From the Guidelines
The treatment of nephrotic syndrome should prioritize immunosuppression to achieve immunological and clinical remission, with regimens combining alkylating agents with glucocorticoids or anti-CD20 agents like rituximab being effective options, as recommended by the 2021 KDIGO clinical practice guideline 1.
Key Considerations
- Initial therapy typically includes corticosteroids like prednisone (1 mg/kg/day for adults or 60 mg/m²/day for children) for 4-16 weeks depending on response.
- Diuretics such as furosemide (20-80 mg daily) help manage fluid retention and edema.
- ACE inhibitors or ARBs (like enalapril 5-40 mg daily or losartan 25-100 mg daily) are prescribed to reduce proteinuria and protect kidney function.
Steroid-Resistant Cases
- Immunossuppressants including cyclophosphamide, cyclosporine, tacrolimus, or mycophenolate mofetil may be added.
- Supportive care involves sodium restriction (2-3 g/day), moderate protein intake (0.8-1 g/kg/day), and sometimes anticoagulants like warfarin or heparin to prevent thrombotic complications if albumin levels are very low.
- Statins help manage hyperlipidemia.
Treatment Duration and Monitoring
- Treatment duration varies based on the underlying cause, with minimal change disease often responding well to steroids while focal segmental glomerulosclerosis may require longer immunosuppression.
- Regular monitoring of kidney function, protein levels, and medication side effects is essential throughout treatment, as noted in the Canadian Society of Nephrology commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis 1.
- A risk prediction tool, similar to what is available for IgAN, would be welcome to guide treatment decisions, as mentioned in the KDOQI US commentary on the 2021 KDIGO clinical practice guideline for the management of glomerular diseases 1.
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
- Growth velocity may be affected by corticosteroid treatment, 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 Overview
- Nephrotic syndrome is a kidney disorder characterized by excessive excretion of protein in the urine, leading to complications such as edema, infection, and thrombosis 3, 4, 5, 6, 7.
- The treatment of nephrotic syndrome depends on the underlying cause, with minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) being the most common histopathological lesions in idiopathic nephrotic syndrome (INS) 3.
Corticosteroid Therapy
- Corticosteroids are considered first-line therapy for the treatment of nephrotic syndrome, with prednisone being the most commonly used steroid 5, 6, 7.
- The initial dose of prednisone is typically 60 mg/m2 per day, administered for 4 to 6 weeks, followed by a tapering dose of 40 mg/m2 every other day for at least 6 to 8 weeks 5.
- Corticosteroid therapy can induce remissions in most cases of minimal change nephrotic syndrome (MCNS), with response rates of 93% in children and 81% in adults 7.
Alternative Therapies
- For patients who are steroid-resistant or steroid-dependent, alternative therapies such as cyclophosphamide, cyclosporine, and levamisole may be used 3, 4, 7.
- Cyclophosphamide can induce long-term remissions in 25-70% of children and is also beneficial in adults, while cyclosporine produces complete remissions in 85% of children and 79% of adults with steroid dependence 7.
- Levamisole may be helpful in steroid-dependent cases, but data about its efficacy are conflicting 7.
Treatment Outcomes
- The course of nephrotic syndrome in most patients is characterized by relapse and remission, with significant morbidity due to acute kidney injury and other complications 4, 5.
- With proper treatment, most patients can achieve remission and preserve kidney function, although some may progress to end-stage renal disease 4, 5.