What is the initial treatment for nephrotic syndrome?

Initial Treatment for Nephrotic Syndrome

Corticosteroids are the recommended first-line treatment for nephrotic syndrome, specifically prednisone or prednisolone at a daily single dose of 1 mg/kg (maximum 80 mg) or alternate-day dose of 2 mg/kg (maximum 120 mg). 1

Treatment Protocol for Initial Presentation

For Children:

1. Initial therapy:

   • Prednisone/prednisolone 60 mg/m² per day (maximum 60 mg) as a single daily dose 1
   • Continue daily dosing for 4-6 weeks 1

2. Continuation phase:

   • After initial daily dosing, switch to alternate-day dosing at 40 mg/m² (maximum 40 mg) 1
   • Continue alternate-day dosing for 4-6 weeks 1
   • Total treatment duration: 8-16 weeks 1

3. Tapering:

   • After remission, taper steroids slowly over a period of up to 6 months 1
   • Recommended tapering rate: 10 mg/m² per week 1

For Adults:

1. Initial therapy:

   • Prednisone/prednisolone 1 mg/kg/day (maximum 80 mg) as a single daily dose 1
   • Continue for a minimum of 4 weeks if complete remission is achieved 1
   • May require up to 16 weeks if complete remission is not achieved 1

2. Tapering:

   • After achieving remission, taper slowly over a period of up to 6 months 1

Special Considerations

Patients with Contraindications to High-Dose Corticosteroids:

For patients with relative contraindications (uncontrolled diabetes, psychiatric conditions, severe osteoporosis):

• Consider calcineurin inhibitors (CNIs) as first-line therapy instead 1
  • Cyclosporine: 3-5 mg/kg/day in divided doses 1
  • Tacrolimus: 0.05-0.1 mg/kg/day in divided doses 1

Monitoring Response:

• Definition of remission: Urine dipstick trace/negative for at least 3 consecutive days 1
• Expected response time:
  • Children: Average 11 days 2
  • Adults: May take up to 16 weeks 2, 3

Treatment of Relapses

1. For infrequent relapses:

   • Same initial dose as for first episode (60 mg/m² or 1 mg/kg/day) 1
   • Continue until remission (at least 3 days of negative/trace proteinuria) 1
   • Then switch to alternate-day dosing and taper over 4 weeks 1

2. For frequent relapses or steroid-dependent nephrotic syndrome:

   • Consider steroid-sparing agents:
     • Cyclophosphamide: 2-2.5 mg/kg/day for 8 weeks 1
     • Calcineurin inhibitors: for 1-2 years 1
     • Mycophenolate mofetil: 500-1000 mg twice daily for 1-2 years 1

Important Caveats and Pitfalls

1. Dosing considerations:

   • Weight-based dosing (2 mg/kg/day) is not equivalent to BSA-based dosing (60 mg/m²/day) for children weighing <30 kg 4
   • BSA-based dosing is preferred for children to ensure adequate treatment 4

2. Steroid toxicity:

   • Monitor for adverse effects: growth velocity reduction, hypertension, glucose intolerance, bone density loss, cataracts 5
   • Pediatric patients require careful monitoring of growth velocity 5

3. Diagnostic considerations:

   • In steroid-resistant cases, kidney biopsy is required 1
   • Evaluate kidney function and quantify proteinuria 1

4. Infection prevention:

   • For children with frequently relapsing nephrotic syndrome, consider giving daily glucocorticoids 0.5 mg/kg/day during upper respiratory infections for 5-7 days to reduce relapse risk 1
   • Pneumococcal and influenza vaccination recommended 1
   • Defer live vaccines until prednisone dose is below 1 mg/kg daily or 2 mg/kg on alternate days 1

The treatment of nephrotic syndrome requires careful monitoring of response and adjustment of therapy based on clinical presentation. While corticosteroids remain the mainstay of initial treatment, the approach may need to be modified based on response, underlying cause, and patient-specific factors.