Beaufils H, Alphonse J C, Guedon J, Legrain M
Nephron. 1978;21(2):75-85. doi: 10.1159/000181374.
70 cases of focal glomerulosclerosis (FGS) followed for periods ranging from 1 to 24 years are reported. 39 patients had a nephrotic syndrome. 31 patients had asymptomatic proteinuria. 97 renal biopsies were performed. At least one biopsy specimen from every patient showed focal and segmental involvement of glomeruli, but no lesions were observed on the first biopsy in 6 patients. 26 of the 35 patients with a nephrotic syndrome were treated with steroids alone and/or chemotherapy and/or indomethacin. Resistance to treatment was encountered in 21 patients. Complete remission was observed in 6 cases, despite persistence or accentuation of histological lesions on serial biopsies in 3 cases. Actuarial renal survival rate at 10 years was 45% in the group with a nephrotic syndrome versus 91% in patients with proteinuria. FGS with a persistent nephrotic syndrome represents the 'malignant' form of the disease. Recurrence of the disease was observed after transplantation in 2 cases and was absent in 1.
报告了70例局灶节段性肾小球硬化（FGS）患者，随访时间为1至24年。39例患者患有肾病综合征。31例患者有无症状蛋白尿。共进行了97次肾活检。每位患者至少有一份活检标本显示肾小球局灶性和节段性受累，但6例患者首次活检未观察到病变。35例肾病综合征患者中的26例接受了单独使用类固醇和/或化疗和/或吲哚美辛治疗。21例患者出现治疗抵抗。6例患者完全缓解，尽管3例患者在系列活检中组织学病变持续存在或加重。肾病综合征组10年实际肾脏生存率为45%，蛋白尿患者为91%。持续性肾病综合征的FGS代表该疾病的“恶性”形式。2例患者移植后疾病复发，1例未复发。
