Cattran D C, Alexopoulos E, Heering P, Hoyer P F, Johnston A, Meyrier A, Ponticelli C, Saito T, Choukroun G, Nachman P, Praga M, Yoshikawa N
Toronto General Research Institute, University Health Network, Toronto, Ontario, Canada.
Kidney Int. 2007 Dec;72(12):1429-47. doi: 10.1038/sj.ki.5002553. Epub 2007 Sep 26.
Management of idiopathic glomerular disease associated with nephrotic syndrome (INS) remains controversial and one of the most complex areas relates to utilization of the drug cyclosporin. This is despite its demonstrated effectiveness in several histologic types of the INS in randomized controlled trials. Cyclosporin is effective in inducing remission of proteinuria in approximately 80% of steroid-sensitive cases of minimal change disease (MCD). Cyclosporin is also effective in both the induction of remission and long-term preservation of renal function in steroid-dependent/-resistant MCD and steroid-resistant focal segmental glomerulosclerosis (FSGS). The overall response rate in FSGS is lower than in MCD, and long-term therapy (>12 months) may be required to both achieve remission and sustain it. Cyclosporin therapy is also of benefit in reducing proteinuria in 70-80% of patients with steroid-resistant membranous nephropathy (MGN). In MGN, the maximum benefit is often delayed compared to MCD (>12 weeks). Cyclosporin is generally well tolerated and safe. The major concern remains the nephrotoxicity, but with careful monitoring of the patient's renal function; minimizing the maintenance dose and utilizing repeat renal biopsy in those receiving long-term therapy, this risk can be minimized. The algorithms have been developed derived from the best evidence in the literature in each of the histologic types to help provide a guide to the integration of cyclosporin into the management of INS for the practicing nephrologist.
与肾病综合征（INS）相关的特发性肾小球疾病的管理仍存在争议，最复杂的领域之一涉及环孢素的使用。尽管在随机对照试验中已证明其对几种组织学类型的INS有效，但情况依然如此。环孢素在约80%的微小病变病（MCD）激素敏感病例中可有效诱导蛋白尿缓解。环孢素在激素依赖型/抵抗型MCD和激素抵抗型局灶节段性肾小球硬化（FSGS）的缓解诱导及肾功能长期维持方面也有效。FSGS的总体缓解率低于MCD，可能需要长期治疗（>12个月）才能实现并维持缓解。环孢素治疗对70 - 80%的激素抵抗型膜性肾病（MGN）患者减少蛋白尿也有益处。在MGN中，与MCD相比，最大益处通常延迟出现（>12周）。环孢素一般耐受性良好且安全。主要担忧仍然是肾毒性，但通过仔细监测患者肾功能；尽量减少维持剂量，并对接受长期治疗的患者进行重复肾活检，这种风险可以降至最低。已根据每种组织学类型的文献最佳证据制定了算法，以帮助为执业肾病学家将环孢素纳入INS管理提供指导。
