Frassinetti Castelo Branco Camurça Fernandes Paula, Bezerra Da Silva Geraldo, De Sousa Barros Fernando A, Costa Oliveira Claudia M, Kubrusly Marcos, Evangelista João B
Division of Nephrology, School of Medicine, Walter Cantidio University Hospital, Federal University of Ceará, Fortaleza, Ceará - Brazil.
J Nephrol. 2005 Nov-Dec;18(6):711-20.
Cyclosporine (CsA) has been used in steroid-resistant idiopathic nephrotic syndrome (INS) in many previous studies.
To evaluate if CsA is a therapeutic option for steroid-resistant nephrotic syndrome.
We performed a retrospective cohort study to evaluate the effects of CsA in 17 steroid-resistant INS patients. The main laboratorial data, before and after the use of CsA, and the response to CsA were evaluated. A literature review on this subject was also done.
Patient age ranged from 2-43 yrs. Pre-treatment renal biopsy demonstrated focal segmental glomerulosclerosis (FSGS) (64%), membranous nephropathy (MGN) (12%), mesangial glomerulonephritis (MSGN) (12%) and minimal change disease (MCD) (12%). Pre-treatment laboratory tests showed a mean 24-hr proteinuria of 4372 +/- 2686 mg/dL. Treatment with CsA was given for a minimum of 3 months and a maximum of 98 months. Mean 24-hr proteinuria declined from 3181 +/- 2277 before CsA to 915 +/- 1140 mg/24 hr after CsA (p<0.001). Remission was seen in 70.5% of patients, being 52.9% complete and 17.6% partial. The adverse effects observed were nephrotoxicity (11.7%), hypertrichosis (5.8%) and gingival hyperplasia (5.8%). Relapses were seen in eight patients (47%), with posterior remission in six patients (75%).
Data from the literature suggest that CsA is a good therapeutic option for patients with steroid-resistant INS, being effective in reducing proteinuria. The beneficial effect of CsA demonstrated in our study was limited due to its design and the small sample size.
在许多先前的研究中，环孢素（CsA）已被用于治疗激素抵抗型特发性肾病综合征（INS）。
评估环孢素是否为激素抵抗型肾病综合征的一种治疗选择。
我们进行了一项回顾性队列研究，以评估环孢素对17例激素抵抗型INS患者的疗效。评估了使用环孢素前后的主要实验室数据以及对环孢素的反应。还对该主题进行了文献综述。
患者年龄在2至43岁之间。治疗前肾活检显示局灶节段性肾小球硬化（FSGS）（64%）、膜性肾病（MGN）（12%）、系膜增生性肾小球肾炎（MSGN）（12%）和微小病变病（MCD）（12%）。治疗前实验室检查显示24小时平均蛋白尿为4372±2686mg/dL。环孢素治疗时间最短3个月，最长98个月。24小时平均蛋白尿从环孢素治疗前的3181±2277mg/dL降至治疗后的915±1140mg/24小时（p<0.001）。70.5%的患者病情缓解，其中完全缓解率为52.9%，部分缓解率为17.6%。观察到的不良反应有肾毒性（11.7%）、多毛症（5.8%）和牙龈增生（5.8%）。8例患者（47%）复发，其中6例患者（75%）随后病情缓解。
文献数据表明，环孢素是激素抵抗型INS患者的一种良好治疗选择，可有效降低蛋白尿。由于本研究的设计和样本量较小，环孢素在本研究中显示的有益效果有限。
