特发性膜性肾病肾小球局灶节段性病变的临床及病理特点

Clinical and pathological features of idiopathic membranous nephropathy with focal segmental lesion

目的探讨特发性膜性肾病（idiopathic membranous nephropathy，IMN）中肾小球局灶节段性病变（focal segmental lesion，FSL）的临床病理特点及对患者预后的影响。方法回顾性分析298例IMN，将其分成不伴FSL组（FSL-组）、伴有早期局灶节段病变组（early focal segmental lesion。EFSL组）及伴有局灶节段肾小球硬化组（focal segmental glomerulosclerosis，FSGS组），比较分析3组患者临床病理及预后间的差异。结果病理方面，FSGS组患者伴有肾小管间质慢性病变的比例以及肾小球球性硬化的比例较高，病理分期较晚，与FSL『组和EFSL组比较，差异均有统计学意义（均P〈0．05）。临床方面，与FSL一组比较，EFSL组男性比例较高（P〈0．01），血浆白蛋白水平较低（P〈0．05）；而FSGS组病程更长，血压、血肌酐水平较高（均P〈0．05）。3组间EFSL组缓解率较低，生存分析显示，FSGS组病变预后较差（FSGS组比FSL^-组，P=0．005；FSGS组比EFSL组，P=0．008）。进行危险因素分析发现肾活检时的三酰甘油水平（OR=1．519，P=0．017）、肾小球球性硬化（OR=1．073，P=0．041）及FSGS病变（OR=5．960，P=0．009）是IMN肾脏死亡的独立危险因素。结论EFSL有着不同于FSGS的临床及病理表现，伴FSGS病变是IMN肾脏死亡的独立危险因素，同时EFSL组缓解率最低。

Objective To investigate the clinical- pathological features and prognosis of idiopathic membranous nephropathy （IMN） with focal, segmental lesion. Methods Two hundred and ninety- eight patients with biopsy- proven IMN in our hospital were retrospectively analyzed. The patients were divided into three groups： without focal segmental lesion group （FSL^-）, with focal segmental glomerulosclerosis group （FSGS） and with early focal segmental lesion group （EFSL）. The differences of clinical and pathological features and prognosis in the 3 groups were studied. Results There were later pathological stage, higher ratio of chronic renal tubulointerstitial damage and global glomerular sclerosis in FSGS group than those in the other two groups （all P 〈 0.05）. The male ratio in EFSL group was higher than that in FSL^- group （P 〈 0.01）, while the level of serum albumin was lower （P 〈 0.05）. Compared with FSL^- group, there was longer average course before renal biopsy, higher blood pressure and levels of Scr in FSGS group （all P 〈 0.05）. Furthermore, the remission rate in EFSL group was lower than that in FSGS group and FSL^- group. Survival analysis showed that FSGS group had worse prognosis （FSGS to FSL^-, P=0.005, FSGS to EFSL, P=0.008）. The analysis of risk factors suggested that triacylglycerol （OR=1.519, P=O.017）, glomerulosclerosis （OR=1.073, P=0.041） and FSGS lesion （OR= 5.960, P---O.O09） were independent risk factors for renal death. Conclusions There were some differences between EFSL and FSGS lesion, both in clinical manifestations and pathology. FSGS lesion was independent predictive factor for progression to renal death. And the lowest remission rate was in EFSL group.