微小病变样IgA肾病与微小病变肾病患者临床病理及预后的比较  

The comparison of clinico-pathological features and outcome between Ig A nephropathy with minimal change disease and minimal change disease

作　　者：李小伟[1,2] 成水芹[2] 梁少珊[2] 乐伟波[2] 曾彩虹[2] 王金泉[2] 刘志红[1,2] 

机构地区：[1]东南大学临床医学院,南京210009 [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

出　　处：《肾脏病与透析肾移植杂志》2015年第5期401-406,共6页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：国家科技支撑计划课题(2013BAI09B04;2015BAI12B05);江苏省临床医学中心项目(BL2012007)

摘　　要：目的：比较微小病变样Ig A肾病（MCD-Ig AN）与微小病变肾病（MCD）患者的临床病理特点、治疗反应及预后。方法：回顾性分析Ig AN随访登记数据库中80例随访时间≥3年的MCD-Ig AN患者和77例随访时间≥3年的MCD患者的资料。结果：（1）两组患者临床表现相似,以青年男性多见,多数患者以肾病综合征为主要表现,部分患者伴不同程度的镜下血尿,但与MCD患者相比,MCD-Ig AN患者肾活检时基线血清白蛋白、估算的肾小球滤过率（e GFR）更低（P均〈0.01）,而尿N-乙酰-β-D氨基葡萄糖苷酶、尿视黄醇结合蛋白水平更高（P均〈0.01）。（2）肾脏病理：MCD-Ig AN与MCD患者相比,肾小管间质急性和慢性病变均较重（P〈0.05）。（3）激素疗效：80例MCD-Ig AN与77例MCD患者激素治疗的疗效比较,8周完全缓解、部分缓解及未缓解均无显著差异（88.8%vs 90.9%,10.0%vs 5.2%,1.3%vs 3.9%,P均〉0.05）。达到缓解的中位时间分别是4周（1～24周）和4周（1～28周）。两组患者激素疗效无显著差异。（4）复发和预后：至3年随访期末,MCD-Ig AN患者复发率显著低于MCD患者（45.0%vs 63.6%,P〈0.05）。无一例患者进入ESRD,仅2例MCD-Ig AN患者e GFR较基线下降〉50%。结论：MCD-Ig AN与MCD患者在临床表现、病理特点、激素治疗的疗效及预后上均无明显差异。值得注意的是,MCD-Ig AN患者伴较显著的肾小管间质病变和损伤。Objective： To investigate the clinic-pathological characteristics,treatment response and prognosis of Ig A nephropathy patients with minimal change lesion（ MCD-Ig AN）. Methodology： Eighty patients with biopsy-proven MCD-Ig AN and followed for ≥ 3 years from the Jinling Hospital Ig A nephropathy Registry were enrolled into this retrospective study,and 77 patients with MCD were regarded as disease control. The clinic-pathological characteristics,treatment response and prognosis were compared with between two groups. Results： The clinical presentations were similar between the two groups,both with young male predominance,the majority of these patients presented with nephrotic syndrome,only few MCD-Ig AN had varying degrees of macroscopic hematuria. Compared with the MCD group,patients with MCD-Ig AN had lower levels of baseline serum albumin（ P〈0. 01） and e GFR（ P〈0. 01）,higher levels of urinary nacetyglucosaminidase and retino-bingding protein（ P 〈0. 01）. The pathological features showed more severe acute and chronic tubulointerstitial injury in patients with MCD-Ig ANrenal pathology（ P〈 0. 01,P 〈0. 05,respectively）,while the glomerular lesion was similar between two groups. After 8 weeks of corticosteroid therapy,no significant differences were observed in the rate of complete remission,partial remission and no remission between MCD-Ig AN and MCD（ 88. 8% vs90. 9%,10. 0% vs 5. 2%,1. 3% vs 3. 9%,P〉 0. 05）. The median time to achieve remission was 4 weeks（ range 1-24weeks） and 4 weeks（ range 1-28 weeks） respectively. During the three-year follow-up period,there was a significantly lower rate of relapse in MCD-Ig AN patients than that in MCD patients（ 45. 0% vs 63. 6%,P〈0. 05）. No patients between two groups entered ESRD,only 2 patients（ 2. 5%） with MCD-Ig AN had greater than 50% reduction of e GFR.Conclusion： The clinical manifestations,pathological features,response to corticosteroid therapy and renal outcome were similar between MCD-Ig AN and MCD.

关 键 词：IGA肾病 微小病变肾病 临床病理 治疗 预后 

分 类 号：R692[医药卫生—泌尿科学]