What is the treatment for Nephrotic Syndrome?

From the Guidelines

Treatment of nephrotic syndrome should start with corticosteroids, such as prednisone, at a dose of 1 mg/kg/day (maximum 80 mg) for 4-16 weeks, as recommended by the KDIGO guideline 1.

Initial Treatment

The initial treatment of nephrotic syndrome focuses on addressing the underlying cause and managing symptoms.

• Corticosteroids, such as prednisone, are the first-line treatment, usually started at 1 mg/kg/day (maximum 80 mg) for 4-16 weeks depending on response, followed by a gradual taper 1.
• The use of corticosteroids has been shown to improve proteinuria and slow progression to end-stage renal disease (ESRD) in patients with nephrotic syndrome 1.

Steroid-Resistant Cases

For steroid-resistant cases, calcineurin inhibitors like cyclosporine (3-5 mg/kg/day divided into two doses) or tacrolimus (0.05-0.1 mg/kg/day divided into two doses) may be used 1.

• These medications work by reducing inflammation in the kidneys and decreasing protein leakage through the glomerular membrane.
• The choice of calcineurin inhibitor depends on the patient's specific condition and potential side effects, with cyclosporine being preferred over tacrolimus in patients with a high risk of developing diabetes 1.

Supportive Care

Supportive care is essential and includes:

• Diuretics like furosemide (20-80 mg daily) to manage edema.
• ACE inhibitors or ARBs such as enalapril (5-40 mg daily) or losartan (25-100 mg daily) to reduce proteinuria and protect kidney function.
• Statins to address hyperlipidemia.
• Anticoagulation with low molecular weight heparin or warfarin may be necessary if there's a high risk of thrombosis.
• Dietary modifications include moderate sodium restriction (2-3 g/day) and adequate protein intake (0.8-1 g/kg/day) 1.

Recent Guidelines

The most recent guideline from the KDIGO 1 suggests that children with frequently relapsing nephrotic syndrome be offered a glucocorticoid-sparing agent, such as rituximab, which has been shown to be effective in reducing the frequency of relapses.

• Regular monitoring of kidney function, protein levels, and medication side effects is crucial for effective management of nephrotic syndrome.

From the FDA Drug Label

Published studies provide evidence of efficacy and safety in pediatric patients for the treatment of nephrotic syndrome (patients >2 years of age) The treatment for nephrotic syndrome includes the use of corticosteroids, such as prednisone, in pediatric patients over 2 years of age 2.

• Key points:
  • Efficacy and safety have been established in pediatric patients for the treatment of nephrotic syndrome
  • Pediatric patients should be carefully observed with frequent measurements of blood pressure, weight, height, intraocular pressure, and clinical evaluation for the presence of infection, psychosocial disturbances, thromboembolism, peptic ulcers, cataracts, and osteoporosis
  • The linear growth of pediatric patients treated with corticosteroids should be monitored, and the potential growth effects of prolonged treatment should be weighed against clinical benefits obtained and the availability of treatment alternatives.

From the Research

Nephrotic Syndrome Treatment

• Nephrotic syndrome is a collection of symptoms indicating kidney damage, particularly to the glomeruli, which can be caused by various diseases including minimal change disease 3, 4, 5, 6.
• The first-line treatment for minimal change disease, a common cause of nephrotic syndrome, is corticosteroids, with prednisone being a commonly used steroid 3, 5, 6.
• Studies have shown that corticosteroids can induce remission in most cases of minimal change disease, with response rates of 93% in children and 81% in adults 5.
• The standard dose of prednisone for treating nephrotic syndrome is 60 mg/m2 per day (2 mg/kg per day) for 4 to 6 weeks, followed by 40 mg/m2 per dose (1.5 mg/kg) every other day for at least 6 to 8 weeks 6.
• However, some studies suggest that lower doses of prednisone, such as 1-1.5 mg/kg/day, may be effective in achieving remission of steroid-sensitive nephrotic syndrome relapse, with a significantly lower cumulative dose 7.
• Alternative therapies, such as cyclophosphamide, chlorambucil, and cyclosporine, may be used in cases of steroid-dependent or steroid-resistant nephrotic syndrome, although they can have toxic adverse effects 5.
• Tacrolimus monotherapy has been shown to be an effective alternative treatment for patients with minimal change disease who wish to avoid steroid therapy 3.

Treatment Outcomes

• Complete remission of nephrotic syndrome can be achieved in a significant proportion of patients with minimal change disease using corticosteroids or alternative therapies 3, 4, 5.
• Relapses of nephrotic syndrome are common, but can often be managed with further treatment, including corticosteroids or alternative therapies 5, 6, 7.
• The choice of treatment and dose should be individualized based on the patient's response and tolerance to therapy, as well as the presence of any complications or comorbidities 6, 7.

Complications and Comorbidities

• Patients with nephrotic syndrome are at risk of developing complications such as dyslipidemia, infection, and thrombosis, which should be recognized and managed promptly 6.
• The use of corticosteroids and alternative therapies can also be associated with adverse effects, which should be carefully monitored and managed 5, 6, 7.