目的 研究青年特发性膜性肾病(IMN)的临床及病理特点.方法 纳入首都医科大学附属北京安贞医院2012年1月至2016年9月经肾穿刺病理检查诊断为IMN的病例,比较≤35岁(A组)和＞35岁(B组)IMN患者的临床、病理表现及治疗效果.结果 (1) IMN患者537例,占同期肾活检病例的25.8％及原发性肾小球疾病的32.5％,其中A组占IMN的20.9％.(2)与B组比较,A组患者病程较短,呈肾病综合征及肾功能不全的比例较低,而呈无症状性蛋白尿的比例较高(P＜0.05);此外,A组患者伴发肥胖、糖尿病、高血压的比例较低(P＜0.05或P＜0.01).(3)肾小球毛细血管壁上IgG4亚类及PLA2R沉积的阳性率分别为95.0％及91.8％,A、B两组阳性率比较差异无统计学意义(P＞0.05).11.4％肾小球无PLA2R沉积的患者中,肾小球THSD7A沉积阳性,A、B两组比较差异也无统计学意义(P＞0.05).(4)两组患者的近期治疗疗效相似(P＞0.05).结论 青年IMN患者的肾小球内致病抗体与抗原沉积及治疗疗效与中老年患者相似,提示二者不存在实质性区别.两组患者的某些临床表现和治疗方案的不同可能为年龄因素及并发症所致.%Objective To investigate the clinicopathological features of idiopathic membranous nephropathy (IMN) in young adult Chinese patients.Methods The clinicopathological manifestations and therapeutic effects were retrospectively analyzed in the patients with IMN diagnosed by renal biopsy from Jan.2012 to Sep.2016,and compared between group A (≤ 35 years) and group B (＞35years).Results There were 537 patients with IMN in this study,accounting for 25.8％ of the cases of renal biopsy and 32.5％ of the cases of primary glomerular disease.Among them,there were 20.9％ patients in group A.The disease course of group A was shorter than that of group B (P＜0.05).The proportions of nephrotic syndrome and renal insufficiency in group A were lower than those in group B,while the proportion of asymptomatic proteinuria in group A was higher than that in group B (P＜0.05).The patients with obesity,diabetes or/and hypertension in group B were significantly more than those in group A (P＜0.05 or P＜0.01).95.0％ and 91.8％ patients had the deposition of predominant IgG4 subclass and PLA2R on glomerular capillary wall,respectively.Among the patients without glomerular PLA2R deposition,11.4％ had THSD7A deposition on glomerular capillary wall.However,the differences of the positive rates of predominant IgG4,PLA2R and THSD7A deposition in glomeruli between A and B groups were not significant (P＞0.05).There was no statistical difference in treatment effects between the two groups (P＞0.05).Conclusion The deposition of pathogenic antibodies and antigens in glomeruli and the treatment effects were similar between the IMN patients in group A and group B,which suggests there are no substantial differences between the patients of these two groups.Some differences in clinical manifestations and treatment regimens between these two groups may caused by age factor and complications.
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