不典型膜性肾病与特发性膜性肾病的临床特征与病理改变比较

摘要

Objective To compare the clinicopathological characteristics of atypical membranous nephropathy (AMN) and idiopathic membranous nephropathy (IMN).Methods Seventy nine patients with AMN and 134 patients with IMN were enrolled in the study.The general information,clinical manifestations,laboratory findings,renal pathology were compared between two groups.Results Compared with IMN group,the age of patients with AMN was younger and the incidence of nephrotic syndrome was higher (all P＜0.05).There were no significant differences in gender,body weight,BMI and incidence of hypertension,acute renal injury,thrombosis and embolism between two groups (all P ＞0.05).The urine RBC count,24h urinary protein quantification,urine NAG and D-dimer were higher and serum albumin was lower in AMN group than those in IMN group (all P＜0.05).There was no significant difference in levels of triglyceride,cholesterol,serum uric acid,serum creatinine,eGFR,IgG,IgA,IgM,C3,C4,CRP and positive rates of HBsAg,HBeAg,and antinuclear antibody (all P ＞0.05).There was no significant difference in the proportion of glomerular sclerosis between the two groups (P ＞0.05).The acute and chronic tubulointerstitial injury was more sever pathologically in patients with AMN (P＜0.05).The positive expression rates of IgA,IgM,C4,C1q,IgG1,IgG2 and IgG3 in renal tissue were significantly higher and the positive expression rates of IgG4 and PLA2R were significantly lower in AMN than those in IMN (all P＜0.05).Compared with the IMN group,the electron dense granules were more frequently observed in the mesangial area and glomerular basement membrane in AMN group(P＜0.05).Conclusion The clinicopatholo-gical characteristics of AMN are different from those of IMN,suggesting that the pathogenesis and disease prognosis may be different between AMN and IMN.%目的 比较不典型膜性肾病(AMN)与特发性膜性肾病(IMN)的临床特征与病理改变,以助于鉴别诊断.方法 选取临床资料完整、初发初治AMN患者79例(AMN组),IMN患者134例(IMN组).比较两组患者一般资料及临床表现、实验室检查结果、肾脏病理学检查结果.结果 与IMN组比较,AMN组患者年龄较小、肾病综合征发生率较高(均P＜0.05).两组患者性别、体重、BMI及高血压、急性肾损伤、血栓和栓塞发生率比较差异均无统计学意义(均P＞0.05).与IMN组比较,AMN组患者尿RBC、24h尿蛋白定量、尿NAG、血清D-二聚体较高,血清白蛋白较低(均P＜0.05).两组患者血清TC、TG、血尿酸、Cr、ANA阳性率、HBsAg阳性率、HBeAg阳性率、IgG、IgA、IgM、C3、C4、CRP、eGFR比较差异均无统计学意义(均P＞0.05).两组患者肾小球硬化比例比较差异无统计学意义(P＞0.05),但AMN组患者肾小管间质急性、慢性病变均较重(均P＜0.05).AMN组患者肾组织IgA、IgM、C4、C1q、IgG1、IgG2、IgG3阳性率均高于IMN组(均P＜0.05),IgG4、PLA2R阳性率均低于IMN组(均P＜0.05).AMN患者在系膜区、基底膜部位比IMN患者更易出现电子致密物沉积(P＜0.05).结论 AMN患者在临床特征和病理改变上均与IMN存在差异,提示AMN的发病机制及疾病预后可能不同于IMN.