| Abstract Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises a group of severe autoimmune diseases characterized by necrotizing inflammation of the vessel wall, with multisystem involvement. Severe AAV frequently presents with life-threatening complications, including rapidly progressive glomerulonephritis, diffuse alveolar hemorrhage, and central nervous system involvement, and is associated with a critical clinical course and poor prognosis. In recent years, owing to advances in the understanding of its pathogenesis and the iterative development of biological agents, a paradigm shift has emerged in the diagnostic and therapeutic approach to severe AAV. This shift has transitioned the treatment paradigm from traditional broad-spectrum, intensive immunosuppression toward a precision-targeted, low-toxicity, and highly efficacious strategy, primarily centered on the anti-CD20 monoclonal antibody (rituximab) and the complement C5a receptor antagonist (avacopan). This article systematically reviews diagnostic stratification, optimization of induction and maintenance therapeutic strategies, application of novel targeted agents, complication management, and prognostic evaluation for severe AAV. By integrating the latest clinical guidelines and evidence-based findings, this review aims to provide a practical reference for standardized clinical practice. |
