ANCA-associated vasculitis-treatment standard.
A treatment standard for ANCA-associated vasculitis describes how the disease has been transformed from a frequently fatal condition into a relapsing/remitting one, and reviews the modern shift toward rituximab, the glucocorticoid-sparing C5a-receptor antagonist avacopan, and reduced glucocorticoid exposure.
Background
ANCA-associated vasculitides (AAV) cause small-vessel necrotizing inflammation and were frequently fatal before immunosuppressive therapy. Treatment has converted AAV into a relapsing/remitting disease, but at the cost of drug-related toxicity and accrued organ damage.
Established and evolving therapy
Glucocorticoids, cyclophosphamide, and immunosuppressants (azathioprine, mycophenolate, methotrexate) were optimized through sequential trials to set a standard of care. Improved understanding of pathophysiology has driven B-cell depletion (rituximab) and complement inhibition for granulomatosis with polyangiitis and microscopic polyangiitis, and IL-5 inhibition (mepolizumab) for eosinophilic GPA, leading to approval of newer agents.
Key recent evidence
The review focuses on recent trial evidence for glucocorticoids, avacopan (an oral C5a-receptor antagonist that reduces glucocorticoid exposure), plasma exchange, rituximab, and mepolizumab, interpreted alongside the 2022 EULAR management recommendations, with increased attention to minimizing treatment adverse effects and managing comorbidities.
Clinical implications
Contemporary AAV care emphasizes rituximab for induction and maintenance, glucocorticoid minimization (aided by avacopan), and individualized therapy that balances effective immunosuppression against infection and long-term toxicity — with plasma exchange reserved for rapidly progressive glomerulonephritis or severe disease.
Category
Transplant
Source
Nephrol Dial Transplant
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