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Steroid-Dependent Nephrotic Syndrome PDF
Steroid-Dependent Nephrotic Syndrome PDF
Nephrotic Syndrome
Nicola Sumorok
January 10, 2012
Idiopathic Nephrotic Syndrome
• Nephrotic syndrome without known etiology
• Heavy Proteinuria > 3.5 g/d in adults or > 1.0 g/m² in children
• Hypoalbuminemia < 3.0 g/dL in adults or < 2.5 g/dL in children
• Edema
• Hypercholesterolemia
• The three leading histological variants associated with INS are:
• Minimal change disease (MCD)
• Focal segmental glomerulosclerosis (FSGS)
• Membranous nephropathy (MGN)
• Prolonged nephrotic range proteinuria leads to renal scarring
and eventual renal failure
Idiopathic Nephrotic Syndrome
• The duration and severity of proteinuria are known to be
surrogate markers of the progression of glomerular disease
Tejani, et al. KI
(1988) 33:729-734
Results
• 14/20 achieved remission (disappearance of edema,
resolution of proteinuria for at least 3 days, serum albumin
>2.5mg/dl, and normalization of cholesterol)
• There was reduction in proteinuria in the 6 who did not remit
• 40% sustained remission at 1 yr after discontinuation of tx:
Tejani, et al. KI
(1988) 33:729-734
Meyrier, A. NDT
(2003) 18: vi79-vi86
Tacrolimus
• Retrospective cohort study of 10 children with steroid-
dependent nephrotic syndrome who were treated with
Tacrolimus
• 9 pts with minimal change on biopsy, 1 with FSGS
• All patients had initially responded to steroids, and were then
treated with Cyclophosphamide followed by Cyclosporine and
then TAC as steroid sparing agents
• Patients received TAC 0.1 mg/kg/day in two divided doses,
with a target trough level of 5-10 μg/L
• Compared the responses to TAC vs Cyclosporine
• # of relapses per year
• Amount of Prednisone required
Sinha, et al. NDT (2006)
21: 1848-1854
• Mean duration of treatment with CYA was 2 yrs and subsequently with
TAC was 5 yrs
• Adverse events:
• CYA – decrease in GFR (4 pts), histological evidence of CNI toxicity
(2 pts), and new onset HTN (1 pt)
• TAC – new onset HTN (1 pt), new insulin-dependent diabetes (1 pt)
and CNI toxicity (1 pt)
• Overall, no benefit to using TAC over CYA
Sinha, et al. NDT (2006)
21: 1848-1854
Cyclosporine vs Cyclophosphamide
• Prospective, randomized, multicenter, controlled study
• 73 patients with steroid-sensitive idiopathic NS (frequent
relapses or steroid dependence)
• 11 adults and 55 children (7 lost to follow-up not included)
• After inducing remission with Prednisone, patients were
randomized to receive:
• Cyclophosphamide 2.5mg/kg/day x 8 weeks
• Cyclosporine 5mg/kg/day (in adults) or 6mg/kg/day (in children)
x 9months then tapered off over 3 months