狼疮性肾炎足细胞病患者的临床病理特征及预后

发布时间：2018-01-06 11:16

本文关键词：狼疮性肾炎足细胞病患者的临床病理特征及预后　出处：《肾脏病与透析肾移植杂志》2015年04期 　论文类型：期刊论文

【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%)(P0.01),肾小管间质急性病变程度也明显高于其他两组(P0.05)。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(P0.01)。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%)(P0.01)。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。
[Abstract]:Objective: to retrospectively analyze the clinical, pathological features and long-term prognosis of patients with lupus nephritis podocyte disease (lupus podocyte disease). Methods: systemic lupus erythematosus (SLE) with renal damage. Renal biopsy and electron microscopy were performed in 53 patients with lupus podocytosis (48 female, 5 male, median age 31 years). The median course of disease was 1.5 months. Results 53 cases of lupus podocytosis accounted for 1.41% of lupus nephritis in the same period, of which 50 cases showed nephrotic syndrome. There were 17 cases with acute renal injury, 9 cases with hematuria and 9 cases with hypertension. According to the light microscope changes of renal biopsy, MP was divided into Mesangial proliferative lesions. The ratio of median podocyte to foot process fusion was observed by electron microscope in the three groups: MCDN 13) and focal segmental glomerulosclerosis (FSGSN 9). The incidence of AKI in FSGS group (77.8%) was significantly higher than that in MP group (22.6%) and MCD group (23.1P 0.01). The degree of acute renal tubulointerstitial lesion was also significantly higher than that of the other two groups (P 0.05, compared with MCD group). The proportion of hypoC3 in MP group (83.9) and FSGS group (88.9) was significantly higher than that in control group (P 0.01). 69.8% achieved complete remission. The complete remission rate in FSGS group was significantly lower than that in MCD group (92. 3%) and in MP group 74.2% (P 0. 01). The median follow-up time was 60 months. 29 cases (54.7%) Nephropathy recurred in 13 cases and repeated renal biopsy was performed in 13 cases, of which 6 cases underwent pathological transformation. Conclusion: lupus podocyte disease is characterized by nephrotic syndrome or AKI, and histological changes may be MCDMP or FSGS. The incidence of AKI was high, the renal tubule injury was serious and the treatment remission rate was low in the patients with hormone or hormone combined immunosuppressive agents. The recurrence rate of the disease was high, and the pathological transformation was observed in some repeated renal biopsies. Long-term follow-up has a good prognosis.
【作者单位】：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;
【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)
【分类号】：R593.242
【正文快照】： 狼疮性肾炎(LN)的临床表现和病理改变存在一定联系,临床表现为肾病综合征(NS)的LN患者肾活检病理通常见免疫复合物沉积于肾小球毛细血管袢[1-2]。但近年研究发现,少数临床表现为NS的LN患者肾活检病理仅见免疫复合物沉积于肾小球系膜区,电镜下见肾小球足细胞足突广泛融合,与微

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