Meyrier A, Simon P, Perret G, Condamin-Meyrier M C
Br Med J (Clin Res Ed). 1986 Mar 22;292(6523):789-92. doi: 10.1136/bmj.292.6523.789.
Nephrotic syndrome in minimal change lipoid nephrosis and focal segmental glomerulosclerosis may be due to alteration of glomerular anionic sites by a lymphokine. Six adults with nephrotic syndrome who were resistant to treatment with corticosteroids and immunosuppressants were treated with cyclosporin A. In three patients with minimal change lipoid nephrosis who had been nephrotic for 3.5 to 23 years proteinuria resolved within 12 to 42 days. Subsequently, these patients became dependent on cyclosporin A. In three patients with focal segmental glomerulosclerosis who had been nephrotic for four to six years mean (SD) 24 hour urinary protein decreased from 14.7 (8.4) g to 3.6 (0.6) g within 20 to 25 days, serum albumin concentration rose, and oedema subsided. One patient died of myocardial infarction when still in partial remission after 11 weeks' treatment. Two patients remained proteinuric despite continuing treatment with cyclosporin A, but control of sodium balance was easy and serum albumin concentrations remained higher than without cyclosporin A. In all patients renal function improved during treatment. These preliminary results show that cyclosporin A may be effective in the treatment of patients with nephrotic syndrome that resists every other form of treatment and especially in the treatment of those with minimal change lipoid nephrosis. The results are in keeping with a T lymphocyte mediated mechanism of minimal change lipoid nephrosis and focal segmental glomerulosclerosis, but they also suggest that minimal change lipoid nephrosis and focal segmental glomerulosclerosis are separate entities.
微小病变性类脂性肾病和局灶节段性肾小球硬化症中的肾病综合征可能是由于一种淋巴因子改变了肾小球阴离子位点所致。6例对皮质类固醇和免疫抑制剂治疗无效的肾病综合征成人患者接受了环孢素A治疗。在3例微小病变性类脂性肾病患者中,他们的肾病已持续3.5至23年,蛋白尿在12至42天内消失。随后,这些患者对环孢素A产生了依赖。在3例局灶节段性肾小球硬化症患者中,他们的肾病已持续4至6年,平均(标准差)24小时尿蛋白在20至25天内从14.7(8.4)g降至3.6(0.6)g,血清白蛋白浓度升高,水肿消退。1例患者在治疗11周后仍处于部分缓解状态时死于心肌梗死。2例患者尽管继续使用环孢素A治疗仍有蛋白尿,但钠平衡易于控制,血清白蛋白浓度仍高于未使用环孢素A时。所有患者在治疗期间肾功能均有改善。这些初步结果表明,环孢素A可能对抵抗其他各种治疗形式的肾病综合征患者有效,尤其是对微小病变性类脂性肾病患者。这些结果与微小病变性类脂性肾病和局灶节段性肾小球硬化症的T淋巴细胞介导机制相符,但也表明微小病变性类脂性肾病和局灶节段性肾小球硬化症是不同的实体。