From the Guidelines
The treatment of ANCA-associated glomerulonephritis should involve a two-phase approach, with induction of remission using rituximab (375 mg/m² weekly for 4 weeks) or cyclophosphamide (1.5-2 mg/kg/day orally or 15 mg/kg IV every 2-3 weeks) in combination with high-dose corticosteroids, followed by maintenance therapy with less toxic agents like azathioprine, mycophenolate mofetil, or rituximab, as supported by the most recent and highest quality study 1.
Induction of Remission
The induction phase typically lasts 3-6 months and aims to rapidly control the autoimmune process and prevent further kidney damage.
- Rituximab has been shown to be equally effective as cyclophosphamide in achieving remission, with a similar rate of infectious complications 1.
- Cyclophosphamide can be administered orally or intravenously, with the intravenous route resulting in a lower total dosage and reduced risk of leukopenia 1.
- High-dose corticosteroids, such as methylprednisolone 500-1000 mg IV for 3 days, followed by prednisone 1 mg/kg/day with gradual taper, are used in combination with rituximab or cyclophosphamide.
Maintenance Therapy
The maintenance phase aims to prevent relapse and minimize toxicity, using less toxic agents like:
- Azathioprine (2 mg/kg/day)
- Mycophenolate mofetil (1-2 g/day)
- Rituximab (500 mg every 6 months)
- Low-dose corticosteroids are continued during the maintenance phase.
Plasma Exchange and Supportive Care
- Plasma exchange may be considered in severe cases with pulmonary hemorrhage or rapidly declining kidney function, although its routine use is not recommended 1.
- Supportive care includes blood pressure control, management of fluid and electrolyte imbalances, and dialysis if needed.
Certainty of Evidence
The overall certainty of evidence is moderate, with the RCTs comparing rituximab with cyclophosphamide reporting important outcomes of remission and relapse, and the certainty of the evidence rated as moderate for these outcomes due to serious imprecision 1.
From the Research
Treatment Options for ANCA-Associated Glomerulonephritis
- The treatment for ANCA-associated glomerulonephritis typically involves immunosuppressive therapy, with the goal of inducing remission and preventing further kidney damage 2, 3, 4, 5, 6.
- Several studies have compared the efficacy of different treatment regimens, including rituximab, cyclophosphamide, and plasma exchange 2, 3, 4, 5, 6.
Induction Therapy
- Rituximab and cyclophosphamide are commonly used as induction therapy for ANCA-associated glomerulonephritis, with both treatments showing similar efficacy in achieving remission 2, 3, 5.
- A study published in 2022 found that rituximab and cyclophosphamide had similar remission rates, but the addition of plasma exchange to cyclophosphamide induction therapy conferred short-term benefits with higher dialysis-free rates at 12 months 2.
- Another study published in 2010 found that rituximab was not inferior to cyclophosphamide for induction of remission in severe ANCA-associated vasculitis, and may be superior in relapsing disease 3.
Combination Therapy
- Combination therapy with rituximab, low-dose cyclophosphamide, and plasma exchange has been shown to be effective in treating severe ANCA-associated vasculitis, with high rates of disease remission and recovery of independent kidney function 4, 6.
- A study published in 2021 found that combination treatment with rituximab, low-dose cyclophosphamide, and plasma exchange resulted in a high rate of disease remission and prolonged B cell depletion, with low rates of relapse and serious infections 6.
Plasma Exchange
- Plasma exchange has been shown to be beneficial in patients with severe ANCA-associated glomerulonephritis, particularly those with diffuse alveolar hemorrhage or rapidly progressive glomerulonephritis 2, 4, 6.
- A study published in 2022 found that the addition of plasma exchange to cyclophosphamide induction therapy conferred short-term benefits with higher dialysis-free rates at 12 months 2.