成人特发性局灶节段性肾小球硬化病248例临床病理分析

Clinicopathologic study of 248 patients with FSGS

目的 重新评价肾活检标本中特发性局灶节段性肾小球硬化病 (FSGS)的发生率、主要临床及病理特点。方法 收集 1994年 7月～ 2 0 0 0年 7月间 2 48例特发性FSGS的患者 ,观察肾组织的光镜、荧光及电镜的特点。根据肾小球病变将特发性FSGS分为 3种亚型。结果 2 48例成人特发性局灶节段性肾小球硬化占同期肾活检的 12 .4% ,占成年人原发性肾小球疾病的 19.0 % ,以 18～ 30岁为发病高峰年龄 (约占 44 .4% )。临床表现为不同程度的蛋白尿 ,以肾病综合征为表现者 143例 (占 5 7.7% )。FSGS的 3种病理亚型中 ,经典型FSGS仍占绝大多数 (91.5 % ) ,尖端型为 4.4% ,塌陷型为 4.1%。肾活检时 ,约 1/ 3以上为肾功能不全 (约占 35 .1% ) ,13例尿毒症中 76 .9%病理亚型为塌陷型。结论 局灶节段性肾小球硬化病约占原发性肾小球病 1/ 5 ,FSGS患者肾活检时 ,除表现为蛋白尿、肾病综合征外 ,肾功能不全也较常见。塌陷型FSGS常表现为进行性的不可逆的肾功能衰竭 ,其预后差。

Objective To analyze the incidence,clinical presentations and pathological changes of patients with primary focal segmental glomerulosclerosis (FSGS). Methods 248 patients with biopsy proven primary FSGS were enrolled. They were hospitalized during the period of 1994～2000. All tissues were evaluated by LM, IF and EM. They were divided into three subgroups based on glomerular changes. Results The incidence of adult primary FSGS was 12.4% in all the biopsied patients in our hospital. About 19.0% of primary glomerular nephritis were FSGS, in which about 44.4% of patients aged 18 30. The patients presented with nephrotic syndrome (143/248) or varying degrees of proteinuria (105/248). More than 1/3 of patients had renal failure, 14.9%(13/248) patients had uremia. Among the subgroups of pathologic changes, 91.5% of patients had classical FSGS, 4.4% glomerular tip lesion and 4.1% collapsing glomerulo nephropathy. Conclusion FSGS occupies a high percentage in primary glomerular nephritis (1/5). Renal insufficiency is common, whereas peogressive irreversible renal failure is frequently seen in collapsing glomerular nephropathy with poor prognosis. (Shanghai Med J, 2001,24∶ 89 91 )