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IgG4: Rare, but new treatments on the horizon

Janet Pope, MD, MPH, FRCPC

IgG4 is a rare autoimmune disease with excessive IgG4 immunoglobulins that can affect multiple organs yielding plasma cell deposition (inflammation) and fibrosis.

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Are we putting the CAR-T before the horse? CAR T-cells have been a revolutionary development in rheumatology. We have seen a population of patients with severe refractory autoimmune conditions almost overnight presented with the prospect of not just improvement, but of a cure. https://t.co/niPU5cAojP
Dr. John Cush RheumNow ( View Tweet)
This and other reports on palindromic rheumatism show that PR is a pre-clinical RA state, both having same predictive factors for progression to RA! https://t.co/jfpraGfe72

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Dr. John Cush RheumNow ( View Tweet)

If you missed this pattern, you missed an autoimmune classic What’s it? https://t.co/zO48zPCkqP

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DocXus docxusofficial ( View Tweet)

#ACR25 Sheds Light on Disparities, Age-Related Trends, and Epidemiology in Autoimmune Rheumatic Diseases At #ACR25, new research explored critical disparities and epidemiologic trends shaping outcomes in autoimmune rheumatic diseases across diverse populations and age groups. https://t.co/CPob0ppjv3

Dr. John Cush RheumNow ( View Tweet)
NEPTUNUS: Ianalumab in Sjogren's https://t.co/K9Xm51Kb5X Finally good news in Sjogrens disease. @RheumNow #ACR25

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Nouf Al hemmadi NoufAhmedAlham2 ( View Tweet)

Needle in a haystack What T cell to target for #autoimmune diseases T cell repetoires (TCR) not all are sequenced and many can exist to present antigens Diff scales of approaches Implications CAR-T CD19 CD20 BCMA Treg HLAB27 etc! #ACR25 @ACRheum @RheumNow Alok Joglekar #26S08 https://t.co/Qgw8Akz1xk

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Janet Pope Janetbirdope ( View Tweet)
What dictates how severe CRS is following CAR-T? (Often the critical question, and will especially be in the autoimmune space, where CAR-T is largely less time-critical) @CarolineDiorio @ChildrensPhila in the #ACR25 BCRC pre-meeting @RheumNow https://t.co/u0w4lx7YVn

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David Liew drdavidliew ( View Tweet)

Post-CAR-T myelopathy has been an issue in heme onc, and is still an unpredictable quantity without a clear cause. Will this be a problem for autoimmune CAR-T as we gain more experience? @CarolineDiorio @ChildrensPhila in the #ACR25 BCRC pre-meeting @RheumNow https://t.co/RhhdOiyEAX

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David Liew drdavidliew ( View Tweet)
Phase 1 study of CAR-Treg in difficult to treat RA 4/6 pts had 50% reduction in TJC/SJC, no CYC/fludrabine required & good prelim safety data CAR-T remains exciting, but less of the "totally magical everyone cured" stuff we saw w/initial CAR-T reports @RheumNow #ACR25 https://t.co/kjBIkLCYpF

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Mike Putman EBRheum ( View Tweet)
Sjögren’s: two Phase 3 RCTs (NEPTUNUS-1 [QM], -2 [Q3M]) test Ianalumab (BAFF/BAFF-R) vs placebo. ΔESSDAI at 48wk: Ianalumab QM > PBO, statistically significant But ΔESSDAI ≈ 0.5–1 pt (vs PBO) below MCII (≥ 3) Promising biology, modest clinical gain #ACR25 @RheumNow LB24 https://t.co/8y580ch9iJ

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Jiha Lee JihaRheum ( View Tweet)

Kohler et al. Phase 1 study autologous CAR-Tregs in refractory RA. No lymphodepletion needed! 6 participants. 2 doses, second cohort with higher dose better and more sustained response. No CRS or other toxicities. @RheumNow #ACR25 Abstr#LB023 https://t.co/nWva2R7WOQ

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Richard Conway RichardPAConway ( View Tweet)
The next VEXAS, but also maybe we can treat it? IDH1/2 Another clonal hematopoesis known to cause heme malignancy, but also some inflamm sequelae (autoinflamm-like e.g. PMR/GCA) & you can directly inhibit it Wow. We will hear more #ACR25 LB21 @RheumNow https://t.co/WL2ai5zb8Q https://t.co/Yi31xkhLO9

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David Liew drdavidliew ( View Tweet)
#ACR25 Abstr#LB21 Are they novel somatic variants beyond #VEXAS in driving inflammation in undiagnosed SAID?Unbiased enrichment analysis in 265 UBA1-negative adults identified IDH1/2 hotspot mutations. Cd be used to screen esp those with cytopenia. Sorry pix not allowed @RheumNow

Md Yuzaiful Md Yusof Yuz6Yusof ( View Tweet)

Dr. John Stone on IgG4RD ⬆️ common in males ⬆️internal organ involvement 🔸Mimics cancer 🔸Indolence makes it dangerous —-> organ complications Diagnosis highly dependent on the rheumatologist based on clinical features, serologies, imaging & pathological data #ACR25 @Rheumnow https://t.co/St4EbA7Dcj

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sheila RHEUMarampa ( View Tweet)
IgG4-RD continues to present in new and unusual ways Hasbani et al. report 9 IgG4-RD patients with coronary artery involvement RCA and LAD most affected Seen on CTA Majority received RTX 7/9 pt at least partially improved imaging on f/u @RheumNow #ACR25 Abst 2531 https://t.co/Na0gdq3dgb

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Brian Jaros, MD Dr_Brian_MD ( View Tweet)
Mimics of ANCA-associated vasculitis: How can we tell? With increased disease awareness and more regular, accessible testing for anti-neutrophil cytoplasmic antibodies (ANCA), ANCA-associated vasculitis (AAV) has become an increasingly recognized clinical entity in rheumatologic https://t.co/LYRk0rHlzx
Dr. John Cush RheumNow ( View Tweet)
“I never thought I’d live to old age with MCTD, now I plan for it.” This patient’s journey highlights the power of evolving therapies, resilience, and lifestyle adaptation to age well with chronic autoimmune disease. Patient perspectives abstract #PP04 @rheumnow #ACR25

Jiha Lee JihaRheum ( View Tweet)

Management of cardiac and joint sarcoidosis. Use of TNFi in treatment refractory cases. Alana Nevares-Berrios @RheumNow #ACR25 https://t.co/hXOfkgQHP9

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Antoni Chan MD (Prof) synovialjoints ( View Tweet)
Soluble IL2 receptors help assess macrophages behavior. Ferritin also asses the same in Macrophage activation syndrome. ADA2 is the newer biomarker for hemophagocytic syndromes. #ACR25 @RheumNow Interferon gamma also a newer important target in MAS https://t.co/7sWwninBTM

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Bella Mehta bella_mehta ( View Tweet)
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