成人特发性局灶节段性肾小球硬化症的临床及病理分析

An analysis of the clinical and pathological manifestations of adult idiopathic focal segmental glomerulosclerosis

目的 :了解特发性局灶节段性肾小球硬化症 (focalsegmentalglomerulosclerosis ,FSGS)在肾脏疾病中的构成变化及其临床病理诊断要点和意义。方法 :对 1 990年 1月至 2 0 0 1年 1 2月在我院肾科住院行肾活检确诊为特发性FSGS的 6 5例患者的临床资料及病理改变进行总结分析。结果 :(1 )特发性FSGS占肾活检患者的 2 .2 % ,占原发性肾小球疾病的 3.2 % ,占肾病水平蛋白尿患者的 5 .8% ,1 2年中发病无上升趋势。 (2 )其病变类型为单纯门部病变占 1 2 .3% ,单纯周缘部病变占 2 3.1 % ,两者的混合型占 6 0 .0 % ,尖端型占 1 .5 % ,塌陷性占 3.1 %。同时伴随的病变有 :足细胞增生、肥大和空泡变性 (5 8.5 % ) ,球囊粘连 (90 .8% ) ,肾小管间质病变 (92 .3% )以及节段性内皮和系膜细胞增生 (2 0 .0 % )。由于病变分布呈局灶节段的特点 ,且病变多样、重叠出现 ,在病理诊断中应警惕误、漏诊的可能。 (3)伴节段性内皮和系膜细胞增生的患者常表现为肾病水平蛋白尿 (92 .3% )。结论 :在肾脏病理工作中 ,提高警惕性和检查方法对于正确诊断特发性FSGS具有重要意义。特发性FSGS不是构成本组肾活检患者的常见病理类型。

Objective:To investigate the relative frequency of idiopathic focal segmental glomerulosclerosis (FSGS) in renal biopsy proven diseases, and its criteria and significance of clinicopathological diagnosis. Methods: We reviewed all the inpatients that were diagnosed by renal biopsies from 1990 to 2001, of whom 65 were identified as idiopathic FSGS. Their histological characteristics were analyzed together with their clinical findings. Results: (1) The incidence of idiopathic FSGS accounted for 2.2% of all the renal biopsies, 3.2% of primary glomerular diseases, and 5.8% of patients with massive proteinuria. (2) The subtypes of idiopathic FSGS were hilar lesion (12.3%), peripheral lesion (23.1%), mixture lesion (60.0%), collapsing lesion (3.1%), and tip lesion (1.5%), which were frequently accompanied by other morphologic variants, such as synechia of Bowman's capsule, podocyte hyperplasia and hypertrophy, segmental endothelial and mesangial proliferation, and interstitial fibrosis, etc. As the sclerotic lesions distributed segmentally and overlapped by other variants, it was difficult to get the correct diagnosis. (3) Most of the patients with segmental endothelial and mesangial proliferation had massive proteinuria. Conclusion: Idiopathic FSGS was not a common glomerular disease in our study. Podocyte lesion, segmental endothelial and mesangial proliferation may play an important role in the formation of segmental sclerosis in idiopathic FSGS.