狼疮性肾炎足细胞病伴系膜增生患者的临床病理特点  

Clinic-pathological research of lupus podocytopathy with mesangial proliferation

作　　者：王少凡[1] 陈樱花[1] 陈独群 刘正钊[1] 徐峰[1] 曾彩虹[1] 刘志红[1] 胡伟新[1] 

机构地区：[1]南京军区南京总医院肾脏科,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》2016年第6期507-512,共6页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：国家自然科学基金(81470042);国家科技支撑计划课题(2015BAI12B05);第一批国家临床重点专科项目(2014ZDZK001);江苏省创新能力建设专项(BM2015004);南京军区南京总医院院管课题(2015026)

摘　　要：目的：比较系膜增生狼疮性肾炎伴足细胞病与不伴足细胞病患者的临床病理特征及预后差别。方法：108例肾活检显示肾小球系膜增生性狼疮性肾炎患者[女99例,男9例,中位年龄30（21-39.3）岁],根据电镜检查足突融合和蛋白尿程度分为足细胞病组（足突融合范围〉50%且伴肾病综合征）和系膜增生组（足突融合≤50%）。系膜增生和肾小管间质病变程度进行半定量评分,系膜增生组根据有无蛋白尿分为血尿组（仅有镜下血尿,无蛋白尿）和蛋白尿组（蛋白尿伴或不伴血尿）两个亚组。比较不同组别临床病理特征、治疗反应及预后的差异。结果：足细胞病组和系膜增生组分别有28例和80例,两组足突融合比例分别为82.5%（70%,85%）和15%（10%,25%）。足细胞病组以肾病首发（60.7%vs 23.8%,P〈0.001）、肾病综合征（100%vs1.25%,P〈0.001）、急性肾损伤（25.0%vs 2.5%,P=0.001）比例及肾小管间质急性病变程度均显著高于系膜增生组,而关节炎、发热、低补体C4血症（28.6%vs 52.5%,P=0.029）、抗磷脂抗体阳性及血尿发生率（21.4%vs42.5%,P=0.047）显著低于系膜增生组。两组系膜区免疫球蛋白和补体沉积、系膜增生程度无明显差异,足细胞病组肾小管免疫球蛋白沉积率显著高于系膜增生组（35.7%vs 2.5%,P〈0.001）。激素或激素联合免疫抑制剂治疗两组缓解率无明显差异,足细胞病组的肾脏复发率明显高于系膜增生组（53.6%vs 29.0%,P=0.025）。在分别随访74个月（足细胞病组）和51个月（系膜增生组）期间,两组均无死亡或进展为ESRD。结论：有足细胞病的系膜增生性狼疮性肾炎的临床和形态学特征及转归与无足细胞病的系膜增生性狼疮性肾炎存在显著差异,两者应属于不同类型的狼疮性肾炎。Objective： To compare the differences of clinical and immunological features,renal morphology and outcomes of mesangial proliferative lupus nephritis（ LN） with or without podocytopathy. Methodology： one hundred and eight biopsies from 3 750 SLE patients who showed mesangial immune deposits with mesangial proliferation were included.They were divided into two groups according to the extent of foot process effacement（ FPE） and proteinuria： podocytopathy group（ FPE〉50%,with nephrotic syndrome,n = 28） and mesangial group（ PFE≤50%,n = 80）. Mesangial proliferation and tubular-interstitial lesion were semi-quantitatively scored. Results： The median FPE were 82. 5%（ 70%,85%） in podocytopathy group and 15%（ 10%,25%） in mesangial group（ P〈0. 001）. To compare with mesangial group,the incidence of renal onset（ 60. 7% vs 23. 8%）,nephrotic syndrome,acute kidney injury（ 25. 0% vs 2. 5%）,severe tubular interstitial lesion,and urinary NAG were much higher,while incidence of arthritis,fever,serum positive APL,low serum C4,hematuria was much lower in podocytopathy group. The rate of sever acute tubular-interstitial lesion and tubular immune deposition,renal relapse rate（ 53. 6% vs 29. 0%） were significant higher in podocytopathy group than that inmesangial group. No patients died or developed ESRD during follow-up in both two groups. Conclusion： Mesangial proliferative lupus nephritis with podocytopathy should be differentiated from mesangial proliferative lupus nephritis without podocytopathy,and the classification of lupus nephritis should be re-classified.

关 键 词：狼疮性肾炎 系膜增生 足细胞病 

分 类 号：R593.242[医药卫生—内科学]