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

From the Guidelines

The initial treatment for ANCA vasculitis typically involves a two-phase approach: induction therapy followed by maintenance therapy, with the most recent and highest quality study recommending rituximab or cyclophosphamide in combination with glucocorticoids for induction, and less toxic medications such as azathioprine, methotrexate, or mycophenolate mofetil for maintenance 1.

Induction Therapy

• The goal of induction therapy is to rapidly control inflammation and prevent organ damage.
• A combination of high-dose corticosteroids (usually prednisone 1 mg/kg/day, maximum 60-80 mg daily) plus either cyclophosphamide or rituximab is recommended.
• Cyclophosphamide can be given orally (2 mg/kg/day) or intravenously (15 mg/kg every 2-3 weeks), while rituximab is administered as 375 mg/m² weekly for four weeks.

Maintenance Therapy

• After 3-6 months of induction or once remission is achieved, maintenance therapy begins with less toxic medications.
• Options for maintenance therapy include azathioprine (2 mg/kg/day), methotrexate (20-25 mg weekly), or mycophenolate mofetil (2 g/day), while gradually tapering corticosteroids.
• Pneumocystis pneumonia prophylaxis with trimethoprim-sulfamethoxazole is essential during immunosuppression.

Recent Recommendations

• The 2024 KDIGO clinical practice guideline for the management of ANCA-associated vasculitis recommends rituximab or cyclophosphamide in combination with glucocorticoids for induction, and less toxic medications such as azathioprine, methotrexate, or mycophenolate mofetil for maintenance 1.
• The guideline also suggests that plasma exchange may be added for severe disease with organ failure, and that a reduced dose regimen of glucocorticoid may reduce serious infections 1.

From the FDA Drug Label

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 15 years of age or older, diagnosed with GPA (75% of patients) or MPA (24% of patients) according to the Chapel Hill Consensus conference criteria (1% of the patients had unknown vasculitis type) All patients had active disease, with a Birmingham Vasculitis Activity Score for Granulomatosis with Polyangiitis (BVAS/GPA) greater than or equal to 3, and their disease was severe, with at least one major item on the BVAS/GPA. Ninety-six (49%) of patients had new disease and 101 (51%) of patients had relapsing disease Patients in both arms received 1,000 mg of pulse intravenous methylprednisolone per day for 1 to 3 days within 14 days prior to initial infusion. 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 initial treatment for ANCA (Antineutrophil Cytoplasmic Antibodies) vasculitis is Rituximab or Cyclophosphamide.

• Rituximab: 375 mg/m2 once weekly for 4 weeks
• Cyclophosphamide: 2 mg/kg daily for 3 to 6 months Both treatments are used in the remission induction phase, and are typically preceded by pulse intravenous methylprednisolone (1,000 mg per day for 1 to 3 days) and followed by oral prednisone (1 mg/kg/day, not exceeding 80 mg/day) with pre-specified tapering 2

From the Research

Initial Treatment for ANCA Vasculitis

The initial treatment for ANCA (Antineutrophil Cytoplasmic Antibodies) vasculitis typically involves a combination of immunosuppressive medications and glucocorticoids.

• The goal of treatment is to induce remission and prevent further damage to affected organs.
• Several studies have compared the efficacy of different treatment regimens, including rituximab and cyclophosphamide, in inducing remission in ANCA-associated vasculitis 3, 4, 5.

Treatment Options

Some of the treatment options for ANCA vasculitis include:

• Rituximab, a monoclonal antibody that targets B cells, which are involved in the immune response 3, 4, 5, 6.
• Cyclophosphamide, an immunosuppressive medication that is often used in combination with glucocorticoids 3, 4, 5.
• Mycophenolate mofetil, an immunosuppressive medication that has been used as an alternative to cyclophosphamide 7.
• Glucocorticoids, such as prednisolone, which are used to reduce inflammation and suppress the immune system 3, 4, 5, 6.

Efficacy of Treatment Regimens

Studies have shown that rituximab-based regimens can be effective in inducing remission in ANCA-associated vasculitis, with remission rates ranging from 70-90% 3, 4, 5.

• A study comparing rituximab and cyclophosphamide found that both regimens had similar remission rates and safety profiles 3.
• Another study found that a combination of rituximab and cyclophosphamide was effective in inducing remission in patients with ANCA-associated vasculitis, with a median time to complete remission of 4 months 4.
• A randomized clinical trial found that a reduced-dose glucocorticoid plus rituximab regimen was noninferior to a high-dose glucocorticoid plus rituximab regimen in inducing remission in ANCA-associated vasculitis 6.