Korean J Pediatr.  2008 Mar;51(3):293-280. 10.3345/kjp.2008.51.3.293.

Therapeutic response of cyclosporine and outcome in steroid resistant nephrotic syndrome

• Choi HS
• Lee JH
• Park YS

• KMID: 2280402
• DOI: http://doi.org/10.3345/kjp.2008.51.3.293

Abstract

PURPOSE: The aim of our study was to evaluate the therapeutic response to cyclosporine, time to remission and side effects in steroid resistant nephrotic syndrome (SRNS).
METHODS
This study included 22 children with idiopathic SRNS who were treated with cyclosporine between June 1989 and August 2006. Medical records were reviewed retrospectively.
RESULTS
The mean age of patients at diagnosis was 5.2+/-3.3 years. The male to female ratio was 1.2:1. Pre-treatment renal biopsies showed minimal change (MCD) in 12 (54.5%), focal segmental glomerulosclerosis (FSGS) in 8 (36.4%), membranous nephropathy (MGN) in one (4.5%) and mesangioproliferative glomerulonephritis in one (4.5%). 15 (68.2%) patients responded to cyclosporine, of whom 11 (91.6%) patients were MCD, 3 (37.5%) patients FSGS, and 1 patient MGN (MCD vs FSGS, P<0.05). The time to remission in patients who responded to cyclosporine was 31.5+/-15.2 days. Four of the 15 cyclosporine responders maintained complete remission even after cessation of the medication Seven still received cyclosporine, 2 were intermittently treated with steroids after discontinuation of cyclosporine, and two were treated with cyclosporine and steroids. The mean duration of cyclosporine therapy was 546.5+/-346.2, 1,392.9+/-439.7, 439.5+/-84.1, and 433.5+/-74.2 days, respectively. We performed post-treatment biopsies in 8 patients and partial interstitial fibrosis and tubular atrophy were found in two.
CONCLUSION
The thrapeutic response of cyclosporine is good in steroid resistant nephrotic syndrome, especially in minimal change. But, there is a problem of long term cyclosporine dependency.

Keyword

Children; Steroid resistant nephrotic syndrome; Cyclosporine

MeSH Terms

Atrophy
Biopsy
Child
Corneal Dystrophies, Hereditary
Cyclosporine
Dependency (Psychology)
Female
Fibrosis
Glomerulonephritis
Glomerulonephritis, Membranous
Glomerulosclerosis, Focal Segmental
Humans
Male
Medical Records
Nephrotic Syndrome
Steroids
Corneal Dystrophies, Hereditary
Cyclosporine
Glomerulosclerosis, Focal Segmental
Steroids