ANCA-associated vasculitis therapy reference (2026)

ANCA-associated vasculitis (granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis) therapy in 2026 organises around induction, maintenance, and refractory disease.

Induction (severe disease): rituximab is the modern preferred induction agent over cyclophosphamide for most patients, given the substantially better safety profile. High-dose oral corticosteroid is paired with rituximab. Avacopan (C5a receptor antagonist) is added for severe active ANCA-associated vasculitis and substantially reduces the cumulative glucocorticoid dose.

Induction (less severe disease): rituximab or methotrexate (in mild-to-moderate disease) plus corticosteroid.

Maintenance: rituximab in scheduled maintenance every 4 to 6 months for 18 to 24 months or longer is the modern standard, replacing earlier azathioprine maintenance protocols. Methotrexate or azathioprine remain options in selected populations.

Eosinophilic granulomatosis with polyangiitis (EGPA): mepolizumab carries EGPA approval and is now widely used; benralizumab has reported positive EGPA pivotal data. Standard induction-and-maintenance applies for the more vasculitic component.

Refractory disease: alternative induction agents, additional immunosuppression, plasma exchange in selected severe cases.

Emerging mechanism programs: BAFF-targeted, JAK pathway, additional complement-pathway programs in late-stage trials.

The diagnostic-and-monitoring pathway is integral. ANCA testing (PR3-ANCA, MPO-ANCA), tissue biopsy when feasible, and structured organ-system assessment are part of the standard workup; relapse monitoring guides maintenance decisions.