💥 Could this be MAS in SLE? We are seeing it more often, 🗝️ - Clues in clinical practice Fever Pancytopenia Organomegaly ⬇️ ESR with disease activity #ECNeph @brammahin @Dilushiwijay @myadla @acssjr @dra_miliflores

Differentiating fever from flare in lupus #ECNeph @brammahin @myadla @Dilushiwijay @dra_miliflores @acssjr NLR CRP/ESR ratio <2/2-15/>15 : <2 s/o infection

#ECNeph @brammahin @myadla @dra_miliflores @acssjr @Dilushiwijay Lets check criteria of Lupus podocytopathy Lupus Podocytopathy: An Overview. Adv Chronic Kidney Dis. 2019 Sep;26(5):369-375

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

22/n Take home points 🏠 📌 IgG4-RDs are rare, protean, and often underdiagnosed 👀 High clinical suspicion is key—especially in AIN + low complements 💊 Excellent steroid response in early inflammatory phase #ECNeph @myadla.bsky.social @dramiliflores.bsky.social @theisn.org

21/n Inebilizumab vs Rituximab 📌 Target: CD19 vs CD20 🧬 B-cell range: Inebilizumab depletes plasmablasts too 💰 Cost: Inebilizumab $$$ > Rituximab $$ 🧪 Use: Rituximab still widely used off-label #ECNeph @myadla.bsky.social @dramiliflores.bsky.social

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

19/n IgG4-RD Emerging therapies 💡 🎯 B-cell targeting: CD20+: Rituximab, Ofatumumab;                                     CD19+: Inebilizumab (targets plasmablasts!) 🎯 Plasma cell targeting 🎯 Proteasome inhibition 🎯 Anti-fibrotic: Simtuzumab #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

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

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

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

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

12/n IgG4-RKD: Laboratory features 🧪 IgG4↑ (50–70%) 🧬 IgG4:IgG >10%, IgG4:IgG1 >24% 🔻 C3/C4 ↓ (50–70%) 🧬 ANA, RF can be + 🔬Plasmablasts, eosinophilia common #ECNeph @myadla.bsky.social @dramiliflores.bsky.social @theisn.org

11/n Clinical Features of IgG4-RKD 🩺 👨‍⚕️ Male: 75–85% 📆 Avg age: ~65 years 🧪 AKI or progressive CKD 💧 Proteinuria/hematuria (~50%) 🦵 Edema ⚡ Flank/back/abdominal pain 🌡️ Mild systemic symptoms 🧬 Multi-organ IgG4-RD 📖Mayo clinic cohort ⬇️ #ECNeph @myadla.bsky.social @dramiliflores.bsky.social

10/n IgG4-Related Kidney Disease (IgG4-RKD) 🩺🫘 🔹 Retroperitoneal fibrosis (RPF): Seen in ~1/3 of IgG4-RD cases 🔹 Direct renal involvement: < 25%   • 80% = Tubulointerstitial nephritis (TIN)   • 15% = Membranous nephropathy (MN) 📖UK Cohort ⬇️ #ECNeph @myadla.bsky.social @dramiliflores.bsky.social

9/n Let us explore the mimics of IgG4-RD 🧐 🕵️‍♀️ Some clues: 🔹 Lacrimal/salivary glands: Frequent in IgG4-RD 🔹 Pancreas, kidneys: Involved in IgG4-RD 🔹 TIN > MN in IgG4 renal disease 🧬 Mimicry is real—histopathology is key! #ECNeph @myadla.bsky.social @theisn.org

8/n 2020 Revised Diagnostic Criteria for IgG4-RD 🧬 📌 3 Key Pillars: 1️⃣ Clinical & radiologic findings 2️⃣ Serum IgG4 > 135 mg/dL 3️⃣ Pathology (2/3: IgG4+ PCs, storiform fibrosis, obliterative phlebitis) ✅ Definite = All 3 present #ECNeph @myadla.bsky.social

7/n IgG4-RD Clinical Phenotypes: One Disease, Many Faces 🎭🧬 📖https://www.bmj.com/content/369/bmj.m1067 #ECNeph @myadla.bsky.social @dramiliflores.bsky.social @dilushiwijay.bsky.social @kajareeg.bsky.social @theisn.org

6/n What unites the spectrum of IgG4-RD? 🔍 A distinct fibroinflammatory signature: 💥 Tumefactive lesions 🪨 Dense lymphoplasmacytic infiltrate 🔬 IgG4+ plasma cell predominance 🌪 Storiform fibrosis 🩸 Obliterative phlebitis 📈↑ serum IgG4 #ECNeph @myadla.bsky.social @dramiliflores.bsky.social

Clue! #ECNeph

Let’s see what biopsy reveals… #ECNeph

5/n Let’s talk about IgG4-Related Disease (IgG4-RD) 💬 🧭 Where did it all begin? 🤔 📌 2001 NEJM: High serum IgG4 in patients with sclerosing pancreatitis 🧾 Since then, many conditions have been reclassified under IgG4-RD #ECNeph @myadla.bsky.social @dramiliflores.bsky.social @theisn.org

4/n Serum Creatinine Trend 📉 Here’s the Cr trajectory post-biopsy — and it’s all good news! 🙌 🗓️ From a peak of 9.2 mg/dL, Cr steadily declined 📉 🔁 Ongoing improvement, no rebound! #ECNeph @myadla.bsky.social @dramiliflores.bsky.social @theisn.org

3/n Steroids did their magic! ✨ 🧊 IgG4-driven inflammation melted away 📉 Creatinine improved to baseline by 2 months 🔽 Steroids tapered 💊 Mycophenolate(MMF) started as steroid-sparing agent at 1 g/day #ECNeph @myadla.bsky.social @dramiliflores.bsky.social @dilushiwijay.bsky.social @theisn.org

⚠️ Persistent abnormal ratios (>3.1 or <0.26) in CKD still flag potential monoclonal gammopathy #ECNEph www.nature.com/articles/s41...

2/n Timeline of events post renal biopsy ⌛ 🧪Serum IgG4 > 4.78 g/L (0.03-2.01) (478 mg/dl) 💊Oral steroid 40mg/d (0.6 mg/kg/d) 🦠 Complication of Blood Stream infection 📉Serum creatinine improving trend ❌HD discontinued #ECNeph @myadla.bsky.social @dramiliflores.bsky.social @theisn.org

1/n Our patient had IgG4 tubulo-interstitial nephritis (IgG4-TIN). 🏥🔬 Marked IF/TA (over 70%). Would you initiate immunosuppression in this scenario? 🤔💭 Drop your answers below! 👇 #ECNeph @myadla.bsky.social @dramiliflores.bsky.social @theisn.org

Low PTH in CKD ✅ Often linked to adynamic bone disease (ABD) ✅ Or to malnutrition-inflammation cachexia syndrome (MICS) ✅ Low PTH → impaired bone turnover, ↑ mortality risk ⚠️ MICS: driven by inflammation, protein-energy wasting & chronic illness #ECNeph pmc.ncbi.nlm.nih.gov...

AKI sensitivity on CKD patients: ✅ Reduced renal reserve ✅ Chronic inflammation & fibrosis ✅ Altered microvascular response ✅ Impaired tubular repair ✅ Enhanced susceptibility to nephrotoxins @renfamilia.bsky.social @dramiliflores.bsky.social @myadla.bsky.social #ECNeph pmc.ncbi.nlm.nih.gov...#ECNeph

Let’s evaluate differentials of hypercalcemia in this case. Any other potential diagnoses on your mind? @renfamilia.bsky.social @dramiliflores.bsky.social @myadla.bsky.social #ECNeph

Welcome to #ECNeph! Looking forward to the insights & learning! #NephSky #MedSky @myadla.bsky.social @dramiliflores.bsky.social @theisn.org

Starts in 20 min ! Eager to engage for an exciting discussion ? Join us with #ECNeph Share your thoughts @theisn.org @kajareeg.bsky.social @divyaveerssneph.bsky.social @dilushiwijay.bsky.social

⏳ One hour to go… ✨ What’s the renal enigma that will challenge us today? #ECNeph 🩺 Dive in and share your insights! Use #ECNeph to stay in the loop. @dramiliflores.bsky.social @myadla.bsky.social @renfamilia.bsky.social @theisn.org

Folks ! we are back for our monthly session of #ECNeph Join us tomorrow at 12 PM EST with @dramiliflores.bsky.social @renfamilia.bsky.social for an exciting case discussion on BS Happening TODAY 9:30 PM IST on X @theisn.org @kajareeg.bsky.social @dilushiwijay.bsky.social

Major Trials in the use of ASIs targeting difficult-to-control hypertension #NephJC

As data emerges, the 3 new agents are much more selective than prior drugs. Still need more data on Vicadrostat (done but not shown in publications). #nephJC

When identifying hypertension, the reading is everything. Are we measuring it correctly? #NephJC.

What is the “ideal” blood pressure that minimizes cardiovascular and renal adverse events worldwide? Still an issue #NephJC http://www.nephjc.co...

Core Curriculum by Charmaine E. Lok, Theodore You, and Timmy Lee: Hemodialysis Vascular Access: Core Curriculum 2025 bit.ly/4ir8vAF (FREE)

ISN members: Apply to ISN’s expert-led primer courses in GN in Prague and Taipei – free registration and travel grants available ➡️ lite.spr.ly/6007GpZJ

Hello everyone. Anitha here. Joining from Hyderabad, India. #ECNeph

💥 March isn’t over yet—#NephMadness is in full swing! Vote, debate, advocate. Any bets on who’s making it to the Final Four? #NephJC ajkdblog.org/2025/03/01/w...

Check out the awesome #NephJC summary by @brianrifkin.bsky.social 🔗 www.nephjc.com/news/adpkd-k...

Algorithm for use of autoantibodies in Lupus See dx.doi.org/10.1016/j.au... 👇👇👇 #ECNeph

Hello everyone. Anitha here. Joining from Hyderabad, India #ECNeph

Happening Today on Bluesky ! We invite all #Nephbluesky friends to join us for #ECNeph interesting case discussion at 9:30 PM IST @myadla.bsky.social @arunkumarnephro.bsky.social @nihalbashir.bsky.social @kajareeg.bsky.social