263例成人原发性局灶节段肾小球硬化性肾小球肾炎临床病理分析

Clinicopathological analysis of adult primary focal segmental glomerulosclerosis in 263 cases

目的了解我国南方地区原发性局灶节段肾小球硬化性（FSGS）肾小球肾炎的发病情况及探讨FSGS临床与病理特征的关系。方法收集1988年7月至2005年7月经我院肾活检确诊为原发性FSGS的263例成人患者的临床及肾脏病理资料并进行分析。结果（1）原发性FSGS占同期成人原发性肾小球疾病7．02％，占成人原发性肾病综合征（NS）6．33％，其构成比近年有逐渐升高的趋势。青壮年为成人FSGS的主要患病人群，临床以不同程度的蛋白尿为特征，以NS为主要临床表现的有133例，占50．6％。（2）主要病理特征：48．4％患者肾小球硬化比率≥25％，肾小球硬化并伴有肾小管间质病变者占88．6％，其中伴严重肾小管间质病变占25．2％。（3）肾小球硬化程度及小管间质病变程度与Cer呈负相关（P〈0．01），并与Ser水平呈正相关伊〈0．05）。肾小球硬化程度与小管间质病变程度呈正相关（P〈0．01），与血浆白蛋白水平呈正相关（P〈0．05）。肾小管间质病变是FSGS患者出现肾功能不全的重要影响因素。结论原发性FSGS是成人肾病综合征的主要病理类型之一，就诊时肾小球硬化及肾小管间质纤维化已损害明显，并与肾功能损害密切相关。早期诊断和及时治疗，从而延缓FSGS的进展仍是广大肾脏病工作者探索的重要课题。

Objective To investigate the relative frequency and to elucidate the relationship between clinical and pathological features of primary focal segmental glomerulosclerosis （FSGS） in the south of China. Methods The data of 263 adult patients diagnosed as primary FSGS from 1988 to 2005, excluding secondary FSGS by renal biopsies, were reviewed and analyzed retrospectively. Results （1） The incidence of primary FSGS accounted for 7.02% of adult primary glomerular diseases confirmed by renal biopsies, and 6.33% of adult primary nephrotic syndrome （NS） within corresponding period. The predominant patients were young people. The patients presented various degrees of proteinuria, including nephrotic syndrome in 133 cases （about 50.6%）. （2） The main pathological characteristics of the 263 FSGS cases were as follows： 48.4% of the patients had a glomerulosclerosis ratio more than 25%, 88.5% of glomerulosclerosis usually accompanied tubulointerstitial lesion, including severe lesions of graded 2 or 3 （about 25.2%）. （3） Both glomerulosclerosis and tubulointerstitial lesion were correlated with renal insufficiency. The degree of glomerulosclerosis and tubulointerstitial lesion was negatively correlated with creatinine clearance, but positively with serum creatinine. A positive correlation was also found between glomerulosclerosis and tubulointerstitial lesion （P〈0.01）. Tubulointerstitial lesion was a significant influencing factor of renal insufficiency. Conclusions Primary FSGS is one of the major pathological types of adult NS. Glomerulosclerosis and tubulointerstitial lesion, which contribute to renal insufficiency, progress severely when the patients are diagnosed as primary FSGS. Therefore early diagnosis and therapy is recommended to attenuate the disease and is one of the important topics for the nephrologists.