What is the management of Nephrotic Syndrome (a condition characterized by severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema)?

From the Guidelines

Management of nephrotic syndrome should prioritize a comprehensive approach, including renin-angiotensin system blockade, blood pressure control, and combined immunosuppressive treatment with glucocorticoid and one other agent, as recommended by the most recent guidelines 1.

Key Considerations

• The primary goal is to reduce proteinuria and preserve kidney function while managing the syndrome's manifestations.
• Initial treatment should focus on identifying and addressing the primary disease through kidney biopsy when appropriate.
• Supportive care is crucial and includes sodium restriction, fluid restriction in severe edema, diuretics, and ACE inhibitors or ARBs to reduce proteinuria and preserve kidney function.
• Thromboprophylaxis with anticoagulants should be considered in patients with severe hypoalbuminemia due to hypercoagulability risk.
• Statins are recommended for dyslipidemia management.
• Albumin infusions may be used in severe cases with intractable edema or circulatory compromise, always followed by diuretics.

Treatment Approach

• For minimal change disease, the first-line therapy is oral prednisone at 1 mg/kg/day (maximum 80 mg) for 4-16 weeks, followed by a slow taper over 2-5 months.
• For steroid-resistant cases or other histological variants, calcineurin inhibitors like cyclosporine or tacrolimus may be used.
• The choice of immunosuppressive therapy should be individualized based on patient characteristics, such as obesity, diabetes, and psychiatric conditions 1.

Monitoring and Adjustment

• Regular monitoring of kidney function, electrolytes, albumin levels, and proteinuria is essential to assess treatment response and adjust therapy accordingly.
• The treatment approach should be multifaceted, addressing both the immunological mechanisms driving proteinuria and the metabolic complications of the syndrome.
• The most recent guidelines should be consulted to ensure the best possible outcomes for patients with nephrotic syndrome 1.

From the FDA Drug Label

To induce a diuresis or remission of proteinuria in the nephrotic syndrome, without uremia, of the idiopathic type or that due to lupus erythematosus

• Management of nephrotic syndrome: Prednisone can be used to induce a diuresis or remission of proteinuria in the nephrotic syndrome, without uremia, of the idiopathic type or that due to lupus erythematosus 2.
• The use of prednisone in this context is specifically for the idiopathic type or that due to lupus erythematosus, and it is essential to note that it is not indicated for other types of nephrotic syndrome.
• Key consideration: The absence of uremia is a crucial factor in the use of prednisone for managing nephrotic syndrome.

From the Research

Definition and Causes of Nephrotic Syndrome

• Nephrotic syndrome is defined by a triad of clinical features: oedema, substantial proteinuria (> 3.5 g/24 hours) and hypoalbuminaemia (< 30 g/L) 3
• It is often associated with hyperlipidaemia, thromboembolism and an increased risk of infection 3
• Nephrotic syndrome develops following pathological injury to renal glomeruli, which may be a primary problem or secondary to a systemic disorder such as diabetes mellitus 3

Diagnosis of Nephrotic Syndrome

• Patients typically present with periorbital oedema or dependent pitting oedema 3
• Proteinuria should be documented by a quantitative measurement, such as urine protein: creatinine ratio (PCR) or albumin: creatinine ratio (ACR) 3
• PCR > 300-350 mg/mmol indicates nephrotic range proteinuria 3
• Urgent referral to a nephrologist and a renal biopsy are usually necessary to establish the underlying cause of the disease 3, 4

Treatment and Management of Nephrotic Syndrome

• Treatment aims to prevent or slow further kidney damage, addressing the underlying medical condition and symptoms such as edema, proteinuria, hyperlipidemia 4
• Treatment may include fluid and sodium restriction, oral or intravenous diuretics, and angiotensin-converting enzyme inhibitors 5
• Corticosteroids are considered first-line therapy for treatment of nephrotic syndrome, especially in children 6
• Alternative therapies may be used in cases of corticosteroid-dependent or corticosteroid-resistant nephrotic syndrome, but caution must be exercised due to potential toxic adverse effects 6

Complications and Prognosis of Nephrotic Syndrome

• Complications of nephrotic syndrome include venous thromboembolism, acute renal failure, and serious bacterial infection 5
• The course of nephrotic syndrome in most patients is that of relapse and remission 6
• Establishing the underlying cause of the disease and managing symptoms and complications are crucial for effective treatment and management of nephrotic syndrome 7