What is the management of nephrotic syndrome and vasculitis?

From the Guidelines

Management of nephrotic syndrome and vasculitis requires a targeted approach based on the underlying cause, with initial treatment of nephrotic syndrome including corticosteroids such as prednisone at 1 mg/kg/day (maximum 80 mg) for 4-16 weeks depending on response, as recommended by the most recent guidelines 1. For nephrotic syndrome, the treatment approach includes:

• Corticosteroids as the initial treatment, with a recommended dose of prednisone at 1 mg/kg/day (maximum 80 mg) for 4-16 weeks depending on response 1
• Supportive care including diuretics (furosemide 20-80 mg daily), ACE inhibitors (enalapril 5-20 mg daily), and statins for hyperlipidemia
• For steroid-resistant cases, calcineurin inhibitors like cyclosporine (3-5 mg/kg/day in divided doses) or tacrolimus (0.05-0.1 mg/kg/day) are often added For vasculitis management, treatment depends on severity and type, with:
• ANCA-associated vasculitis induction therapy typically including cyclophosphamide (2 mg/kg/day orally or 15 mg/kg IV every 2-3 weeks) or rituximab (375 mg/m² weekly for 4 weeks) combined with high-dose corticosteroids (prednisone 1 mg/kg/day, maximum 80 mg) 1
• Maintenance therapy following with azathioprine (2 mg/kg/day), mycophenolate mofetil (1-1.5 g twice daily), or rituximab (500 mg every 6 months)
• Prophylaxis against Pneumocystis pneumonia with trimethoprim-sulfamethoxazole is essential during immunosuppression Regular monitoring of renal function, proteinuria, blood counts, and drug levels is crucial, as is management of complications like hypertension, edema, and thrombotic risk. Treatment duration varies from months to years depending on disease type, severity, and response, with the goal of achieving remission while minimizing medication side effects.

From the FDA Drug Label

The FDA drug label does not answer the question.

From the Research

Management of Nephrotic Syndrome

• Nephrotic syndrome is defined by a triad of clinical features: oedema, substantial proteinuria (> 3.5 g/24 hours) and hypoalbuminaemia (< 30 g/L) 2
• The most common cause in children is minimal change glomerulonephritis, while in white adults, nephrotic syndrome is most frequently due to membranous nephropathy 2
• Treatment of nephrotic syndrome involves the use of corticosteroids, with most patients responding within 4 weeks to an oral course of prednisone (PDN) 3
• However, corticosteroids have important side effects and 60-80 % of patients relapse, developing frequently relapsing or steroid-dependent forms 3

Management of Vasculitis

• Renal vasculitis presents as rapidly progressive glomerulonephritis and comprises of a group of conditions characterised by acute kidney failure, haematuria and proteinuria 4
• Treatment of renal vasculitis involves the use of steroid and non-steroid agents with or without adjunctive plasma exchange 4
• Plasma exchange as adjunctive therapy significantly reduces the risk of end-stage kidney disease at 12 months 4
• Cyclophosphamide, azathioprine, and mycophenolate mofetil are effective in treating renal vasculitis, with rituximab being an effective remission induction agent 4

Comparison of Treatments

• Cyclosporine versus mycophenolate mofetil for maintenance of remission of steroid-dependent nephrotic syndrome after a single infusion of rituximab showed that cyclosporine appears to be more effective than mycophenolate mofetil for maintaining remission 5
• Non-corticosteroid immunosuppressive medications such as alkylating agents (cyclophosphamide and chlorambucil) significantly reduced the risk of relapse at six to 12 months and 12 to 24 months compared with prednisone alone 6
• Cyclosporin was as effective as cyclophosphamide and chlorambucil at the end of therapy, while levamisole was more effective than steroids alone 6

Treatment Strategies

• Treatment choice is based on the severity of nephrotic syndrome, on patient age, and on drug tolerability 3
• Balancing the use of different drugs to achieve permanent remission with the lowest cumulative number of side effects is essential 3
• Further studies are required to more clearly delineate the appropriate place of newer agents within an evidence-based therapeutic strategy 4