What is the treatment for nephrotic syndrome?

Treatment of Nephrotic Syndrome

The treatment of nephrotic syndrome requires specialized management by a pediatric nephrology team, with therapy tailored to the underlying cause and severity of disease. 1

Initial Assessment and Classification

• Perform clinical and biological assessment including:

  • Screening for congenital/secondary infections
  • Genetic analysis
  • Evaluation of blood volume status
  • Assessment for hypoalbuminemia, proteinuria, and hyperlipidemia

• Classify nephrotic syndrome:

  1. Congenital/infantile (presenting in first 3 months)
  2. Primary/idiopathic (most common in adults: focal segmental glomerulosclerosis and membranous nephropathy) 2, 3
  3. Secondary (diabetes mellitus is most common cause in adults) 2

Treatment Algorithm

1. Symptomatic Management (All Patients)

• Fluid and sodium restriction to manage edema 2

• Diuretics:

  • Furosemide (0.5-2 mg/kg per dose, IV or oral up to six times daily; maximum 10 mg/kg per day)
  • Administer over 5-30 minutes to minimize ototoxicity
  • If potassium-sparing diuretic needed, prefer amiloride over spironolactone 1

• Anti-proteinuric agents:

  • Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs)
  • These reduce glomerular protein loss via dose-dependent mechanism 1

2. Cause-Specific Treatment

For Primary/Idiopathic Nephrotic Syndrome:

• Corticosteroids (first-line therapy):
  • Prednisone is indicated for idiopathic nephrotic syndrome 4, 5
  • Steroid-responsiveness has greater prognostic value than renal histology 5

For Steroid-Resistant or Complicated Cases:

• Consider second-line agents:
  • Calcineurin inhibitors (cyclosporine)
  • Mycophenolate mofetil
  • Rituximab
  • Alkylating agents 3, 5

For Infection-Associated Nephrotic Syndrome:

• Treat with specific antimicrobial agents
• For congenital syphilis: penicillin G (50,000 U/kg IV)
• For congenital CMV: ganciclovir (6 mg/kg, every 12h for 15-21 days) followed by valganciclovir 1

3. Prevention and Management of Complications

• Thromboembolism prophylaxis:

  • High-risk complication requiring preventive measures 2, 3
  • Prophylactic anticoagulation if central venous access is required 1

• Infection prevention:

  • Monitor for and prevent bacterial and viral infections
  • Patients are at increased risk for serious bacterial infections 2

• Hyperlipidemia management:

  • Monitor and treat elevated cholesterol levels 6

• Nutritional support:

  • Ensure adequate nutrition to prevent undernutrition 6
  • Consider growth hormone substitution in children with growth issues 1

Special Considerations for Congenital Nephrotic Syndrome

• Refer to specialized pediatric nephrology unit
• Avoid intravenous fluids and saline
• Use albumin infusions based on clinical indicators of hypovolaemia (oliguria, acute kidney injury, prolonged capillary refill time, tachycardia, hypotension)
• Consider nephrectomy for persistent, severe disease despite optimal management 1

Follow-up and Monitoring

• Regular monitoring of kidney function, proteinuria, and albumin levels
• Evaluate patients 3 months after an AKI episode for resolution or worsening of kidney disease 7
• Monitor pediatric patients for growth velocity as this may be affected by corticosteroid treatment 4

Pitfalls to Avoid

1. Overreliance on immunosuppression for genetic forms: Most congenital nephrotic syndrome cases are caused by genetic abnormalities not targeted by immunosuppressive agents 1

2. Fluid overload: Excessive fluid administration without proper assessment of volume status can lead to pulmonary edema 7

3. Medication toxicity: Corticosteroids can cause significant adverse effects, especially in pediatric patients (growth velocity decrease, psychosocial disturbances, cataracts, osteoporosis) 4

4. Missing secondary causes: Always screen for underlying conditions like diabetes, infections, or drugs that can cause nephrotic syndrome 8

5. Delayed recognition of complications: Thromboembolism, acute kidney injury, and serious infections require prompt recognition and management 2, 3