Ren Hong, Shen Pingyan, Li Xiao, Pan Xiaoxia, Zhang Qianying, Feng Xiaobei, Zhang Wen, Chen Nan
Department of Nephrology, Ruijin Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, PR China.
Contrib Nephrol. 2013;181:109-18. doi: 10.1159/000348468. Epub 2013 May 8.
This study aimed to analyze the treatment, clinical outcomes, and risk factors that affect the prognosis of patients with primary focal segmental glomerulosclerosis (FSGS) and to provide theoretical evidence for various treatment options in these patients. The study reviewed the clinical, laboratory, and pathological data of 168 patients with primary FSGS treated at Ruijin Hospital between January 2002 and October 2011. Of these patients, 108 were male (64.3%) and 60 were female (35.7%). The median age of disease onset was 38 years (range 12-78 years). The median case history was 10 months (range 4 days to 30 years). The mean proteinuria level was 2.3 ± 0.6 g/day. 75 (44.6%) patients had nephrotic syndrome. The mean serum creatinine was 108.1 ± 8.9 μmol/l. Over a follow-up period of 25.3 ± 11.4 months, end-stage renal failure occurred in 4 patients, and all 4 survived. In the group treated with angiotensin-converting enzyme inhibitor/angiotensin receptor blocker, the following factors were identified as risk factors for experiencing a 50% increase in serum creatinine over the baseline: a baseline eGFR <60 ml/min, proteinuria >1 g/day during the follow-up period, glomerular sclerosis >grade 1, and tubulointerstitial lesions >stage 1. In the group treated with steroids, patients who achieved a stable remission had better preserved renal function and milder glomerular sclerosis than steroid-dependent patients (p < 0.01). Steroid-resistant FSGS patients had a worse histological severity of glomerular sclerosis than steroid-dependent patients (p < 0.01). The prognosis of FSGS was correlated with the amount of proteinuria, the level of serum creatinine, and the severity of glomerular sclerosis and tubulointerstitial lesions. Steroids may be more effective in those who have better preserved renal function and milder glomerular sclerosis.
本研究旨在分析原发性局灶节段性肾小球硬化（FSGS）患者的治疗方法、临床结局及影响预后的危险因素，为这些患者的各种治疗选择提供理论依据。该研究回顾了2002年1月至2011年10月在瑞金医院接受治疗的168例原发性FSGS患者的临床、实验室及病理数据。其中，男性108例（64.3%），女性60例（35.7%）。疾病发病的中位年龄为38岁（范围12 - 78岁）。中位病程为10个月（范围4天至30年）。平均蛋白尿水平为2.3±0.6g/天。75例（44.6%）患者患有肾病综合征。平均血清肌酐为108.1±8.9μmol/l。在25.3±11.4个月的随访期内，4例患者发生终末期肾衰竭，且这4例患者均存活。在接受血管紧张素转换酶抑制剂/血管紧张素受体阻滞剂治疗的组中，以下因素被确定为血清肌酐较基线水平升高50%的危险因素：基线估算肾小球滤过率（eGFR）<60ml/min、随访期间蛋白尿>1g/天、肾小球硬化>1级以及肾小管间质病变>1期。在接受类固醇治疗的组中，达到稳定缓解的患者比依赖类固醇的患者肾功能保留更好，肾小球硬化更轻（p<0.01）。激素抵抗型FSGS患者的肾小球硬化组织学严重程度比依赖类固醇的患者更差（p<0.01）。FSGS的预后与蛋白尿的量、血清肌酐水平、肾小球硬化及肾小管间质病变的严重程度相关。对于肾功能保留更好且肾小球硬化更轻的患者，类固醇可能更有效。
