.932, and 1.086, all P<0.05). Conclusion The clinical manifestations of AAV are mostly pulmonary and renal involvement. Age, eGFR, serum albumin level and BVAS are independent risk factors of death in AAV patients. Interstitial lung disease, high level of D-dimer and hyponatremia may be associated with prognosis of AAV.