What is the management of nephrotic syndrome?

Management of Nephrotic Syndrome

The management of nephrotic syndrome requires a comprehensive approach centered on fluid and sodium restriction, diuretic therapy with furosemide (0.5-2 mg/kg per dose), and prevention of life-threatening complications including thromboembolism, infection, and malnutrition. 1

Initial Diagnostic Workup

Before initiating treatment, obtain the following laboratory studies to establish baseline status and guide therapy:

• Complete blood biochemistry: hemogram, sodium, chloride, albumin, magnesium, creatinine, urea, total proteins, cholesterol, triglycerides, and glucose 1, 2
• Thyroid function: TSH and free T4, plus serum IgG levels 1, 2
• Calcium-phosphate metabolism: ionized calcium, phosphate, alkaline phosphatase, PTH, and vitamin D 1, 2
• Imaging: abdominal ultrasound to assess renal echogenicity, size, and detect ascites or effusions 1, 2
• Cardiac ultrasound: to evaluate for effusions and left ventricular mass 2

Core Management Strategy

Fluid and Edema Control

Sodium restriction is the first-line intervention for edema management, followed by judicious diuretic use. 1, 2

• Restrict dietary sodium intake as the primary intervention for controlling edema 1, 2
• Restrict fluid intake when feasible, particularly in patients with hyponatremia and severe edema 1, 2
• Administer furosemide 0.5-2 mg/kg per dose (up to six times daily) for patients with intravascular volume overload and preserved renal function 1, 2
• Avoid diuretics entirely in patients showing evidence of intravascular hypovolemia 1, 2
• Do not exceed furosemide doses >6 mg/kg per day for longer than 1 week to prevent complications 2

Albumin Infusion Protocol

• Administer intravenous albumin only when clinically indicated, not routinely to normalize serum albumin levels 2
• The purpose of albumin infusion is to support intravascular volume and reduce edema, not to correct laboratory values 2
• Consider administering furosemide (0.5-2 mg/kg) at the end of albumin infusions unless marked hypovolemia or hyponatremia is present 1, 2
• Home administration by trained caregivers can be considered to reduce hospitalizations and improve quality of life 2

Prevention and Management of Complications

Thromboembolism

• Monitor closely for thromboembolism, especially when serum albumin is significantly low 1, 3
• Consider prophylactic anticoagulation when central venous access is required 1, 2

Infection Prevention

• Implement infection prophylaxis measures, particularly in severe cases and children with congenital nephrotic syndrome 1, 2
• Prophylactic antibiotics are not routinely recommended in adults 4

Nutritional Support

• Ensure adequate nutrition to prevent malnutrition and support growth, especially critical in pediatric patients 1, 2
• Consider growth hormone substitution in children with growth impairment 2

Metabolic Bone Disease

• Supplement with vitamin D (colecalciferol) or 25-OH-D3 (calcifediol) and calcium (250-500 mg/day) when 25-OH-D3 is low, ionized calcium is low, or PTH is elevated 1

Hyperlipidemia

• Consider statin therapy when LDL cholesterol remains persistently elevated in patients with additional cardiovascular risk factors 1

Anemia

• Monitor and treat iron deficiency 1
• Administer erythropoietin in patients with anemia despite iron supplementation 1

Disease-Specific Considerations

Focal Segmental Glomerulosclerosis (FSGS)

• Use SGLT2 inhibitors for renal protection in patients with FSGS, eGFR ≥20 mL/min/1.73 m², and UACR ≥200 mg/g 1

Congenital Nephrotic Syndrome

• Refer to specialized tertiary pediatric nephrology centers for management by multidisciplinary teams 2
• Pursue prolonged conservative management with a stepwise approach; avoid routine early nephrectomies 2
• Consider nephrectomy only in patients with persistent hypovolemia, thrombosis, and failure to thrive despite optimal conservative management 2
• Do not use immunosuppressive agents for genetic forms, as they are unlikely to respond 2, 3
• Ambulatory management is preferred when possible to reduce nosocomial infection risk and improve quality of life 2

Corticosteroid Therapy

• Corticosteroids are frequently first-line therapy for many forms of nephrotic syndrome, particularly in children >2 years of age 5
• Monitor pediatric patients carefully for growth suppression, as growth velocity may be affected even at low systemic doses and without HPA axis suppression 5
• Titrate to the lowest effective dose to minimize growth effects in children 5
• Monitor for complications including infection, thromboembolism, peptic ulcers, cataracts, and osteoporosis 5
• In elderly patients, use cautiously with particular attention to increased risk of diabetes mellitus, fluid retention, and hypertension 5

Critical Pitfalls to Avoid

• Never use diuretics in hypovolemic patients—this can precipitate acute kidney injury and worsen outcomes 1, 2
• Do not administer immunosuppressive therapy for genetic forms of nephrotic syndrome—these will not respond and expose patients to unnecessary toxicity 2, 3
• Avoid routine early nephrectomy in congenital nephrotic syndrome—conservative management should be optimized first 2
• Do not use prophylactic antibiotics or anticoagulation routinely in adults—reserve for specific high-risk situations 4
• Avoid excessive diuretic dosing (>6 mg/kg/day for >1 week)—this increases risk of intravascular volume depletion 2