#ECNeph @brammahin @Dilushiwijay @myadla @acssjr @dra_miliflores pubmed.ncbi.nlm.nih.... pubmed.ncbi.nlm.nih....#ECNeph ☄️ More than few reported cases exist

ℹ️Turns out patient has hypogammaglobulinemia ❓MMF related/disease related Ig A - 134 mg/dl ( 70-400) ⬇️Ig G - 669 mg/dL (700-1600) ⬇️⬇️Ig M - 38 mg/dL (40-230) 😏Does it explain all? #ECNeph @brammahin @Dilushiwijay @myadla @acssjr @dra_miliflores

#ECNeph @brammahin @myadla @Dilushiwijay @acssjr @dra_miliflores @Dilushiwijay @ISNkidneycare Admitted with febrile illness Flare vs infection Any other DD ? Thoughts plz ! Mild rise in creatinine Kidney Bx : Now Class III LN

#ECNeph @brammahin @myadla @ISNkidneycare @Dilushiwijay @acssjr @dra_miliflores Class switching in LN Bomback AS. Nonproliferative Forms of Lupus Nephritis: An Overview. Rheum Dis Clin North Am. 2018 Nov;44(4):561-569

Let start the discussion! 23 YO women with Lupus and recurrent admissions #ECNeph @kidneying.bsky.social @myadla.bsky.social @dilushiwijay.bsky.social @dramiliflores.bsky.social

Hi #ECNeph Dr Manjusha From India Eager to learn

#ECNeph @brammahin @myadla @Dilushiwijay @acssjr @dra_miliflores @Dilushiwijay @ISNkidneycare Is the diagnosis obvious ? Should we check labs? ANA+VE/low C3/hemat /Musculo skeletal involvement /Kidney Biopsy done in v/o proteinuria : Class 1 Can it be podocytopathy

Thank you for a good case prsentation @karnephro.bsky.social #ECNeph @dilushiwijay.bsky.social @theisn.org

Recent publication from CKJ @karnephro.bsky.social #ECNeph @dilushiwijay.bsky.social @theisn.org

⚡️PLA2R antibody seropositivity doesn’t mean that we do not investigate for underlying malignancies. Here is a Case Report showing PLA2rAb MN having Carcinoma Oesophagus #ECNeph @theisn.org @myadla.bsky.social @dramiliflores.bsky.social @arvindcanchi.bsky.social

KDIGO recommends Population and Age-appropriate Screening for malignancies irrespective of antigen status #ECNeph @theisn.org @myadla.bsky.social @dramiliflores.bsky.social @arvindcanchi.bsky.social

Yes, some tests came after biopsy #ECNEPh

@karnephro.bsky.social #ECNeph @dilushiwijay.bsky.social @theisn.org

Check the distribution of solid malignancies in NS @karnephro.bsky.social #ECNeph @dilushiwijay.bsky.social @theisn.org

Differentiating between PMN and SMN @karnephro.bsky.social #ECNeph @dilushiwijay.bsky.social @theisn.org

Final Diagnosis #ECNeph @dramiliflores.bsky.social @karnephro.bsky.social @myadla.bsky.social @dilushiwijay.bsky.social

👉 🫣 Can IF differentiate from Primary and Secondary MN? IgG 4 is the dominant class in primary MN 👉 IgG1,IgG2, IgG3 are dominant Immunoglobulins in Secondary MN #ECNeph @theisn.org @myadla.bsky.social @dramiliflores.bsky.social @arvindcanchi.bsky.social

Paraneoplastic glomerulopathies @karnephro.bsky.social #ECNeph @dilushiwijay.bsky.social @theisn.org

Some more info about PNS ! @karnephro.bsky.social #ECNeph @dilushiwijay.bsky.social @theisn.org

@karnephro.bsky.social #ECNeph @dilushiwijay.bsky.social @theisn.org Paraneoplastic glomerulopathy can occur before /sfter or simultaneously with the diagnosis of MN Need for evaluation of cases of MN for underlying malignancy is suggested

Primary vs Secondary Membranous Nephropathy #ECNeph http://springer.com/...#ECNeph@dramiliflores.bsky.social @karnephro.bsky.social

🔬 The characteristic histopathologic features of MN, light microscopy, IF, EM, are falling into place. So… where are we in this case? Granular IgG, GBM thickening, no crescents... What do you think? #ECNeph @dramiliflores.bsky.social @karnephro.bsky.social www.ajkd.org/action/...

A positive anti-PLA2R antibody test is “virtually” diagnostic of a lesion of MN (but may not always be able to reliably distinguish primary and secondary forms) #ECNeph #MedSky #NephSky @kdjhaveri.bsky.social @dramiliflores.bsky.social @karnephro.bsky.social

Different types of tumor secrete different types of antigen. Here is the proposed mechanism of malignancy associated MN. #ECneph @dramiliflores.bsky.social @myadla.bsky.social @theisn.org www.frontiersin.org/journals/med...

Great infographic about NS in elderly @karnephro.bsky.social #ECNeph @dilushiwijay.bsky.social @theisn.org

Genetic testing for elderly ? Any lit supporting this ? @karnephro.bsky.social #ECNeph @dilushiwijay.bsky.social @theisn.org

Elderly with massive proteinuria Membranous nephropthy Amyloidosis Dn if (pt is DM) FSGS #ECNeph @karnephro.bsky.social #ECNeph @dilushiwijay.bsky.social @theisn.org

@karnephro.bsky.social #ECNeph @dilushiwijay.bsky.social @theisn.org Searching for clues .....

Thankyou @karnephro.bsky.social for sharing case details #ECNeph @dilushiwijay.bsky.social @theisn.org

Hello Shubharthi ,From Sylhet, Bangladesh .Thank you @theisn.org for selecting our case from #Bangladesh No COI #ECneph

Please introduce yourself, tell us where you're joining from, and don’t forget to use #ECNeph! 💬 Let’s keep learning together, let´s join the case chat now! 🎓🩺

Hi @myadla.bsky.social joining from India No COI #ECNeph

🎙️ Milagros Flores, @dramiliflores.bsky.social 🇭🇳 🇲🇽 I’m your host today #ECNeph No COI. On the other side @karnephro.bsky.social 🙌 Let’s get started! #MedSky #NephSky @theisn.org Are you ready to learn, discuss, and share your thoughts?💥

⏳ 60 min to go! 👣 Let’s walk through proteinuria findings 🧪🌳 🗓️ Join today for our #ECNeph Case Discussion 🕛 30th June 2025 at 12 PM EST @dramiliflores.bsky.social @karnephro.bsky.social Are you ready? #MedSky #NephSky

Dont miss Join us in 20 min #ECNeph @theisn.org @dilushiwijay.bsky.social

🧪 Proteinuria: Not a Walk in the Park 👣 Could this be more than just "nephrotic syndrome"? 🕵️ 🗓️ Join us #ECNeph Chat on Bluesky 🕛 30th June 2025, 12 PM EST @dramiliflores.bsky.social @karnephro.bsky.social @theisn.org #NephSky #MedSky @theisn.org

Hi this is Dilushi, joining a bit late! And lurking tonight #ECNeph

15/n Diagnostic algorithm for IgG4-RKD Diagnosis = Multimodal 🧠 High suspicion in AIN w/ low complement 🔬 Biopsy: Storiform fibrosis, IgG4+ PCs >10/hpf 🧪 Supportive labs & imaging 📊 Diagnostic criteria evolving (2011 → 2020 updates) #ECNeph @myadla.bsky.social @dramiliflores.bsky.social

18/n IgG4-TIN Treatment Case Series 🩺 📄https://pubmed.ncbi.nlm.nih.gov/21719792/ 📉 Treatment Response: Most patients responded dramatically to corticosteroids #ECNeph @myadla.bsky.social @dramiliflores.bsky.social @theisn.org

Let’s follow this clinical case that @renfamilia.bsky.social , @dramiliflores.bsky.social and @myadla.bsky.social prepared for us! Follow the 🧵

20/n MITIGATE trial- Inebilizumab 💊 Steroid sparing effect, ↓flare  🛡️ Immunosuppression for relapsers 💉 Inebilizumab – 1st FDA-approved drug for IgG4-RD 💰 Costly (~$150k/30 mL) 📖 https://www.nejm.org/doi/full/10.1056/NEJMoa2409712 #ECNeph @myadla.bsky.social @dramiliflores.bsky.social @theisn.org

Glad to have discussed the case for #ECNeph. Thank you all for your support and guidance! @myadla.bsky.social @dramiliflores.bsky.social @dilushiwijay.bsky.social @theisn.org 🙌

23/n Conclusion 🔚 Once an elusive mystery, IgG4-RD was hard to spot and often misdiagnosed. 🌊 But decades of research have turned the tide. ✨ Today, IgG4-RD stands in the spotlight—defined, diagnosable, and treatable. #ECNeph @myadla.bsky.social @dramiliflores.bsky.social  @theisn.org

17/n IgG4-RD-Pathophysiology and Novel Targets 🎯B-T cell collaboration seems to be central to the pathophysiology of IgG4-RD at different levels #ECNeph @myadla.bsky.social @dramiliflores.bsky.social @dilushiwijay.bsky.social  @theisn.org

pmc.ncbi.nlm.nih.gov/articles/PMC... From AIIMS Delhi #ECNeph

www.nature.com/articles/s41... #ECNeph

16/n Diagnostic criteria for IgG4-TIN 🔍 🔬 Histopath: >10 IgG4+ plasma cells/hpf, TBM immune deposits 🖼️ Imaging: Enlarged kidneys, cortical nodules, wedge/diffuse lesions 🧪 Serology: Elevated serum IgG4 or total IgG 🫀 Systemic clues #ECNeph @myadla.bsky.social @dramiliflores.bsky.social

14/n IgG4-RKD: Imaging features 🖼️ USG/CT: Enlarged kidneys, hypodense lesions 🎯 FDG-PET/Ga Scans: Hot spots 📌 May mimic tumors or infections 🧊 Retroperitoneal fibrosis, renal pelvic thickening #ECNeph @myadla.bsky.social @dramiliflores.bsky.social @theisn.org

13/n IgG4-RD: Biomarkers Traditional and Novel 📌 Serum IgG4, IgE, eosinophils → disease activity & relapse risk 📉 Fall with treatment, ↑ may signal flare 🧬 Plasmablasts = sensitive marker 🧪 C3/C4↓, ESR/CRP↑ in active disease #ECNeph @myadla.bsky.social @dramiliflores.bsky.social @theisn.org