What is the initial treatment approach for patients diagnosed with vasculitis?

From the Guidelines

The initial treatment approach for patients diagnosed with vasculitis should involve a combination of glucocorticoids and either cyclophosphamide or rituximab for remission-induction of new-onset organ-threatening or life-threatening ANCA-associated vasculitis (AAV) 1. This approach is based on the 2016 EULAR/ERA-EDTA recommendations for the management of AAV, which suggest that treatment with a combination of glucocorticoids and either cyclophosphamide or rituximab is recommended for remission-induction of new-onset organ-threatening or life-threatening AAV 1. Some key points to consider in the treatment of vasculitis include:

• The use of glucocorticoids, such as prednisone, to quickly control inflammation and prevent organ damage
• The addition of immunosuppressive agents, such as cyclophosphamide or rituximab, as steroid-sparing agents and to maintain remission
• The importance of prophylaxis against steroid-induced complications, including calcium/vitamin D supplementation, proton pump inhibitors, and Pneumocystis pneumonia prophylaxis with trimethoprim-sulfamethoxazole
• The need for treatment duration to vary by vasculitis type, but typically involving high-dose steroids for 2-4 weeks followed by a gradual taper over months, while immunosuppressants may continue for 1-2 years It's also important to note that patients with AAV should be managed in close collaboration with, or at, centres of expertise, as recommended by the 2022 update of the EULAR recommendations for the management of AAV 1. Additionally, the 2016 EULAR/ERA-EDTA recommendations suggest that plasma exchange should be considered for patients with AAV and a serum creatine level of ≥500 mmol/L (5.7 mg/dL) due to rapidly progressive glomerulonephritis in the setting of new or relapsing disease 1. Overall, the treatment of vasculitis requires a multidisciplinary approach and should be individualized based on the specific type and severity of the disease, as well as the patient's overall health and medical history.

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 initial treatment approach for patients diagnosed with vasculitis (specifically GPA and MPA) is 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, with the goal of achieving complete remission at 6 months, defined as a BVAS/GPA of 0, and off glucocorticoid therapy 2.

From the Research

Initial Treatment Approach for Vasculitis

The initial treatment approach for patients diagnosed with vasculitis depends on the severity and type of vasculitis.

• For severe antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, a combination regimen of rituximab, low-dose intravenous cyclophosphamide, oral glucocorticoids, and plasma exchange may be effective 3.
• For cutaneous vasculitis, treatment should include avoidance of triggers and exclusion of vasculitis-like syndromes, and may involve leg elevation, avoidance of standing, and therapy with NSAIDs, colchicine, or dapsone 4.
• For ANCA-associated vasculitis, rituximab may be as effective as cyclophosphamide for remission induction, and may be superior in relapsing disease 5.
• For giant cell arteritis, tocilizumab, an interleukin-6 inhibitor, has been approved for treatment and may increase the rate of sustained remission 6, 7.

Treatment Options

• Glucocorticoids are commonly used to treat vasculitis, but may be associated with severe morbidity and mortality 4, 7.
• Immunossuppressants such as cyclophosphamide, azathioprine, and methotrexate may be used to treat severe vasculitis 4, 5.
• Biologic therapies such as rituximab and tocilizumab may be effective in certain settings, such as ANCA-associated vasculitis and giant cell arteritis 3, 5, 7.
• Plasma exchange may be used in combination with other treatments for severe vasculitis 3.