抗中性粒细胞胞质抗体相关性小血管炎肾损害的病理分型及预后

目的分析抗中性粒细胞胞质抗体(antineutrophil cytoplasmic antibody,ANCA)相关性小血管炎(ANCA associated vasculitis,AAV)的病理分型特点及影响预后的相关因素。方法回顾性分析2004年1月至2017年2月于浙江大学医学院附属第一医院肾脏病中心住院治疗、具有肾活检结果的AAV患者。按照Berden分型分为4型,比较各型患者的临床、病理特点和预后。用Kaplan-Meier法绘制各型患者的生存曲线,并使用Log-rank检验比较生存曲线的差异。以进入维持性透析作为终点事件,使用Cox回归分析预后的影响因素。结果共175例AAV患者,其中局灶型59例(33.7%)、新月体型39例(22.3%)、硬化型32例(18.3%)、混合型45例(25.7%);新月体型组、硬化型组基础血肌酐水平较高,显著高于局灶型组、混合型组(均P<0.05);硬化型组袢坏死比例较低,显著低于局灶型组、新月体型组(均P<0.05)。中位随访11.8(0.5~86.7)个月,以死亡或维持性透析作为终点事件,末次随访时局灶型组、混合型组、新月体型组、硬化型组的无事件生存率分别为83.1%、77.8%、64.1%、50.0%(Log-rank χ^2=11.537,P=0.009)。Cox回归分析显示甲状旁腺素水平高(HR=1.013,95%CI1.007~1.019,P<0.001)、肾小球球性硬化≥50%(即硬化型,HR=10.532,95%CI2.903~38.203,P<0.001)是影响AAV患者进入维持性透析的独立危险因素,而基础估算肾小球滤过率高(HR=0.943,95%CI0.896~0.993,P=0.025)是保护性因素。结论AAV肾损害预后依据局灶型、混合型、新月体型、硬化型依次变差。基础估算肾小球滤过率低、甲状旁腺素水平高、肾小球球性硬化≥50%(即硬化型)是AAV患者进入维持性透析的独立危险因素。

Pathological classification and prognosis of renal damage caused by antineutrophil cytoplasmic antibody associated vasculitis

Objective To analyze the pathological characteristics and prognostic factors of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Methods A retrospective analysis of AAV patients with renal biopsy results admitted to Kidney Disease Center of the First Affiliated Hospital from January 2004 to February 2017 was performed. The patients were divided into 4 types according to Berden classification, and their clinical, pathological characteristics and prognosis were compared. The survival curves of each type of patients were plotted by Kaplan-Meier method, and the difference of survival curves was compared using Log-rank test. With entering the maintenance dialysis as the endpoint, Cox regression was used to analyze the prognostic factors. Results A total of 175 patients with AAV, including 59 cases (33.7%) of focal type, 39 cases (22.3%) of crescent type, 32 cases (18.3%) of sclerosis type, 45 cases (25.7%) of mixed type. The basal serum creatinine levels in crescent type group and sclerosis type group were significantly higher than those in the focal type group or mixed type group (all P＜0.05), and loop necrosis rate in sclerosis type group was significantly lower than chat in the focal type group or crescent type group (both P＜0.05). The median follow-up period was 11.8 (0.5-86.7) months. The event-free survival rates were 83.1%, 77.8%, 64.1% and 50.0% in the focal type, mixed type, crescent type and sclerotic type groups (Log-rank χ2=11.537, P=0.009). Cox regression analysis showed higher parathyroid hormone (HR=1.013, 95%CI 1.007-1.019, P＜0.001), glomerular sclerosis ≥50% (HR=10.532, 95%CI 2.903-38.203, P＜0.001) were independent risk factors for AAV patients entering maintenance dialysis, and higher estimated glomerular filtration rate (HR=0.943, 95%CI 0.896-0.993, P=0.025) was protective factor. Conclusion The prognosis of AAV renal damage is worsened according to focal, mixed, crescent and sclerosis types. Lower estimated glomerular filtration rate, higher parathyroid hormone and glomerular sclerosis ≥50% are independent risk factors for AAV patients entering maintenance dialysis.