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.

In this episode of GN in Ten, hosted by Dr. Kenar Jhaveri and Dr. Koyal Jain, takes a deep dive into the specialized world of pediatric glomerular disease. Joining the show are world-renowned pediatric nephrologists Dr. Louise Oni (Great Ormond Street Hospital, London) and Dr. Jun Oh (University of Hamburg, Germany) to discuss the "pediatric lag" in drug development and the massive global efforts currently underway to bring targeted therapies to children.

Key Highlights

• The C3G Breakthrough: Discussion on the "wave of excitement" following adult trials for iptacopan and pegcetacoplan, and how these results are paving the way for pediatric use.
• SGLT2 Inhibitors in Children: Insights into the collaborative global effort that overcame initial industry waivers to launch a landmark clinical trial for SGLT2 inhibitors in children aged 2–17.
• Challenges in Rare Disease: Why international collaboration and registries are vital for treating ultra-rare conditions like Anti-GBM disease and C3G in small pediatric populations.
• The Future of Genetics: A look toward the next 15 years, where genetic screening may shift the focus from treating active disease to early prevention.
• Managing Long-term Immunosuppression: The delicate balance of using B-cell depletion (Rituximab) in developing immune systems to preserve "childhood" and avoid dialysis.

Featured Guests

• Dr. Louise Oni: Professor of Pediatric Nephrology at Great Ormond Street and Chair of the ESPN Glomerular Disease Working Group.
• Dr. Jun Oh: Professor of Pediatrics at the University of Hamburg and a leader in the ISGD Pediatric Committee.

Resources & Mentioned Studies

• International Society of Glomerular Disease (ISGD): Learn more about the society’s mission and join for free!
• ESPN Glomerular Disease Working Group: Clinical research and educational resources for pediatric nephrologists.
• NephCure: A key partner in driving global pediatric clinical trials and supporting families affected by rare kidney disease.
• APPEAR-C3G Trial (Iptacopan): Phase 3 study evaluating the first approved treatment for C3G.
• VALIANT Trial (Pegcetacoplan): Recent Phase 3 results showing success in both adult and adolescent patients (12+), published in the New England Journal of Medicine.

In this Board Review Bonus episode of GN in Ten, hosts Dr. Kenar Jhaveri (Northwell Health/Hofstra University) and Dr. Koyal Jain (UNC Chapel Hill) dive into the crucial topic of Lupus Nephritis. Special guest, Northwell fellow Dr. Mahmoud Abdelaziz, asks Kenar and Koyal the tough questions about key topics in diagnosing and managing LN.

Tune in to learn about diagnosis and initial suspicion of kidney involvement in Systemic Lupus Erythematosus (SLE), the six histological classifications (Class I-VI) of lupus nephritis, and an overview of induction and maintenance treatment algorithms for Class III and IV (proliferative) and Class V (membranous) lupus nephritis, including the use of reduced-dose glucocorticoids, MMF, cyclophosphamide (Euro-lupus vs. NIH protocols), and newer agents like voclosporin and belimumab. The GN in Ten hosts also discuss essential supportive care, like pneumocystic pneumonia prophylaxis (and the associated controversy with sulfamethoxazole/trimethoprim), multidisciplinary management, and management of special situations like pregnancy and Thrombotic Microangiopathy (TMA).

Referenced Resources and Studies:
KDIGO 2024 Clinical Practice Guideline for the Management of Lupus Nephritis
American College of Rheumatology (ACR) Guidelines for Lupus Nephritis and Systemic Lupus Erythematosus
Euro-Lupus Nephritis Trial and Protocol
NIH Lupus Nephritis Trial and Protocol
LUNAR Trial