AS
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renfamilia.bsky.social
AS
@renfamilia.bsky.social
160 followers 120 following 39 posts
Nephrologist. Hyderabad, India 🇮🇳
Firm believer in Poe’s law and it’s corollary. Student for life. GlomCon 2022| NSMC 2023.
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Reposted by AS
kidneying.bsky.social
💥 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

July 31, 2025 at 12:52 PM
Reposted by AS
kidneying.bsky.social
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
July 31, 2025 at 12:51 PM
Reposted by AS
kidneying.bsky.social
#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
July 31, 2025 at 12:17 PM
renfamilia.bsky.social
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
May 30, 2025 at 4:58 PM
renfamilia.bsky.social
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
May 30, 2025 at 4:57 PM
renfamilia.bsky.social
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
May 30, 2025 at 4:56 PM
renfamilia.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
May 30, 2025 at 4:55 PM
renfamilia.bsky.social
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
May 30, 2025 at 4:54 PM
renfamilia.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
May 30, 2025 at 4:53 PM
renfamilia.bsky.social
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
May 30, 2025 at 4:51 PM
renfamilia.bsky.social
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
May 30, 2025 at 4:50 PM
renfamilia.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
May 30, 2025 at 4:48 PM
renfamilia.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
May 30, 2025 at 4:47 PM
renfamilia.bsky.social
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
May 30, 2025 at 4:44 PM
renfamilia.bsky.social
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
May 30, 2025 at 4:44 PM
renfamilia.bsky.social
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
May 30, 2025 at 4:42 PM
renfamilia.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
May 30, 2025 at 4:39 PM
renfamilia.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
May 30, 2025 at 4:38 PM
renfamilia.bsky.social
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
May 30, 2025 at 4:37 PM
renfamilia.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
May 30, 2025 at 4:34 PM
renfamilia.bsky.social
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
May 30, 2025 at 4:33 PM
Reposted by AS
renfamilia.bsky.social
Clue! #ECNeph
May 30, 2025 at 4:28 PM
Reposted by AS
theisn.org
Let’s see what biopsy reveals…
#ECNeph
May 30, 2025 at 4:26 PM
renfamilia.bsky.social
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
May 30, 2025 at 4:32 PM
renfamilia.bsky.social
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
May 30, 2025 at 4:32 PM