Treatment of steroid-resistant nephrotic syndrome with cyclosporine: study of 17 cases and a literature review.

摘要

BACKGROUND: Cyclosporine (CsA) has been used in steroid-resistant idiopathic nephrotic syndrome (INS) in many previous studies. OBJECTIVE: To evaluate if CsA is a therapeutic option for steroid-resistant nephrotic syndrome. METHODS: 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. RESULTS: 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%). CONCLUSION: 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.