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IgG4: Rare, but new treatments on the horizon
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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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
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
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
Links:
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
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
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
Links:
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
Bella Mehta bella_mehta ( View Tweet)
Low percentage of glycosylated Ferritin was more specific for Stills disease but not used frequently in clinical practice.
@RheumNow #ACR25 https://t.co/wTK1eIcSKX
Bella Mehta bella_mehta ( View Tweet)
Who talks to their neighbour?
+SSA / Ro talk differently to nearby cells in #SjD #salivary #glands vs NEG Ro SjD
Ro52 acts differently than Ro60
?implications for Rx response? 🤷♀️
#ACR25 @RheumNow @ACRheum #Sjogren’s
Abst#1684
#ACRBest!
Elegant work from Sara McCoy https://t.co/UWUKAxi0UW
Links:
Janet Pope Janetbirdope ( View Tweet)
Primary CNS vasculitis: what else causes vessel wall enhancement on dedicated brain MRI?
From Dr. Hajj-Ali https://t.co/v5ZtyezX3x
Brian Jaros, MD Dr_Brian_MD ( View Tweet)
Sun et al. Telitacicept in SARD-ILD (pSS, ASS, RA). 25 patients. Improvements in CT, PFTs, 6MWT. @RheumNow #ACR25 Abstr#1361 https://t.co/q679LOaOyL
Richard Conway RichardPAConway ( View Tweet)
Need better lymphoma biomarkers in Sjögren’s?
APRIL joins BAFF as a promising candidate—high APRIL levels linked to ↑ B-cell activation, disease activity & future lymphoma.
Easily measurable, potentially targetable.
@RheumNow #ACR25 Abstract #1683
Jiha Lee JihaRheum ( View Tweet)
In Sjögren’s, cancer risk is real.
>9yr multicenter cohort (n=314), overall cancer risk ↑ (SIR 1.68), NHL >15× expected
Predictors: older age, smoking, LAD, splenomegaly, cryoglobulinemia (strongest for lymphoma).
Malignancies = 24% of deaths
@RheumNow #ACR25 Abstract #1681
Jiha Lee JihaRheum ( View Tweet)
Who with #SSc-ILD will progress?
progression is non linear - can stabilize and worsen
Search for Predictive biomarker
For clinical care & enriching RCTs
Maybe #KL6 🤷♀️
Maybe IP-10, MIG
No strong #biomarker
Depends on what is used
#ACR25 @RheumNow @ACRheum
Abst#0882 #0883 https://t.co/lyTRUEY9SI
Links:
Janet Pope Janetbirdope ( View Tweet)
Pregnancy risk in Sjögren’s & scleroderma
Meta-analysis (n=4,820) shows ↑ risk of miscarriage in Sjögren’s (RR 1.8) & ↑ preterm birth in SSc (RR 2.4).
Calls for preconception counseling & multidisciplinary high-risk OB care.
@RheumNow #ACR25 Abstract #1398
Jiha Lee JihaRheum ( View Tweet)
Can tissue give us an underlying diagnosis in aortitis?
Dr. Kermani breaks down the autoimmune differential based on histopathology
@RheumNow #ACR25 https://t.co/twcH021dxS
Brian Jaros, MD Dr_Brian_MD ( View Tweet)
