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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 manjusha yadla Milagros Flores Dilushi Wijayaratne Fong Voon Ken Int Society of Nephrology

13/n IgG4-RD: Biomarkers 📌 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 manjusha yadla Milagros Flores Int Society of Nephrology

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 manjusha yadla Milagros Flores Dilushi Wijayaratne Fong Voon Ken Int Society of Nephrology

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 Int Society of Nephrology

18/n Int Society of Nephrology manjusha yadla AS Dilushi Wijayaratne #ECNeph What are the clinical manifestations of IgG4 RD? Check this excellent infographic by Fong Voon Ken

19/n Int Society of Nephrology manjusha yadla AS Dilushi Wijayaratne Fong Voon Ken #ECNeph One of the imp modifiable risk factor for IgG4RD : Smoking Elderly age vulnerability Male dominance relapsing/remitting course

16/n Diagnostic criteria for IgG4-TIN 🔍 🔬 Histology: >10 IgG4+ plasma cells/hpf, TBM immune deposits 🖼️ Imaging: Enlarged kidneys, cortical nodules 🧪 Serology: Elevated serum IgG4 or total IgG 🫀 Systemic clues #ECNeph manjusha yadla Int Society of Nephrology

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 manjusha yadla Milagros Flores Dilushi Wijayaratne Fong Voon Ken Int Society of Nephrology

20/n Int Society of Nephrology manjusha yadla AS Dilushi Wijayaratne Fong Voon Ken #ECNeph What are the types of IgG4RD ?

18/n IgG4-TIN Treatment Case Series 🩺 📄pubmed.ncbi.nlm.nih.gov/21719792/ 📉 Treatment Response: Most patients responded dramatically to corticosteroids #ECNeph manjusha yadla Milagros Flores Dilushi Wijayaratne Fong Voon Ken Int Society of Nephrology

21/n Int Society of Nephrology manjusha yadla AS Dilushi Wijayaratne Fong Voon Ken #ECNeph How to approach IgG4RD ? Check the tissue diagnosis findings

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 manjusha yadla Milagros Flores Int Society of Nephrology

22/n Int Society of Nephrology manjusha yadla AS Dilushi Wijayaratne #ECNeph Beautiful infographic by Fong Voon Ken

20/n MITIGATE trial- Inebilizumab 💊 Excellent steroid response early 🛡️ Immunosuppression for relapsers 💉 Inebilizumab – 1st FDA-approved drug for IgG4-RD 💰 Costly (~$150k/30 mL) 📖nejm.org/doi/full/10.10… #ECNeph manjusha yadla Milagros Flores Dilushi Wijayaratne Fong Voon Ken Int Society of Nephrology

23/n Int Society of Nephrology manjusha yadla AS Dilushi Wijayaratne Fong Voon Ken #ECNeph Lets check indications for urgent Rx in IgG4RD What are various Rx regimens ? Kidney involvement with TIN - indication for Rx !

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 manjusha yadla Milagros Flores Dilushi Wijayaratne Fong Voon Ken Int Society of Nephrology

24/n Int Society of Nephrology manjusha yadla AS Dilushi Wijayaratne Fong Voon Ken #ECNeph Various diagnostic criteria for diagnosis of IgG4RD 1.Revised Comprehensive diagnostic criteria

25/n Int Society of Nephrology manjusha yadla AS Dilushi Wijayaratne Fong Voon Ken #ECNeph Diagnostic criteria by JSN for the diagnosis of IgG4RD

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 manjusha yadla Milagros Flores Dilushi Wijayaratne Fong Voon Ken Int Society of Nephrology

23/n Conclusion 🔚 Once an elusive mystery, IgG4-RD was hard to spot and misdiagnosed 🌊 Decades of research have turned the tide ✨ Today, IgG4-RD stands in the spotlight—defined, diagnosable, and treatable #ECNeph manjusha yadla Milagros Flores Dilushi Wijayaratne Fong Voon Ken Int Society of Nephrology