狼疮性肾炎足细胞病患者的临床病理特征及预后  

Clinic-pathological characteristics and outcomes of patients with lupus podocytopathies

作　　者：王少凡[1] 陈浩[1] 陈樱花[1] 刘正钊[1] 徐峰[1] 刘志红[1] 胡伟新[1] 

机构地区：[1]南京大学医学院附属金陵医院(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》2015年第4期301-306,共6页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：国家科技支撑计划课题(2011BAI10B04,2013BAI09B04,2015BAI12B05);江苏省自然科学基金(BK20131326)

摘　　要：目的:回顾性分析狼疮性肾炎足细胞病(狼疮足细胞病)患者的临床、病理特征及远期预后。方法:系统性红斑狼疮(SLE)伴肾损害,经肾活检组织学和电镜检查符合狼疮足细胞病的患者53例(女48例,男5例,中位年龄31岁,中位病程1.5月)。回顾性分析其临床、病理特征及远期预后。结果:53例狼疮足细胞病占同期狼疮性肾炎的1.41%,其中50例临床表现肾病综合征,17例(32.1%)伴急性肾损伤(AKI),合并镜下血尿和高血压各9例(17.0%)。根据肾活检光镜改变分为系膜增生性病变(MP,n=31)、轻微病变(MCD,n=13)和局灶节段性肾小球硬化(FSGS,n=9)三组。电镜观察中位足细胞足突融合比例85%,三组间无明显差异。FSGS组AKI发生率(77.8%)显著高于MP组(22.6%)和MCD组(23.1%)(P<0.01),肾小管间质急性病变程度也明显高于其他两组(P<0.05)。与MCD组(23.1%)相比,MP组(83.9%)和FSGS组(88.9%)低C3血症的比例显著升高(P<0.01)。经激素或激素联合免疫抑制剂诱导治疗后,69.8%获得完全缓解,FSGS组完全缓解率(22.2%)显著低于MCD组(92.3%)和MP组(74.2%)(P<0.01)。中位随访时间60月,29例(54.7%)肾病复发,13例复发后行重复肾活检,其中6例发生病理转型,无终末期肾病或死亡病例。结论:狼疮足细胞病以肾病综合征或伴AKI为主要特征,组织学改变可为MCD、MP或FSGS,激素或激素联合免疫抑制剂治疗敏感,FSGS者AKI发生率高、肾小管损伤重且治疗缓解率低。该病复发率高,部分重复肾活检可见病理转型,长期随访预后良好。Objective： To investigate the clinical,histological characteristics and outcomes of SLE patients with lupus podocytopathy. Methodology： Fifty three SLE cases with renal involvement and biopsy-proven lupus podocytopathy were collected in this retrospective study. Their clinical,immunological pathological features and prognosis were analyzed.Results： The patients with lupus podocytopathy accounted for 1. 41% of lupus nephritis patients in the same period. 50（ 94. 3%） cases presented as nephrotic syndrome in which 17（ 14%） cases were complicated by acute kidney injury（ AKI）. The pathological manifestations under LM showed mesangial proliferation（ MP） in 31（ 58. 5%） cases,minimal change disease（ MCD） in 13（ 24. 5%） and focal segmental glomerulonephritis（ FSGS） in 9（ 16. 9%） cases. The incidence of AKI was much higher in FSGS group than that in MCD and MP group（ P〈0. 01）. Compared with MCD group,the ratio of low serum C3 level was significantly higher in MP and FSGS group（ P〈0. 01）. 37（ 69. 8%） cases received complete remission（ CR） undering induction treatment of glucocorticoid or steroids in combination with immune depressant.The CR rate in FSGS group was much lower than that in the other two groups（ P〈0. 01）. 29（ 54. 7%） cases relapsed during follow-up. 13 patients had repeat renal biopsy while histological transition was found in 6. No patient progressed to end stage renal failure or death. Conclusion： Lupus podocytopathy was characterised by nephrotic syndrome,often complicated by AKI,and had different histological changes including MCD,MP and FSGS. It had high remission rate and benign prognosis,but easy to relapse. Patients with FSGS showed more serious renal lesion and worse therapeutic response.

关 键 词：狼疮性肾炎 足细胞病 病理分型 预后 

分 类 号：R593.242[医药卫生—内科学]