What is the initial treatment for ANCA (Antineutrophil Cytoplasmic Antibody) vasculitis?

From the Guidelines

The initial treatment for ANCA vasculitis should involve induction therapy with high-dose corticosteroids combined with either rituximab or cyclophosphamide, with rituximab being the preferred option for relapsing disease, as recommended by the most recent guidelines 1.

Key Considerations

• The choice of induction therapy should be based on the severity of the disease and the presence of organ-threatening or life-threatening manifestations.
• Rituximab is preferred over cyclophosphamide for relapsing disease, due to its superior remission rate and lower risk of adverse events 1.
• Cyclophosphamide can be administered orally or intravenously, with the intravenous route resulting in a lower total dosage and reduced risk of leukopenia 1.
• Glucocorticoids should be used at a starting dose of 50-75 mg prednisolone equivalent/day, with a stepwise reduction according to clinical guidelines 1.
• Pneumocystis pneumonia prophylaxis should be considered for all patients receiving immunosuppressive therapy.

Treatment Options

• Rituximab: 375 mg/m² weekly for four weeks or two 1000 mg infusions separated by two weeks.
• Cyclophosphamide: oral (1.5-2 mg/kg/day) or intravenous (15 mg/kg every 2-3 weeks).
• Glucocorticoids: oral prednisolone or prednisone starting at 1.0 mg/kg/d, with a gradual tapering regimen.

Maintenance Therapy

• After induction of remission, maintenance therapy with less toxic agents like azathioprine, methotrexate, or rituximab should be initiated 1.
• The duration of maintenance therapy should be individualized based on the patient's disease severity and response to treatment.

From the FDA Drug Label

Following 2 years of treatment with RITUXAN + MTX, 57% of patients had no progression of structural damage. A total of 197 patients with active, severe GPA and MPA (two forms of ANCA Associated Vasculitides) were treated in a randomized, double-blind, active-controlled, multicenter, non-inferiority study, conducted in two phases – a 6 month remission induction phase and a 12 month remission maintenance phase. Patients were randomized in a 1:1 ratio to receive either RITUXAN 375 mg/m2 once weekly for 4 weeks or oral cyclophosphamide 2 mg/kg daily for 3 to 6 months in the remission induction phase The main outcome measure for both GPA and MPA patients was achievement of complete remission at 6 months defined as a BVAS/GPA of 0, and off glucocorticoid therapy The study demonstrated non-inferiority of RITUXAN to cyclophosphamide for complete remission at 6 months

The initial treatment for ANCA (Antineutrophil Cytoplasmic Antibody) vasculitis is either RITUXAN 375 mg/m2 once weekly for 4 weeks or oral cyclophosphamide 2 mg/kg daily for 3 to 6 months, as part of a remission induction phase, with the goal of achieving complete remission at 6 months, defined as a BVAS/GPA of 0, and off glucocorticoid therapy 2. RITUXAN has been shown to be non-inferior to cyclophosphamide for complete remission at 6 months. Key points to consider include:

• Treatment options: RITUXAN or cyclophosphamide
• Remission induction phase: 6 months
• Goal: Complete remission, defined as BVAS/GPA of 0, and off glucocorticoid therapy
• Efficacy: RITUXAN has been shown to be non-inferior to cyclophosphamide for complete remission at 6 months 2

From the Research

Initial Treatment for ANCA Vasculitis

The initial treatment for ANCA (Antineutrophil Cytoplasmic Antibody) vasculitis typically involves a combination of medications to induce remission and manage symptoms. Some key points to consider include:

• The use of glucocorticoids in combination with rituximab and/or cyclophosphamide as induction therapy 3
• Plasma exchange should be considered in patients with severe renal impairment and diffuse alveolar haemorrhage 4, 3
• Rituximab is a preferred option for remission induction, especially in situations where cyclophosphamide may be problematic 5

Treatment Options

Some treatment options for ANCA vasculitis include:

• Combination therapy with rituximab and cyclophosphamide, which has been shown to be efficacious and well-tolerated 6
• Glucocorticoids, which are commonly used to induce remission, but can have significant side effects 7
• Plasma exchange, which may be beneficial in patients with severe disease, but did not reduce the incidence of death or end-stage kidney disease in one study 4

Maintenance Therapy

Maintenance therapy is an important aspect of ANCA vasculitis management, with options including:

• Rituximab, which has been shown to be effective in maintaining remission 5
• Monitoring of kidney function, proteinuria, ANCA, and immunoglobulin levels to adjust treatment as needed 3