From the Guidelines
The full workup for nephrotic syndrome includes a comprehensive laboratory evaluation, imaging studies, and a kidney biopsy to identify the underlying cause and assess disease severity, with initial treatment guided by the 2024 KDIGO clinical practice guideline for the management of lupus nephritis, which recommends renin-angiotensin system blockade, blood pressure control, and combined immunosuppressive treatment with glucocorticoid and one other agent 1. The initial laboratory evaluation should include:
- Urinalysis with microscopy
- 24-hour urine collection for protein (with >3.5g/day confirming nephrotic-range proteinuria)
- Serum albumin (typically <3.5g/dL)
- Lipid panel (to assess hyperlipidemia)
- Basic metabolic panel to evaluate kidney function Additional blood tests should include:
- Complete blood count
- Serum protein electrophoresis
- Complement levels (C3, C4)
- Antinuclear antibody
- Hepatitis B and C serologies
- HIV testing
- Age-appropriate cancer screening Imaging typically includes a renal ultrasound to assess kidney size and structure and rule out obstruction. The definitive diagnostic procedure is a kidney biopsy, which identifies the specific glomerular pathology causing nephrotic syndrome, such as minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, or other conditions, as recommended by the KDIGO practice guideline on glomerulonephritis 1. In patients with nephrotic syndrome, immunosuppression has been shown to improve proteinuria and slow progression to ESRD, with options including corticosteroids, cyclosporin, and mycophenolate mofetil, as discussed in the Canadian Society of Nephrology commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis 1. The treatment of nephrotic syndrome should be guided by the underlying cause, with the KDIGO 2024 clinical practice guideline for the management of lupus nephritis providing recommendations for the management of nephrotic-range proteinuria, including renin-angiotensin system blockade, blood pressure control, and combined immunosuppressive treatment 1.
From the FDA Drug Label
The FDA drug label does not answer the question.
From the Research
Treatment Overview
The treatment for nephrotic syndrome typically involves the use of corticosteroids, with the goal of inducing remission and managing relapses.
- The initial treatment for nephrotic syndrome often involves high-dose corticosteroids, such as prednisone, which can induce remissions in most cases 2, 3, 4, 5, 6.
- The dose of prednisone is typically 60 mg/m(2)/day (maximum 80 mg/day) for 4-6 weeks, followed by 40 mg/m(2) on alternate days for at least 6-8 weeks 2, 4.
Steroid-Dependent and Steroid-Resistant Cases
For patients who are steroid-dependent or steroid-resistant, alternative therapies may be necessary.
- Cyclophosphamide and chlorambucil have been shown to induce long-term remissions in some patients with steroid-dependent or steroid-resistant nephrotic syndrome 2.
- Cyclosporine has also been effective in inducing complete remissions in patients with steroid dependence and resistance 2.
- Levamisole may be helpful in steroid-dependent cases, but the data on its efficacy are conflicting 2.
Relapse Prevention
To prevent relapses, patients may need to continue taking corticosteroids on alternate days for an extended period.
- In adults, prednisone on alternate days for 1 year after the presenting attack can decrease the risk of relapse 2.
- In children with frequently relapsing nephrotic syndrome, daily prednisone during viral infections can reduce the rate of relapse 6.
Side Effects and Complications
Corticosteroid therapy can have significant side effects, including opportunistic infections, osteoporosis, and behavioral disturbances.