What is the initial treatment for nephrotic syndrome?

Initial Treatment for Nephrotic Syndrome

Corticosteroids are the recommended initial 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 Algorithm for Nephrotic Syndrome

Initial Diagnosis and Assessment

• Confirm nephrotic syndrome diagnosis: proteinuria (>3.5g/day), hypoalbuminemia, edema, and hyperlipidemia
• Evaluate kidney function via GFR or eGFR
• Quantify urine protein excretion
• Consider kidney biopsy in adults to determine underlying cause

First-Line Treatment

1. For children:

   • Prednisone/prednisolone 60 mg/m²/day (maximum 60 mg) as a single daily dose for 4-6 weeks 1, 2
   • Follow with 40 mg/m²/day (maximum 40 mg) on alternate days for 4-6 weeks 2
   • Total treatment duration: 8-12 weeks 1

2. For adults:

   • Prednisone/prednisolone 1 mg/kg/day (maximum 80 mg) as a single daily dose 1
   • Maintain high-dose corticosteroids for minimum 4 weeks if remission achieved, maximum 16 weeks if not achieved 1
   • After remission, taper slowly over 6 months 1

Monitoring Response

• Remission defined as absence of proteinuria (trace/negative) on urine dipstick for 3 consecutive days 2
• Continue initial high-dose treatment for minimum 4 weeks if remission achieved
• Continue up to 16 weeks maximum if remission not achieved 1

Special Considerations

Steroid Contraindications or Intolerance

For patients with relative contraindications to high-dose corticosteroids (uncontrolled diabetes, psychiatric conditions, severe osteoporosis):

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

Steroid Resistance

If no response after minimum 8 weeks of corticosteroid treatment:

1. Perform diagnostic kidney biopsy (if not done already)
2. Use CNI as initial therapy for steroid-resistant nephrotic syndrome 1
3. Continue CNI for minimum 6 months; if partial/complete remission achieved, continue for at least 12 months 1
4. Combine with low-dose corticosteroid therapy 1
5. Add ACE inhibitors or ARBs 1

Management of Complications

Edema Management

• Fluid restriction during edematous phase
• Sodium restriction
• Loop diuretics for significant edema 2

Infection Prevention

• For children with frequent relapses: daily prednisone 0.5 mg/kg/day for 5-7 days during infections 1, 2
• Pneumococcal and annual influenza vaccination 1
• Defer live vaccines until prednisone dose <1 mg/kg/day 1

Common Pitfalls to Avoid

1. Dosing errors: Note that dosing by weight (2 mg/kg/day) is not equivalent to dosing by body surface area (60 mg/m²/day) for children weighing <30 kg 3

2. Inadequate initial treatment duration: Ensure minimum 4 weeks of high-dose treatment, with total treatment course of 8-12 weeks 1

3. Rapid steroid tapering: Taper slowly over 6 months after remission to reduce relapse risk 1

4. Overlooking infections: Infections are common triggers for relapses and require prompt treatment 2

5. Inadequate monitoring: Regular assessment of blood pressure, weight, height, and drug toxicity is essential 2

The treatment approach differs between children and adults, with children having a higher likelihood of minimal change disease that responds well to corticosteroids (93% response rate) 4, while adults may require kidney biopsy to guide therapy as they have more diverse underlying causes 5, 6.