From the Guidelines
Nephrotic syndrome should be initially treated with corticosteroids, as recommended by the KDIGO practice guideline on glomerulonephritis 1.
Key Considerations
- The treatment of nephrotic syndrome depends on the underlying cause, which should be identified through kidney biopsy when appropriate.
- Initial management includes angiotensin-converting enzyme inhibitors (ACEIs) like enalapril (10-40mg daily) or angiotensin receptor blockers (ARBs) such as losartan (50-100mg daily) to reduce proteinuria.
- Diuretics like furosemide (20-80mg daily) help manage edema.
- Dietary sodium restriction (<2g/day) and moderate protein intake (0.8-1g/kg/day) are recommended.
Immunosuppressive Therapy
- For primary causes, corticosteroids are often first-line therapy, typically prednisone 1mg/kg/day (maximum 80mg) for 4-16 weeks depending on the specific glomerular disease.
- Immunosuppressants such as cyclophosphamide, cyclosporine, or rituximab may be added for steroid-resistant cases.
- Calcineurin inhibitors (CNIs) can be considered as first-line therapy for patients with relative contraindications or intolerance to high-dose corticosteroids, such as uncontrolled diabetes, psychiatric conditions, or severe osteoporosis 1.
Additional Management
- Statins help manage hyperlipidemia.
- Anticoagulation with low molecular weight heparin or warfarin should be considered for patients with severe hypoalbuminemia (<2.0g/dL) due to increased thrombosis risk.
- Regular monitoring of kidney function, proteinuria, albumin levels, and lipid profiles is essential to assess treatment response.
Pathophysiology
- The pathophysiology involves damage to the glomerular filtration barrier, allowing protein leakage into urine, which triggers compensatory hepatic protein and lipid synthesis, resulting in the characteristic clinical presentation.
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
- Nephrotic syndrome is an indication for the use of prednisone, specifically to induce a diuresis or remission of proteinuria, in the idiopathic type or that due to lupus erythematosus, without uremia 2.
- The pediatric population can be treated with corticosteroids for nephrotic syndrome, with patients older than 2 years of age having evidence of efficacy and safety 2.
From the Research
Definition and Treatment of Nephrotic Syndrome
- Nephrotic syndrome is a condition characterized by the leakage of large amounts of protein from the blood into the urine, often requiring treatment with corticosteroids 3.
- The most common cause of nephrotic syndrome in school-aged children is minimal change disease, which is typically treated with corticosteroids as first-line therapy 3.
Corticosteroid Treatment
- Traditionally, relapses of steroid-sensitive nephrotic syndrome are treated with prednisone 2 mg/kg/day or 60 mg/m2/day 4, 5.
- However, studies have shown that lower doses of prednisone (1-1.5 mg/kg/day) may be equally effective in achieving remission while reducing the cumulative dose 4.
- The dose of prednisone is typically given for 4 weeks and then reduced to 40 mg/m2 on alternate days for a few weeks 5.
Alternative Therapies
- For children with corticosteroid-dependent or corticosteroid-resistant nephrotic syndrome, alternative therapies such as cyclophosphamide, cyclosporine, and levamisole may be used 5, 6.
- These therapies may have variable efficacy and can be associated with toxic adverse effects, requiring careful consideration and monitoring 5, 6.
Management of Complications
- Patients with nephrotic syndrome are prone to complications such as dyslipidemia, infection, and thrombosis, which require recognition and management 3, 7.
- Treatment may include a low-salt diet, diuretics, statins, and other medications to reduce the risk of these complications 7.
Idiopathic Steroid-Resistant Nephrotic Syndrome
- Children with idiopathic steroid-resistant nephrotic syndrome may be treated with immunosuppressive agents such as cyclosporin, tacrolimus, and mycophenolate mofetil 6.
- The efficacy and safety of these agents vary, and further research is needed to determine the best treatment approaches for this condition 6.