Initial Treatment of Anasarca Due to Nephrotic Syndrome
Begin immediate supportive management with aggressive diuresis using loop diuretics (furosemide) combined with sodium restriction to <2 g/day, while simultaneously initiating high-dose corticosteroid therapy with prednisone 1 mg/kg/day (maximum 80 mg) as a single daily dose for adults, or 60 mg/m²/day (maximum 60 mg) for children. 1, 2, 3, 4
Immediate Supportive Care for Anasarca
Fluid and Edema Management:
- Administer loop diuretics (furosemide) as the first-line agent for managing severe edema and anasarca 3
- Restrict dietary sodium to <2.0 g/day to reduce fluid retention 3
- Avoid routine intravenous albumin infusions; use only if clinical indicators of hypovolemia are present (hypotension, tachycardia, poor perfusion), not based on serum albumin levels alone 3
- Avoid intravenous saline administration, which can worsen edema 3
Blood Pressure and Proteinuria Control:
- Initiate ACE inhibitors or ARBs at maximally tolerated doses for blood pressure control and proteinuria reduction 3
- Target systolic blood pressure <120 mmHg in adults using standardized office measurements 3
Corticosteroid Therapy Protocol
Initial Dosing for Adults:
- Prednisone 1 mg/kg/day (maximum 80 mg) as a single daily dose, OR alternate-day dosing at 2 mg/kg (maximum 120 mg) 1, 2, 4
- Continue high-dose therapy for a minimum of 4 weeks if complete remission is achieved 1, 2
- If remission is not achieved, continue high-dose therapy up to a maximum of 16 weeks as tolerated 1, 2
Initial Dosing for Children:
- Prednisone 60 mg/m²/day (maximum 60 mg) as a single daily dose for 6 weeks 1, 3
- After 6 weeks, transition to 40 mg/m² on alternate days for another 6 weeks 1
- Then taper at 10 mg/m² per week down to 5 mg on alternate days, for a total treatment duration of 16 weeks 1
Tapering After Remission:
- Once complete remission is achieved (proteinuria <200 mg/g or trace/negative on dipstick for 3 consecutive days), taper corticosteroids slowly over 6 months 1, 2
Alternative First-Line Therapy
When to Use Calcineurin Inhibitors Instead:
- Consider calcineurin inhibitors (CNIs) as first-line therapy for patients with contraindications to high-dose corticosteroids 1, 2, 3
- Specific contraindications include: uncontrolled diabetes mellitus, severe psychiatric conditions, severe osteoporosis, or morbid obesity with elevated HbA1c 1
CNI Dosing:
- Cyclosporine: 3-5 mg/kg/day in divided doses 1, 3
- Tacrolimus: 0.05-0.1 mg/kg/day in divided doses (adults) or 0.1-0.2 mg/kg/day (children) 1, 3
- Continue CNI therapy for at least 6 months; if partial remission achieved, continue for minimum 12 months 1, 2
Critical Infection Prevention
Vaccination Requirements:
- Administer pneumococcal vaccination (23-valent or conjugate vaccine) before or early in immunosuppressive therapy 2, 3
- Give annual influenza vaccination to patient and household contacts 2, 3
- Avoid live vaccines once immunosuppressive therapy is initiated 2
Prophylactic Antibiotics:
- Consider trimethoprim-sulfamethoxazole prophylaxis for patients receiving high-dose immunosuppression 3
Monitoring During Initial Treatment
Assess Treatment Response:
- Monitor urine protein daily using dipstick or spot urine protein-to-creatinine ratio 2, 3
- Complete remission defined as: urine protein <200 mg/g (<20 mg/mmol) or trace/negative on dipstick for 3 consecutive days 1, 2
- Check serum creatinine and albumin regularly to assess kidney function and nephrotic syndrome severity 3
Monitor for Steroid Complications:
- Blood glucose monitoring (risk of steroid-induced diabetes) 1
- Blood pressure monitoring (steroid-induced hypertension) 3
- Bone density assessment if prolonged therapy anticipated 1
Common Pitfalls to Avoid
Dosing Errors:
- In children weighing <30 kg, dosing by body weight (2 mg/kg/day) gives significantly lower doses than body surface area dosing (60 mg/m²/day); always use body surface area calculation for children 5
- Do not exceed maximum daily doses: 80 mg for adults, 60 mg for children 1
Premature Treatment Decisions:
- Do not declare steroid resistance until at least 8 weeks of adequate corticosteroid therapy has been completed 2
- Do not stop therapy prematurely if partial response is occurring; continue up to 16 weeks 1, 2
Biopsy Timing:
- In adults, kidney biopsy is generally indicated before initiating immunosuppressive therapy to confirm diagnosis and guide treatment 2, 3
- In children with typical presentation (age 1-10 years, no hematuria, normal blood pressure, normal kidney function), biopsy may be deferred if response to initial steroids occurs 1, 2
- Perform biopsy in children if: steroid-resistant after 8 weeks, age <1 year, atypical features present, or suspicion of secondary causes 1, 2