Journal of Vasculitis

Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) encompasses a group of rare autoimmune diseases affecting small blood vessels, including granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis. Diagnosing AAV relies on clinical, biological, radiological, and histological findings. Despite advancements in understanding and treating these conditions, challenges remain in accurate prognosis and therapy management. The Five-Factor Score and Birmingham Vasculitis Activity Score are used to evaluate disease severity and guide treatment decisions, yet these tools have limitations and may not fully capture disease complexity. Recent epidemiological studies have highlighted the geographic variability in AAV incidence and the role of ANCAs as diagnostic and prognostic markers. Treatment involves an initial remission induction phase followed by maintenance therapy, often corticosteroids and immunosuppressants like rituximab and cyclophosphamide. However, newer agents such as mepolizumab and avacopan show promise, particularly for specific AAV subtypes. Evaluating sequelae using indices like the Vasculitis Damage Index is critical, given the increased survival rates and focus on quality of life. While current treatment protocols aim to reduce relapse and manage complications, including infections and metabolic issues, there is a need for more tailored approaches. The revised Chapel Hill classification has improved disease definitions, but the absence of ANCAs sometimes complicates diagnosis. This review underscores the necessity for developing more refined prognostic and activity scores to enhance the clinical management of AAV and calls for ongoing research to optimize treatment strategies and outcomes for affected patients.