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


CONCLUSIONS
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.
Frassinetti Castelo Branco Camurça Fernandes
Paula
P
Division of Nephrology, School of Medicine, Walter Cantidio University Hospital, Federal University of Ceará, Fortaleza, Ceará - Brazil. paulafernandes@fortalnet.com.br
Bezerra Da Silva
Geraldo
G
De Sousa Barros
Fernando A
FA
Costa Oliveira
Claudia M
CM
Kubrusly
Marcos
M
Evangelista
João B
JB
D000293:Adolescent; D000328:Adult; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003404:Creatinine / Q000097:blood; D016572:Cyclosporine / Q000627:therapeutic use*; D018450:Disease Progression; D004351:Drug Resistance; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D009404:Nephrotic Syndrome / Q000097:blood / Q000188:drug therapy* / Q000473:pathology; D012008:Recurrence; D012074:Remission Induction; D012189:Retrospective Studies; D016896:Treatment Outcome
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D003404:Creatinine