Nephrotic Syndrome: Relapsing and Steroid-Dependent Disease

Nephrotic Syndrome

• Nephrotic syndrome is characterized by edema, plasma albumin < 2.5 gm/dl, and proteinuria > 40 mg/m2/h
• Remission is defined as urinary protein excretion < 4 mg/m2/h or dipstick neg/trace for 3 consecutive days
• Steroid response is achieved when remission is achieved with steroid therapy alone
• Relapse occurs when urinary protein excretion > 40 mg/m2/h or dipstick 2+ for 3 consecutive days having previously been in remission
• Frequent relapses are defined as two or more relapses within 6 months of initial response or 3 or more in 12 months
• Steroid dependence is characterized by two consecutive relapses occurring during corticosteroid treatment or within 14 days of cessation
• Steroid resistance is defined as failure to achieve response in spite of 8 weeks of prednisolone 60 mg/m2/day

Treatment of Nephrotic Syndrome

• For frequently relapsing and steroid-dependent disease, prednisone is prescribed at 2 mg/kg/d (60 mg/m2/d) as a single morning dose until the patient has been free of proteinuria for at least 3 days
• Following remission of proteinuria, prednisone is reduced to 1.5 mg/kg (40 mg/m2) given as a single dose on alternate days and tapered over 3 or more months
• A steroid-sparing agent, such as cyclophosphamide or cyclosporin A, may be considered once proteinuria is in remission
• For steroid-resistant disease and focal segmental glomerulosclerosis (FSGS), cyclosporin A is the most frequently recommended treatment
• Alkylating agents, such as cyclophosphamide, may be used in steroid-resistant disease

Corticosteroid Therapy

• Oral prednisone is prescribed at 60 mg/m2/d (2 mg/kg/d) for 6 weeks
• Maintenance therapy involves oral prednisone at 40 mg/m2 (or 1.5 mg/kg) given as a single dose on alternate days for 6 weeks
• Longer duration of alternate-day steroid treatment may further reduce the number of children with subsequent relapses