What is the treatment for vasculitis?

From the Guidelines

Treatment of Vasculitis Treatment of vasculitis typically involves a multi-faceted approach, with the primary goal of reducing inflammation and preventing organ damage. The most effective treatment approach often involves a combination of immunosuppressive agents and glucocorticoids, with the specific treatment regimen depending on the severity and type of vasculitis 1.

Induction of Remission

For patients with ANCA-associated vasculitis, a combination of glucocorticoids and either rituximab or cyclophosphamide is recommended for induction of remission, with rituximab preferred in relapsing disease 1. The use of cyclophosphamide is associated with a risk of bladder cancer, and patients should have periodic urine analysis for the length of their follow-up 1.

• Glucocorticoids, such as prednisone, are typically started at a dose of 50-75 mg/day, with a stepwise reduction in dose according to clinical response 1.
• Rituximab is administered at a dose of 1 gram infused over 4-6 hours, repeated 2 weeks later 1.
• Cyclophosphamide may be used at a dose of 1.5-2.5 mg/kg/day, although its use is associated with significant toxicity 1.

Maintenance of Remission

For maintenance of remission, treatment with rituximab, azathioprine, or methotrexate may be used, with the specific choice of agent depending on the severity and type of vasculitis, as well as patient preferences and comorbidities 1.

• Azathioprine may be used at a dose of 1-2 mg/kg/day 1.
• Methotrexate may be used at a dose of 10-20 mg/week 1.
• Rituximab may be used at a dose of 1 gram infused over 4-6 hours, repeated at intervals of 6-12 months 1.

Special Considerations

Plasma exchange may be considered for patients with severe renal disease or diffuse pulmonary hemorrhage, although its use is not routinely recommended 1. The use of reduced-dose glucocorticoid regimens may reduce the risk of serious infections, and should be considered in patients at high risk of infection 1.

Overall, the treatment of vasculitis requires a personalized approach, taking into account the severity and type of disease, as well as patient comorbidities and preferences. A combination of immunosuppressive agents and glucocorticoids is often the most effective treatment approach, with the specific regimen depending on the individual patient's needs 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 treatment for vasculitis, specifically Granulomatosis with Polyangiitis (GPA) and Microscopic Polyangiitis (MPA), includes:

• Rituximab (RITUXAN): 375 mg/m2 once weekly for 4 weeks
• Cyclophosphamide: 2 mg/kg daily for 3 to 6 months
• Methylprednisolone: 1,000 mg of pulse intravenous per day for 1 to 3 days within 14 days prior to initial infusion
• Prednisone: oral, with pre-specified tapering 2

From the Research

Treatment for Vasculitis

There are no direct research papers provided to assist in answering this question. However, some studies mention vasculitis in the context of COVID-19 and other diseases.

• The study 3 discusses the association between COVID-19 and vascular disease, specifically endotheliitis and vasculitis, but does not provide information on treatment.
• The study 4 mentions the use of antiplatelet agents/anticoagulants as part of a multipronged therapeutic approach for COVID-19, but does not specifically address vasculitis treatment.
• The study 5 discusses the effectiveness of multifaceted interventions in changing health-care professionals' behaviors, but does not provide information on vasculitis treatment.
• The study 6 discusses preclinical multi-target strategies for myocardial ischemia-reperfusion injury, but does not specifically address vasculitis treatment.
• The study 7 discusses the role of antioxidants and natural products in inflammation, but does not provide information on vasculitis treatment.

Potential Treatment Approaches

While there is no direct evidence on vasculitis treatment, some studies suggest that:

• Multifaceted interventions may be effective in treating complex diseases 5
• Antiplatelet agents/anticoagulants may be used to address vascular complications 4
• Antioxidants and natural products may have a role in reducing inflammation 7 However, these findings are not directly related to vasculitis treatment and more research is needed to determine effective treatment approaches.