From the Guidelines
Treatment of p-ANCA associated vasculitis depends on the severity and extent of the disease. The recommended initial treatment is with glucocorticoids, such as prednisone, at a dose of 1 mg/kg/day, with a maximum of 60 mg/day, for 4 weeks 1.
- If the disease is severe or does not respond to glucocorticoids, an immunosuppressor such as cyclophosphamide can be added at a dose of 2 mg/kg/day for 3-6 months, or rituximab at a dose of 375 mg/m²/week for 4 weeks 1.
- In mild or moderate cases, methotrexate can be used at a dose of 20-25 mg/week for 6-12 months, or azathioprine at a dose of 2 mg/kg/day for 6-12 months 1.
- It is essential to monitor renal function, blood pressure, and blood tests to adjust treatment as necessary. The duration of treatment varies according to the clinical response and remission of the disease, but it is generally maintained for at least 12-18 months 1.
- Regular medical follow-up is crucial to evaluate the response to treatment and adjust the dose or change the medication if necessary.
- Additionally, avacopan, an oral C5a receptor antagonist, has been shown to be an effective alternative to glucocorticoid treatment in AAV, with potential to improve kidney outcomes 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. 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 In the RITUXAN group, 44% of patients achieved CR at 6 and 12 months, and 38% of patients achieved CR at 6,12, and 18 months
The treatment for vasculitis associated with p-ANCA (perinuclear anti-neutrophil cytoplasmic antibodies) is Rituximab.
- Rituximab has been shown to be non-inferior to cyclophosphamide in achieving complete remission at 6 months in patients with GPA and MPA, two forms of ANCA-associated vasculitis.
- The study demonstrated that 64% of patients treated with Rituximab achieved complete remission at 6 months, compared to 53% of patients treated with cyclophosphamide 2.
From the Research
Treatment for Vasculitis Associated with p-ANCA
The treatment for vasculitis associated with p-ANCA (perinuclear anti-neutrophil cytoplasmic antibodies) involves the use of various immunosuppressive agents. Some of the key treatment options include:
- Glucocorticoids, which are used to reduce inflammation and suppress the immune system 3
- Non-glucocorticoid immunosuppressants, such as cyclophosphamide, azathioprine, mycophenolate, and methotrexate, which are used to reduce the immune system's attack on the blood vessels 4, 3
- Rituximab, a monoclonal antibody that targets B cells and has been shown to be effective in maintaining disease remission in ANCA-associated vasculitis 5
- Plasma exchange, which may be used in severe cases of vasculitis to remove antibodies and other immune factors from the blood 4
Treatment Regimens
The treatment regimens for vasculitis associated with p-ANCA may vary depending on the severity of the disease and the patient's response to treatment. Some studies have shown that the use of rituximab and other immunosuppressive agents can be effective in reducing the risk of relapse and improving outcomes in patients with ANCA-associated vasculitis 6, 5. The Pan American League of Associations for Rheumatology has developed guidelines for the treatment of ANCA-associated vasculitis, which include recommendations for the use of glucocorticoids, non-glucocorticoid immunosuppressants, and plasma exchange 4.
Special Considerations
The treatment of vasculitis associated with p-ANCA requires careful consideration of the patient's individual needs and circumstances. For example, patients with kidney involvement may require more aggressive treatment to prevent long-term damage 7. Additionally, the use of certain immunosuppressive agents may be limited by socioeconomic factors, such as access to care and the cost of medications 4.