Another fantastic milestone for rare glomerular diseases! What an impressive momentum in kidney therapeutics and the rare disease community
#raredisease #glomerulardisease #nephSky #FSGS
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#GLOMERULARDISEASE
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Take-home from WCN26:
IgAN treatment is shifting from nonspecific immunosuppression to pathway-directed therapy.
The debate is no longer whether to treat, but where in the pathway to intervene first. 🧵 (10)
#GlomerularDisease
#ISNWCN
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Future IgAN management may involve combination or sequential therapy:
• Gut-targeted immunomodulation
• Complement inhibition
• Possibly BAFF/APRIL blockade
Precision treatment for a heterogeneous disease. 🧵 (9)
#GlomerularDisease
#ISNWCN
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🔑 clinical question:
Should therapy first target the cause of IgAN
or the mechanism of kidney injury?
This remains unresolved and likely depends on patient phenotype. 🧵(8)
#GlomerularDisease
#ISNWCN
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However, mechanisms differ.
Nefecon → modifies disease origin (gut immune system)
Complement inhibitors → block final inflammatory pathway in kidney
Upstream control vs downstream protection. 🧵 (7)
#GlomerularDisease
#ISNWCN
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In APPLAUSE-IgAN:
Iptacopan reduced the risk of the composite kidney failure endpoint by 43% vs placebo
(HR ≈0.57).
Strong signal for complement blockade in IgAN progression. 🧵 (6)
#GlomerularDisease
#ISNWCN
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Clinical trials show both strategies slow disease progression.
NefIgArd (Nefecon):
✔ Reduced proteinuria
✔ Slower eGFR decline
APPLAUSE-IgAN (iptacopan):
✔ Reduced proteinuria
✔ Lower risk of kidney failure events
🧵 (5)
#GlomerularDisease
#ISNWCN
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Complement inhibitors such as Iptacopan:
• Block the alternative complement pathway
• Reduce inflammatory injury in the glomerulus
They act downstream, after immune complexes reach the kidney. 🧵 (4)
#GlomerularDisease
#ISNWCN
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Targeted-release budesonide (Nefecon):
• Acts in the terminal ileum
• Suppresses mucosal immune activation
• Reduces production of pathogenic Gd-IgA1
In short: it targets the upstream driver of disease. 🧵 (3)
#GlomerularDisease
#ISNWCN
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IgAN pathogenesis follows a multi-hit model:
1️⃣ Mucosal immune dysregulation → galactose-deficient IgA1
2️⃣ Autoantibody formation
3️⃣ Immune complex formation
4️⃣ Glomerular deposition + complement activation
Therapies target different steps. 🧵 (2)
#GlomerularDisease
#ISNWCN
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♨️ Debate at WCN26:
Should complement inhibition be considered before targeted-release budesonide in patients with IgAN at risk of progression?
A key discussion between upstream vs downstream treatment strategies in IgAN. 🧵(1)
#ISNWCN
#GlomerularDisease
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#ISNWCN “KDIGO IgAN Session” discussing so interesting cases and perspectives 🇺🇸🇨🇳🇯🇵
🔎Effect of therapies on GFR loss and proteinuria
🎯Novel agents
👉🏻Disease activity and clinical background are important to determine appropriate therapy
#GlomerularDisease #KidneyHealthMatters 🌎🫘🌸
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“Understanding and Treating Complement-Mediated Glomerular Disease” #ISNWCN #ISGD
✅KPMP: right treatment, right person, right time
🧬Haplotype and Disease Penetrance- Genetic testing
❌C5b-9: more severe disease
🖊️C5a in AAV - complement therapies
🎯”RESET into homeostasis” #GlomerularDisease
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🌟 Enjoying our #WCN2026 coverage?
A heartfelt thank you to our incredible ISGD social media ambassadors, Dr. @gabyneph.bksy.social and Dr. Belén Gallegos, for bringing us the latest updates and insights in glomerular disease and keeping our community informed and engaged 🙌
#ISGD #GlomerularDisease
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At WCN’26, Tobias Huber highlighted the mission of the ISGD: uniting a 🌎 community to connect, educate, and advance research to improve care for patients with #GlomerularDisease
With 1,700+ members across 102 countries, #ISGD is building a 🌎 network for innovation and collaboration in 🫘 disease
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Heather Reich ISGD Chair of the Membership & Accreditation Committee, is present at the WCN’26
🤩 A great session about exploring the role of APRIL in IgAN
👉🏻 A great discussion of clinical scenarios
#ISGD
#GlomerularDisease
#ISNWCN
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Amazing! Paola Romagnani #ISGD #ISNWCN with “Podocytopathies: Knowns and Unknowns”
👉🏻Podocyte loss and regeneration
👀Autoimmune, genetic, adaptive, infectious, toxic, monoclonal
🎯Anti-slit autoantibodies-> ⬇️ prognosis
#GlomerularDisease #Yokohama 🌸
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Our ISGD president Tobias Huber is present at the WCN’26 🌸
👉🏻 His first session of the day: Kidney tissue-specific gene targeting
🦠 Adenovirus as a potential vector for gene targeting therapies
#ISNWCN
#GlomerularDisease
#ISGD
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So interesting! “Cutting Edge Science in Nephrotic Syndrome/FSGS” #Yokohama #ISNWCN
*Autoimmune Diseases treatments
*B cell depletion - How broad?
*Prognosis given by antibodies
*Anti-human and anti-mouse CD39 antibodies
Amazing treatment options #GLOMERULARDISEASE
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"Finally in #Yokohama for 'Renal Pathology: Native Kidney Disease Pathology.' Covering IgG4-related disease, parasitic glomerular lesions, and genomics in Undeterminate Kidney Disease. Follow for more updates. #Nephrology #GlomerularDisease #Genomics
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An interactive, thought-provoking session at the Primer Course on Glomerular Diseases, Yokohama—learning from the best in the field.
@jonathanbarratt.bsky.social @adrianliew.bsky.social @heatherreich.bsky.social @suceenaalex.bsky.social @hernantrimarchi.bsky.social
#GlomerularDisease #WCN2026
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Proteinuria is never “just protein in the urine.”
Strengthen your approach to evaluation, risk stratification, and first line management of glomerular disease.
📚 bit.ly/4r5CHV8
#Nephrology #KidneyCare #GlomerularDisease #RenalClinicians #CME #NephFellows
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Episode 2 of The Bean Pod is live 🎙️
Pearls in managing Steroid-Resistant Nephrotic Syndrome with Drs. Jason Misurac & Amirtha Chinnadurai, joined by Dr. Michael Somers.
Listen here: buff.ly/OZwJQBe
#PedsNeph #Nephrology #SRNS #GlomerularDisease #ASPN
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Glomerular disease remains complex and rapidly evolving.
Stay current on nephrotic syndrome, proteinuria evaluation, & emerging diagnostic and treatment strategies.
bit.ly/4r5CHV8
#Nephrology #GlomerularDisease #CME #MedEd #KidneyCare #Renal #NephroticSyndrome
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Nephrotic syndrome genomic discovery in the Mass General Brigham Biobank identifies monoallelic MEFV variants as a risk factor for focal segmental glomerulosclerosis
doi.org/10.1016/j.kint.2025.12.013
#MedSky #NephSky #glomerulardisease @bostonchildrens.bsky.social @brighamandwomens.bsky.social
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Early diagnosis matters in C3 glomerulopathy. This #CME activity explores symptom recognition, complement pathways, and emerging therapies.
👉 Watch now: bit.ly/48TbwXO
#MedicalEducation #Nephrology #KidneyDisease #RareDisease #C3G #GlomerularDisease
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Dr. Carla Nester discussed 52-week data on pegcetacoplan for #C3G demonstrating sustained proteinuria reduction (and remission in a third of patients). #nephrology #nephsky #glomerulardisease #kidneywk www.docwirenews.com/post/52-week...
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ASN has shown us the incredible advances being made in glomerular diseases and the opportunities we have to continue improving glomerular science, medicine, and patient care! Join the movement at www.is-gd.org/en/join-isgd
#ISGD #GlomerularDisease #ASNKidneyWeek2025 @tobias-b-huber.bsky.social
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ISGD had an impactful week at ASN 2025. Big Shout out to Matthias Kretzler ISGD Steering Committee Member and ASN Program Co-Chair, for his leadership in shaping the conference, and for keeping GD at the forefront
#ISGD #ASN2025 #GlomerularDisease #KidneyWeek2025 @tobias-b-huber.bsky.social
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📢 New Glomerular Diseases Guideline out now!
🧒 Up-to-date recommendations on diagnosing & treating immune-mediated #GlomerularDisease in children.
💊 Guidance on immunosuppression & supportive care.
✅ Endorsed by @BAPNnephrology
📃: ow.ly/HtZL50XnTCL
#KidneyDisease
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