In this episode of GN in Ten, hosts Dr. Kenar Jhaveri and Dr. Koyal Jain break down the essential "must-knows" for ANCA-associated vasculitis (AAV). Geared toward board preparation and clinical practice, this bite-sized session covers everything from the initial diagnosis and the role of kidney biopsy to the latest advancements in induction and maintenance therapy.

Key Discussion Points

• Diagnosis & Biopsy Strategy: Why checking specific MPO and PR3 titers is more reliable than general C/P-ANCA. The hosts also discuss the value of biopsy in older patients to assess chronicity and guide treatment duration.
• Induction Therapy: Comparing the "three pillars" of induction: Rituximab, Cyclophosphamide (IV vs. oral), and the increasingly popular combination therapy for organ-threatening disease.
• The Steroid Taper: A shift toward "less is more." Following the PEXIVAS protocol, the hosts explain how to aggressively taper steroids to minimize toxicity, especially when using adjunctive therapies.
• Plasma Exchange (PLEX): Does it still have a role? A nuanced look at the PEXIVAS trial results and why PLEX is now largely reserved for severe pulmonary hemorrhage or rapidly worsening AKI.
• Avacopan (C5a Receptor Inhibitor): Insights from the ADVOCATE trial on how this steroid-sparing agent is changing the landscape of AAV treatment.
• Maintenance: Long-term strategies using Rituximab (typically up to 18 months) or Azathioprine, and when to consider switching agents for refractory cases.

Essential Reading & Resources

To master ANCA vasculitis, the hosts recommend reviewing these landmark studies:

• PEXIVAS: Plasma Exchange and Glucocorticoids in ANCA-Associated Vasculitis — Evaluated PLEX and reduced-dose steroids.
• ADVOCATE: Avacopan for the Treatment of ANCA-Associated Vasculitis — The landmark trial for C5a receptor inhibition.
• RAVE: Rituximab versus Cyclophosphamide for ANCA-Associated Vasculitis — Established rituximab as a standard for induction.
• MAINRITSAN: Rituximab versus Azathioprine for Maintenance in ANCA-Associated Vasculitis — Compared maintenance strategies.
• RITUXVAS: Rituximab versus Cyclophosphamide in ANCA-Associated Renal Vasculitis — Induction in patients with severe renal involvement.
• RITAZAREM: Rituximab as Maintenance Therapy in Relapsing ANCA-Associated Vasculitis — Focus on maintenance for relapsing disease.