Treatment of Nephrotic Syndrome

Initial Therapy

The Kidney International guideline recommends initiating high-dose glucocorticoid therapy with prednisone at 60 mg/m²/day (maximum 80 mg/day) for 4-6 weeks, followed by alternate-day therapy at 40 mg/m²/day for 4-6 weeks 1
Lower doses of prednisone (1 mg/kg/day for 8 weeks) with treatment duration extended to ≥16 weeks can improve remission rates, as supported by the American College of Pediatric Nephrology and the Kidney International guideline 2, 3, 4, 5, 6, 7

The Nature Reviews Nephrology guideline suggests using furosemide 0.5-2 mg/kg/dose (up to 6 times daily, maximum 10 mg/kg/day) for significant edema, and preferring amiloride over spironolactone if a potassium-sparing diuretic is needed 8
Albumin infusion (20-25%, 1 g/kg) should be considered only if there are signs of hypovolemia, severe edema causing respiratory distress, and furosemide 0.5-2 mg/kg can be administered at the end of infusion 8

The Kidney International guideline suggests considering cyclophosphamide (2 mg/kg/day for 8-12 weeks, maximum cumulative dose 168 mg/kg) or calcineurin inhibitors (cyclosporine 3-5 mg/kg/day or tacrolimus) for frequent relapses or steroid dependence 1, 7
The American Journal of Kidney Diseases guideline suggests using steroid-sparing agents due to the risk of steroid toxicity, with a strength of evidence of 1C 5, 3
Levamisole (2.5 mg/kg on alternate days for 12-24 months) can be used as a therapeutic option for patients with frequent relapses or corticosteroid dependence, as suggested by the American Journal of Kidney Diseases guideline 5, 7
Mycophenolate mofetil (600-1200 mg/m²/day divided into 2 doses for 6-24 months) can be considered, with monitoring of complete blood count every 4 weeks 7

Regular assessment of proteinuria, renal function, and steroid-related adverse effects is recommended, with a target proteinuria of <0.5-0.7 g/24h by 12 months, as supported by the KDIGO guidelines 9, 7
Infection prevention is crucial, with monitoring for and prompt treatment of infections, and regular monitoring of blood pressure, weight, height, and presence of infections, as recommended by the American Academy of Pediatrics and the KDIGO guidelines 7, 5, 3, 9
The KDIGO guidelines recommend pneumococcal and annual influenza vaccination, and deferring live vaccines until prednisone dose is <1 mg/kg/day, with a strength of evidence of 1C 3, 9

The goal of treatment is to achieve a reduction in proteinuria by at least 25% by 3 months, 50% by 6 months, according to the Annals of the Rheumatic Diseases 10
If partial or complete remission is achieved, treatment should be continued for a minimum of 12 months, as recommended by the International Society of Nephrology 7
If no partial remission is achieved within 6 months, calcineurin inhibitors should be discontinued, as recommended by the Kidney International guideline 7

Short-term complications of nephrotic syndrome include hypovolemia, acute kidney injury, infections, and thrombosis, while long-term complications include growth retardation, hypertension, cataracts, osteoporosis, and diabetes, as noted by the American Journal of Kidney Diseases 7
Common pitfalls in the management of nephrotic syndrome include inadequate initial steroid duration, rapid steroid tapering, delayed introduction of steroid-sparing agents, overlooking infections, and inadequate monitoring of drug toxicity, as highlighted by the Kidney International guideline and the American Journal of Kidney Diseases 7, 5