Best of 2025: Overview of the VEXAS Syndrome
A current systematic review in Rheumatology addresses the clinical features seen in the VEXAS syndrome.
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Telling You Where to Go (12.19.2025)
Dr. Jack Cush reviews the news and journal articles from the past week on RheumNow.com. Who would've thunk - a PsA FMF connection? Labs to diagnose SSc-ILD? IgA-RF may be important or RZV may not work in JAK inhibitor treated patients?
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Disease Activity Criteria for Adult-Onset Still's Disease: EULAR Points to Consider
EULAR has published a "points to consider" (PtCs) guidance on the development of criteria for the assessment of the disease activity in adult-onset Still’s disease (AOSD).
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10 yr European retrospective study of haemophagocytic lymphohistiocytosis found 3345 deaths due HLH from 29 countries. Crude mortality rate = 5.3 per 10million PYs (range: 1.1-11.3). From 2011 to 2021, increasing from 3.9 to 6.6/10millPYs, varying by country, sex, age. https://t.co/58gCQ72saF
Dr. John Cush @RheumNow( View Tweet )
Treating Refractory Still's Disease
A full read systematic review article examined treatment options for difficult to manage systemic juvenile idiopathic arthritis, or Still’s disease. It classified these refractory disease patients as those with either (1) persistent https://t.co/uDeIkIek1d
Dr. John Cush @RheumNow( View Tweet )
Treating Refractory Still's Disease
A full read systematic review article examined treatment options for difficult to manage systemic juvenile idiopathic arthritis, or Still’s disease. It classified these refractory disease patients as those with either (1) persistent arthritis, (2) recurrent or difficult-to-treat
Organ Involvement with Behçet’s
A review of patient data from the International AutoInflammatory Disease Alliance (AIDA) Network registry identifying those with mucocutaneous Behçet’s disease (BD) may progres to major organ involvement (MOI)m especially at later stages. https://t.co/jqM4IZuobe
Dr. John Cush @RheumNow( View Tweet )
Vitamin D and Lupus Outcomes (11.21.2025)
Dr. Jack Cush reviews the news and journal reports from this past week on RheumNow.com.
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Organ Involvement with Behçet’s
A review of patient data from the International AutoInflammatory Disease Alliance (AIDA) Network registry identifying those with mucocutaneous Behçet’s disease (BD) may progres to major organ involvement (MOI)m especially at later stages.
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Retrospective review of 113 AOSD pts, median age 45yrs, ferritin 4626 (IQR 1169–14,239). Advanced Rx (IVIG, IL-1, IL-6) required in 15 (13.3%), NOT predicted by ferritin levels, either measured continuously or by quartiles. Ferritin is not a reliable biomarker in AOSD. https://t.co/pHEPeIf9u2
Dr. John Cush @RheumNow( View Tweet )
Robinson et al has published that Epstein-Barr virus infects and reprograms autoreactive B cells to drive the systemic autoimmune response in SLE https://t.co/vjpvILrJOt https://t.co/HepyZrYW5L
Dr. John Cush @RheumNow( View Tweet )
IL-1 Inhibition in CPPD
Controlled clinical trials in patients with calcium Pyrophosphate Deposition Disease (CPPD) are scant. Now a metanalysis provides evidence that anakinra, an interleukin-1 inhibitor, is a promising alternative for patients with acute CPPD flares.
The purpose of this analysis is
20-40% of Autoimmune hepatitis (AIH) is associated with other autoimmune diseases - lead by autoimmune thyroiditis, type 1 DM, ulcerative colitis, Crohn disease, & celiac (but less so for RA, PBC, SLE, Sjögren syndrome, vitiligo). AIH Dx Abs include ANA, ASMA, LKM1. https://t.co/I3ZPHiRgqZ
Dr. John Cush @RheumNow( View Tweet )
ACR 2025 Rheumatology Round Up
Drs. Jack Cush & Arthur Kavanaugh, two of rheumatology’s most trusted voices, provide a breakdown of the latest breakthroughs and hottest topics in rheumatology from the 2025 ACR Convergence meeting in Chicago.
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Hospitalization in Stills: Is it "Still" an infection?
Dr. Janet Pope discusses abstract 0163, "Trends in hospitalization and outcomes among patients admitted with Adult-onset Still’s disease in the United States: INSIGHTS FROM THE NATIONAL INPATIENT SAMPLE (2016-2022)," https://t.co/3Z0U9XN3nx
Dr. John Cush @RheumNow( View Tweet )
@CarolineDiorio @ChildrensPhila @RheumNow Indeed, there are a lot of familiar HLH/MAS friends in the post-CAR-T CRS mix...
(indeed, rheum friends from many places)
@CarolineDiorio @ChildrensPhila in the #ACR25 BCRC pre-meeting @RheumNow https://t.co/OzWu3MV1FA
Links:
David Liew @drdavidliew( View Tweet )
I've always (very) simplistically considered post-CAR-T CRS and HLH/MAS in the same bucket, and there certainly are substantial similarities.
It raises the obvious question - can we borrow therapies?
@CarolineDiorio @ChildrensPhila in the #ACR25 BCRC pre-meeting @RheumNow https://t.co/2HkHxpl89u
David Liew @drdavidliew( 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
Links:
David Liew @drdavidliew( View Tweet )
CAR-T Cell Therapies Show Promise for Autoimmune Disease
Two pioneering studies presented at #ACR25 spotlight the potential of CAR-T cell therapies to transform treatment for SLE and other autoimmune diseases. These early findings suggest a new frontier in immune modulation and https://t.co/KdNWmVc4Qc
Dr. John Cush @RheumNow( View Tweet )
kids w
👇
Undifferentiated #systemic #autoinflammatory #disease
Rx #IL1i
N=33
=boys &girls 3.5 yrs Sx
Abst#2151
#ACR25 @ACRheum @RheumNow
There are many periodic fever syndromes
🤔some mutations are not yet discovered
Access to &use of
Anakinra
canukinumab
Rilonacept https://t.co/IyTx0nFxKC
Janet Pope @Janetbirdope( 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 )
The Power of Gamma Delta T Cell for Autoimmunity https://t.co/wIMu5r7b3D
More on T Cell engagers , this time with gama delta T cell ( tiny but mighty ). Kate Roslin talks about advantages of this protocol. (No cytokines thus less toxicity).
@Yuz6Yusof
@RheumNow
#ACR25
Nouf Al hemmadi @NoufAhmedAlham2( View Tweet )
How does DADA2 present?
Ten studies provided data on a total 225 patients
1) skin findings
2) fever
3) stroke
4) cytopenias
@RheumNow #ACR25 Abst 1615 https://t.co/xZuTk6YFdJ
Brian Jaros, MD @Dr_Brian_MD( View Tweet )
When MAS is suspected. Soluble IL2 receptor alpha and CXCL9 may be useful (however t cell lymphomas and viral infections like hepatitis may also have them)
#ACR25 @RheumNow
Key takeaways below https://t.co/wJv1dvwU88
Bella Mehta @bella_mehta( View Tweet )
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