An update on the pharmacotherapy of ANCA-associated vasculitis

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare, life-threatening autoimmune conditions requiring complex pharmacological management. Contemporary strategies have transitioned from empirical cytotoxic therapy toward mechanistically targeted approaches, reflecting a deeper understanding of B-cell immunity and the alternative complement pathway. This review synthesizes current guidance and presents recent evidence on treatment modalities and emerging options.

This paper reviews and synthesizes current international guidance from the European League Against Rheumatism (EULAR), the British Society for Rheumatology (BSR), and the American College of Rheumatology (ACR) on remission induction and maintenance for granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Key shifts discussed include the established role of rituximab as a primary induction agent, the implementation of reduced-dose glucocorticoid tapering, and the integration of anti-IL-5 therapies for eosinophilic phenotypes.

In our opinion, the management of AAV has entered a precision era defined by targeted, steroid-sparing regimens. The most significant progress lies in standardizing B‑cell depletion and the emergence of complement inhibition. Despite the evolution of steroid-sparing protocols, mitigation of treatment-related morbidity remains a primary challenge. Ultimately, AAV management must transition toward biomarker-driven precision medicine to individualize therapy and improve long-term outcomes.