嗜酸性肉芽肿性血管炎肾损害的临床病理特征及预后  

Clinic-pathological features and outcomes of patients with eosinophilic granulomatosis with polyangiitis and renal involvement

作　　者：丁月梅[1] 陈樱花[2] 陈惠萍[2] 章海涛[2] 刘正钊[2] 刘志红[2] 胡伟新[2] 

机构地区：[1]南京大学医学院 [2]南京军区南京总医院国家肾脏疾病临床医学研究中心全军肾脏病研究所,南京210016

出　　处：《肾脏病与透析肾移植杂志》2014年第1期23-30,共8页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：国家科技支撑计划课题(2011BAI10B04)

摘　　要：目的：回顾性分析嗜酸性肉芽肿性血管炎（EGPA）肾损害的临床病理特征及转归. 方法：收集1998年1月至2012年11月在南京军区南京总医院肾脏科住院确诊的14例EGPA肾损害患者的临床病理资料.结果：14例EGPA患者中女性8例,男性6例,年龄20～70岁.所有患者血清ANCA阳性,以p-/MPO-ANCA为主（85.7％）.哮喘（8例）为最常见的首发症状,其他首发症状分别为咯血（3例）、肉眼血尿（2例）和关节炎（1例）.均有外周血嗜酸性粒细胞比例增高（10％ ～45％,平均18.14％±9.78％）和血清IgE水平升高（平均541±462 g/L）.除1例外,其余13例均存在蛋白尿和血尿,平均尿蛋白定量2.90±3.53 g/24h,其中5例（35％）表现为肾病综合征,2例为肉眼血尿.患者均存在肾功能不全,平均血清肌酐（SCr）498.6±288.6 μmol/L,其中7例（50％）需肾脏替代治疗.肾活检病理1例为急性间质性肾炎,其余13例为节段坏死性肾小球肾炎伴新月体形成,病理类型分别为混合型6例,新月体型4例,局灶型2例,硬化型1例.12例（85.7％）肾组织见嗜酸性粒细胞浸润,其中3例为嗜酸性粒细胞肉芽肿.患者均接受激素或联合免疫抑制药物治疗,7例需肾脏替代治疗的患者中5例肾功能改善并摆脱透析,1例进入维持性透析,1例死亡.12例患者随访6～83月（中位时间43.5月）,1例进展为终末期肾病,9例慢性肾功能不全,2例尿检和SCr正常;另有1例随访25月时出现肾外复发. 结论：本组EGPA患者肾脏损害重,肾脏病理改变多样,积极的免疫抑制治疗能有效改善肾功能,但总体肾脏预后较差;需要早期诊断和积极治疗以改善肾脏远期预后.Objective：To investigate the clinic-pathological features and outcomes of patients with eosinophilic granulomatosis with polyangiitis and renal involvement. Methodology： Fourteen patients diagnosed as eosinophilic granulomatosis with polyangiitis （EGPA） were investigated in this retrospective study. All of them had renal involvement and were performed renal biopsy. Their clinic-pathological features and outcomes were analyzed. Results：They were 8 female and 6 male with age from 20 to 70 years old, and the vasculitis duration from 1 ~ 600 months. They accounted for 6. 76% of all ANCA associated vasculitis in the same period in our hospital. All patients had positive serum ANCA, among them, 12（ 85.7% ） were p-/MPO-ANCA. The most common onset symptom was asthma （ 57.1%, n = 8）, the others were hemoptysis （21.4%,n = 3 ）, gross hematuria （ 14. 3%, n = 2） and arthritis （ 7. 14%, n = 1 ）. All patients had increased peripheral eosinophil ratio from 10% to 45% with the average of 18.1±9. 8%） accompanied with elevated serum IgE levels of 541±462 g/L. Except one, the other 13 patients had both hematuria and proteinuria. The mean urine protein was 2. 90± 3.53 g/24h ,5 （35%） had nephrotic syndrome, and 2 had gross haematuria. All patients presented renal dysfunction with a high level of Scr 498.6±288.6 μmol/L, and 7 patients required initial renal replacement therapy. Renal biopsy showedpaci-immune segmental necrotizing glomerulonephritis with crescent formation in 13 patients （6 mixed class, 4 crescentic class ,2 focal class, and one sclerotic class） , and acute interstitial nephritis in one. 12 patients （85.7%） had eosinophil infiltration in renal interstitium, and 3 showed eosinophil granuloma. All patients received corticosteroid with immunosuppressant therapy. 5 patients of those who required initial renal replacement therapy got rid of dialysis, one entered maintenance hemodialysis, and one died. 12 patients were followed up for 6 to 83 months （median 43.5 ） , 2 patie

关 键 词：嗜酸性肉芽肿性血管炎 肾脏损害 抗中性粒细胞胞质抗体抗 中性粒细胞胞质抗体相关血管炎 

分 类 号：R692[医药卫生—泌尿科学]