From the Guidelines
Management of nephrotic syndrome should prioritize treating the underlying cause, reducing proteinuria, and preventing complications, with first-line therapy including corticosteroids, such as prednisone 1 mg/kg/day, as recommended by the most recent guidelines 1. The treatment approach should be tailored to the individual patient, taking into account the underlying cause of the nephrotic syndrome, with the following key components:
- Angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin receptor blockers (ARBs) to reduce proteinuria and protect kidney function
- Diuretics, such as furosemide, to manage edema
- Statins, such as atorvastatin, to address hyperlipidemia
- Dietary sodium restriction to less than 2 grams daily and moderate protein intake (0.8-1 g/kg/day) as supportive measures For primary causes, immunosuppressive therapy may be necessary, including:
- Corticosteroids for minimal change disease
- Calcineurin inhibitors, such as cyclosporine, for steroid-resistant cases Thromboprophylaxis with anticoagulants should be considered for patients with severe hypoalbuminemia (albumin <2.0 g/dL) due to increased thrombotic risk, as noted in the guidelines 1. Regular monitoring of kidney function, proteinuria, electrolytes, and albumin levels is essential to assess treatment response and adjust therapy accordingly, with the goal of minimizing morbidity, mortality, and improving quality of life, as emphasized by the most recent and highest quality study 1.
Some key points to consider in the management of nephrotic syndrome include:
- The importance of treating the underlying cause of the disease
- The use of corticosteroids as first-line therapy, as recommended by the guidelines 1
- The need for regular monitoring of kidney function and proteinuria to adjust therapy accordingly
- The consideration of thromboprophylaxis with anticoagulants in patients with severe hypoalbuminemia, as noted in the guidelines 1
- The importance of dietary sodium restriction and moderate protein intake as supportive measures, as recommended by the guidelines 1.
Overall, the management of nephrotic syndrome requires a comprehensive approach that takes into account the underlying cause of the disease, the need for immunosuppressive therapy, and the importance of regular monitoring and supportive measures to minimize morbidity, mortality, and improve quality of life, as emphasized by the most recent and highest quality study 1.
From the FDA Drug Label
Cyclophosphamide is an alkylating drug indicated for treatment of: ... Minimal Change Nephrotic Syndrome in Pediatric Patients: biopsy proven minimal change nephrotic syndrome in pediatric patients who failed to adequately respond to or are unable to tolerate adrenocorticosteroid therapy ( 1. 2) Recommended oral dose: 2 mg per kg once daily for 8 to 12 weeks (maximum cumulative dose 168 mg per kg).
The management of Minimal Change Nephrotic Syndrome in pediatric patients who failed to adequately respond to or are unable to tolerate adrenocorticosteroid therapy is cyclophosph dessert with a recommended oral dose of 2 mg per kg once daily for 8 to 12 weeks 2.
- Key points:
- The treatment is only indicated for pediatric patients.
- The patient should have biopsy-proven minimal change nephrotic syndrome.
- The patient should have failed to adequately respond to or are unable to tolerate adrenocorticosteroid therapy.
From the Research
Management of Nephrotic Syndrome
The management of nephrotic syndrome involves treating the underlying cause, reducing proteinuria, and managing complications.
- Treatment of the underlying cause may involve corticosteroids, cytotoxic agents, and cyclosporine, depending on the histologic subclass of the disease 3, 4, 5.
- Reduction of proteinuria can be achieved through dietary protein restriction, angiotensin-converting-enzyme inhibitors, and nonsteroidal anti-inflammatory drugs 3, 4.
- Management of complications includes the use of diuretics, low-cholesterol and low-fat diets, lipid-lowering agents, and anticoagulants 3, 4.
Treatment of Specific Causes
- Minimal change disease (MCD) is typically treated with prednisone, with a response rate of 93% in children and 81% in adults 5.
- Focal segmental glomerulosclerosis (FSGS) may be treated with cyclophosphamide or cyclosporine, with a partial or complete remission rate of up to 20% 4.
- Membranous glomerulopathy may be treated with prednisolone and chlorambucil, with an improved renal outcome compared to controls 4.
Treatment of Relapses
- Relapses of steroid-sensitive nephrotic syndrome can be treated with lower doses of prednisone, such as 1-1.5 mg/kg/day, which may be equally effective as the standard dose 6.
- Second-line options for refractory steroid-sensitive and -resistant nephrotic syndrome include cyclophosphamide, levamisole, mycophenolic acid, calcineurin inhibitors, and rituximab 7.