A bite-size podcast brought to you by the International Society of Glomerular Disease. Nephrologists and glomerular disease experts Dr. Kenar Jhaveri (Northwell Health/Hofstra University) and Dr. Koyal Jain (UNC Chapel Hill) take a lighthearted look at the latest research, discuss clinical practice, and interview leaders in glomerular medicine — all in a short enough time to listen on your coffee break.
GN in Ten
Claim This Podcastby International Society of Glomerular Disease
5.0(10 reviews)
19 episodes
Updated Bi-weekly
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Podcast Overview
A bite-size podcast brought to you by the International Society of Glomerular Disease. Nephrologists and glomerular disease experts Dr. Kenar Jhaveri (Northwell Health/Hofstra University) and Dr. Koyal Jain (UNC Chapel Hill) take a lighthearted look at the latest research, discuss clinical practice, and interview leaders in glomerular medicine — all in a short enough time to listen on your coffee break.
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9/2/2023
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Recent Episodes
June 16, 2026
Board Review Bonus 6: Hereditary Nephritis
<p><strong>Board Review Bonus 6: Hereditary Nephritis</strong></p><p>In this "Board Review Bonus" (BRB) episode, hosts Dr. Kenar Jhaveri and Dr. Koyal Jain provide a comprehensive clinical overview of hereditary nephritis—also known as Alport syndrome—a complex genetic condition with critical renal and extrarenal implications. From a sneaky real-world case of an 18-year-old female presenting with isolated microscopic hematuria to classic textbook syndromic features , our hosts break down the essential knowledge required for both board preparation and clinical practice.</p><p><br></p><p>The discussion moves beyond the classic X-linked male phenotype to explore autosomal varieties and why thin basement membrane disease (TBMD) is increasingly viewed as part of the Alport spectrum rather than a purely benign entity. Dr. Jain and Dr. Jhaveri emphasize critical management strategies, highlighting why an accurate diagnosis is vital to protect patients from receiving unnecessary and ineffective immunosuppressive therapies.</p><p><br></p><p><strong>Key Topics Covered:</strong></p><ul><li><strong>The Hematuria Differential:</strong> Evaluating a young patient with a family history of hematuria, prioritizing Alport syndrome, thin basement membrane disease, and IgA nephropathy.</li><li><strong>Genetics and Inheritance Patterns:</strong> A detailed look at mutations in the alpha 3, 4, and 5 chains of type IV collagen, spanning X-linked, autosomal recessive, and autosomal dominant variants.</li><li><strong>Clinical and Extrarenal Manifestations:</strong> Identifying the classic trio of kidney involvement, high-pitched sensorineural hearing loss, and ocular abnormalities like anterior lenticonus.</li><li><strong>Biopsy and Pathology Pearls:</strong> Differentiating between simple glomerular basement membrane (GBM) thinning and advanced longitudinal splitting ("basket-weave" lamination) on electron microscopy, alongside the utility of pediatric skin biopsies.</li><li><strong>Clinical Management & Pitfalls:</strong> Navigating first-line RAAS inhibition and SGLT2 inhibitors while strictly avoiding immunosuppressive regimens for secondary FSGS lesions.</li><li><strong>Transplantation Outcomes:</strong> Understanding why Alport syndrome does not recur post-transplant, balanced against the 3% to 4% risk of developing de novo anti-GBM disease in the allograft.</li></ul><p><strong>Recommended Literature:</strong></p><ul><li><a href="https://www.alportuk.org/wp-content/uploads/2014/03/Expert-Guidelines-JAN-2013.pdf">Consensus statement on guidelines for the care of children and adolescents with Alport syndrome.</a></li><li><a href="https://pubmed.ncbi.nlm.nih.gov/22166847/">Early angiotensin-converting enzyme inhibition in Alport syndrome delays renal failure and improves life expectancy.</a></li><li><a href="https://pubmed.ncbi.nlm.nih.gov/33094278/">Alport Syndrome: Classification and Management </a></li></ul>
April 29, 2026
Board Review Bonus 5: Renal Amyloidosis
<p><strong>Episode 5: Board Review Bonus: Renal Amyloidosis<br></strong><br></p><p>In this "Board Review Bonus" (BRB) episode, hosts Dr. Kenar Jhaveri and Dr. Koyal Jain provide a comprehensive clinical overview of amyloidosis—a complex and often enigmatic disease with significant renal implications. From the initial "mushy, smudgy" appearance on light microscopy to the precise measurements of electron microscopy, our hosts break down the essential knowledge required for both board preparation and clinical practice.</p><p>The discussion moves beyond the common AL and AA variants to explore rare types like LECT2 and the hereditary forms. Dr. Jain and Dr. Jhaveri emphasize that "the tissue is the issue," highlighting the diagnostic necessity of biopsying the affected organ and the critical role of mass spectrometry in directing therapy.</p><p><strong>Key Topics Covered:<br></strong><br></p><ul><li><strong>Classification of Amyloidosis:</strong> A detailed look at AL, AA, LECT2, and the hereditary variants including ATTR, fibrinogen A alpha-chain, and Apolipoprotein.</li><li><strong>The Diagnostic Workup:</strong> Understanding the utility of Congo red staining, the significance of birefringence, and the institutional variability of fat pad biopsies.</li><li><strong>Electron Microscopy (EM) Pearls:</strong> A guide to differentiating amyloid fibrils from mimics such as fibrillary GN, immunotactoid GN, and cryoglobulinemia based on diameter and arrangement.</li><li><strong>Clinical Management:</strong> Navigating the multidisciplinary approach between nephrology, hematology, and cardiology, including the nuances of RAAS inhibition and diuretic therapy in proteinuric patients.</li><li><strong>Transplantation Outcomes:</strong> Evaluating recurrence rates and survival benefits for different amyloid types post-kidney transplant.<p></p></li></ul><p><strong>Recommended Resources and Literature:</strong></p><ul><li><ul><li><a href="http://www.nephronpower.com/2021/06/concept-map-causes-of-renal-amyloidosis.html"><strong>Visual Aid:</strong> Concept Map: Causes of Renal Amyloidosis – A comprehensive resource for classifying amyloid variants and their underlying triggers (via NephronPower).</a></li><li><a href="https://pmc.ncbi.nlm.nih.gov/articles/PMC3487569/"><strong>Dandona P, et al.</strong> The amyloidoses: clinical features, diagnosis and treatment. Methodist Debakey Cardiovasc J. 2012 Jul-Sep;8(3):3-7. PMC3487569</a></li><li><a href="https://pmc.ncbi.nlm.nih.gov/articles/PMC12308146/"><strong>Panichella G, et al.</strong> Heart Failure Management in Cardiac Amyloidosis: Towards a Paradigm Shift. Heart Fail Rev. 2024. PMC12308146</a></li><li><a href="https://pubmed.ncbi.nlm.nih.gov/29254900/"><strong>Moreno-Martínez P, et al.</strong> LECT2-associated renal amyloidosis (ALECT2): A case report. Nefrologia. 2018 Jan-Feb;38(1):97-99. PMID: 29254900</a></li><li><a href="https://pubmed.ncbi.nlm.nih.gov/8097946/"><strong>Benson MD, et al.</strong> Hereditary renal amyloidosis associated with a mutant fibrinogen alpha-chain. Nat Genet. 1993 Mar;3(3):252-5. PMID: 8097946</a></li></ul></li></ul>
March 11, 2026
Episode 12: NephMadness Special! IgAN (Again?!): New B-Cell Targets vs. Complement Inhibitors
<p>It’s the "New England Journal of IgA" these days, and we’re just living in it! In this special NephMadness edition of GN in Ten, hosts Dr. Kenar Jhaveri and Dr. Koyal Jain are joined by NephMadness co-creator Dr. Matt Sparks and Duke Fellow Dr. Ale Tomasi to break down the heavy hitters in the IgA Nephropathy bracket. Matt’s dog also joins us for a special, possibly biased cameo.</p><p>We’re moving past "ACE first, think later" and diving into the upstream battle: B-cell modulators (BAFF/APRIL inhibitors) versus Complement inhibitors. Whether you’re team "Hit Zero" or team "Alternative Pathway," this episode covers the latest trial data from ORIGIN, VISIONARY, and APPLAUSE to help you fill out your bracket. </p><p><br></p><p><strong>The "Hit Zero" Hypothesis<br></strong><br></p><p>While we all know the classic four-hit hypothesis of IgAN, new therapies are targeting even further upstream—what some are calling "Hit Zero." </p><ul><li><strong>Pathophysiology Recap</strong>: IgAN starts with galactose-deficient IgA1 (Hit 1), leading to autoantibody production (Hit 2), immune complex formation (Hit 3), and mesangial deposition/damage (Hit 4). </li><li><strong>B-Cell Modulators</strong>: These drugs target <strong>BAFF</strong> (B-cell activating factor) and/or <strong>APRIL</strong> (a proliferation-inducing ligand) to reduce the production of those pesky autoantibodies right at the source. <p></p></li></ul><p><strong>The B-Cell Contenders: Sibeprenlimab & Atacicept<br></strong><br></p><ul><li><strong>Sibeprenlimab ("Sibi")</strong>: A monoclonal antibody directed at APRIL. <ul><li><strong>The Data</strong>: Showed a <strong>50% reduction in proteinuria</strong> at interim analysis and a nearly <strong>98% reduction in APRIL levels</strong>. </li><li><strong>Status</strong>: Currently has conditional FDA approval. </li></ul></li><li><strong>Atacicept</strong>: A fusion protein that dual-blocks both BAFF and APRIL. <ul><li><strong>The Data (ORIGIN trials)</strong>: Demonstrated a <strong>45.7% proteinuria reduction</strong> at 36 weeks and, notably, <strong>stabilization of eGFR slope</strong> in long-term follow-up. </li><li><strong>Pronunciation Debate</strong>: Is it "Attack-a-cept" or "A-tassi-cept"? The investigators say "Attack," because it’s out for blood. <p></p></li></ul></li></ul><p><strong>The Complement Contender: Iptacopan<br></strong><br></p><ul><li><strong>Mechanism</strong>: A factor B inhibitor that specifically targets the <strong>alternative complement pathway</strong>, which is increasingly recognized as a key driver of IgAN damage. </li><li><strong>The APPLAUSE Study</strong>: This oral, twice-daily pill showed a <strong>38% reduction in proteinuria</strong> at nine months. </li><li><strong>Safety First</strong>: Because complement blockade increases the risk of infections from encapsulated bacteria, patients must be up to date with vaccinations for meningitis, streptococcal and pneumococcal infections. <p></p></li></ul><p><strong>The Verdict: Who Wins the Bracket?<br></strong><br></p><ul><li>Tune in to find out what our experts think… then fill out your own bracket!<p></p></li></ul><p><strong>References<br></strong><br></p><p><strong>Atacicept<br></strong><br></p><p>Lafayette R, Barbour SJ, Brenner RM, Campbell KN, Doan T, Eren N, Floege J, Jha V, Kim BS, Liew A, Maes B, Pal A, Pecoits-Filho R, Phoon RKS, Rizk DV, Suzuki H, Tesař V, Trimarchi H, Wei X, Zhang H, Barratt J; ORIGIN Phase 3 Trial Investigators. <a href="https://pubmed.ncbi.nlm.nih.gov/41196369/">A Phase 3 Trial of Atacicept in Patients with IgA Nephropathy</a>. N Engl J Med. 2026 Feb 12;394(7):647-657. doi: 10.1056/NEJMoa2510198. Epub 2025 Nov 6. PMID: 41196369.</p><p>Lafayette R, Barbour S, Israni R, Wei X, Eren N, Floege J, Jha V, Kim SG, Maes B, Phoon RKS, Singh H, Tesař V, Lin CJF, Barratt J. <a href="https://pubmed.ncbi.nlm.nih.gov/38552841/">A phase 2b, randomized, double-blind, placebo-controlled, clinical trial of atacicept for treatment of IgA nephropathy.</a> Kidney Int. 2024 Jun;105(6):1306-1315. doi: 10.1016/j.kint.2024.03.012. Epub 2024 Mar 27. PMID: 38552841.</p><p><strong>Sibeprenlimab<br></strong><br></p><p>Perkovic V, Trimarchi H, Tesar V, Lafayette R, Wong MG, Barratt J, Suzuki Y, Liew A, Zhang H, Carroll K, Jha V, Quevedo A, Han SH, Praga M, Chacko B, Sahay M, Cheung CK, Kooienga L, Walsh M, Xia J, Fajardo C, Shah L, Hafkin J, Rizk DV; VISIONARY Trial Investigators Group. <a href="https://pubmed.ncbi.nlm.nih.gov/41211929/">Sibeprenlimab in IgA Nephropathy - Interim Analysis of a Phase 3 Trial.</a> N Engl J Med. 2026 Feb 12;394(7):635-646. doi: 10.1056/NEJMoa2512133. Epub 2025 Nov 8. PMID: 41211929.</p><p>Mathur M, Barratt J, Chacko B, Chan TM, Kooienga L, Oh KH, Sahay M, Suzuki Y, Wong MG, Yarbrough J, Xia J, Pereira BJG; ENVISION Trial Investigators Group. <a href="https://pubmed.ncbi.nlm.nih.gov/37916620/">A Phase 2 Trial of Sibeprenlimab in Patients with IgA Nephropathy.</a> N Engl J Med. 2024 Jan 4;390(1):20-31. doi: 10.1056/NEJMoa2305635. Epub 2023 Nov 2. PMID: 37916620; PMCID: PMC7615905.</p><p><strong>Iptacopan<br></strong><br></p><p>Perkovic V, Barratt J, Rovin B, Kashihara N, Maes B, Zhang H, Trimarchi H, Kollins D, Papachristofi O, Jacinto-Sanders S, Merkel T, Guerard N, Renfurm R, Hach T, Rizk DV; APPLAUSE-IgAN Investigators. <a href="https://pubmed.ncbi.nlm.nih.gov/39453772/">Alternative Complement Pathway Inhibition with Iptacopan in IgA Nephropathy.</a> N Engl J Med. 2025 Feb 6;392(6):531-543. doi: 10.1056/NEJMoa2410316. Epub 2024 Oct 25. PMID: 39453772.</p><p>Zhang H, Rizk DV, Perkovic V, Maes B, Kashihara N, Rovin B, Trimarchi H, Sprangers B, Meier M, Kollins D, Papachristofi O, Milojevic J, Junge G, Nidamarthy PK, Charney A, Barratt J. <a href="https://pubmed.ncbi.nlm.nih.gov/37914086/">Results of a randomized double-blind placebo-controlled Phase 2 study propose iptacopan as an alternative complement pathway inhibitor for IgA nephropathy.</a> Kidney Int. 2024 Jan;105(1):189-199. doi: 10.1016/j.kint.2023.09.027. Epub 2023 Oct 31. PMID: 37914086. </p><p><br>The hosts and guests of this GN in 10 episode do not have any disclosures to make relevant to the content of this episode.<br><br></p><p><br></p>
19 total episodes available
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