Hmmm Really? (7.11.2025)
Dr. Jack Cush reviews the news and journal reports from this past week - some obvious, other new thoughts and some - hmmm, reallly?
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Overview of the VEXAS Syndrome
A current systematic review in Rheumatology addresses the clinical features seen in the VEXAS syndrome.
https://t.co/45qeSb14GB https://t.co/2mHEHh7BmU
Dr. John Cush @RheumNow( View Tweet )
Referral of 304 suspected autoinflammatory Dz (SAID) to a UK national centre for primarily adult SAID in 2022, 121 (39.5%) were diagnosed with SAID, w/ half of these having monogenic Dz. Median Dx delay was 2 to 6 yrs, worse in late-onset SAID. https://t.co/x8VOFyjuVA https://t.co/3R2HdYUlSg
Dr. John Cush @RheumNow( View Tweet )
Overview of the VEXAS Syndrome
A current systematic review in Rheumatology addresses the clinical features seen in the VEXAS syndrome.
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Japanese cohort study compared 12 pts w/ Stills Dz or intravascular lymphoma. Stills pt had significantly higher ferritin and IL-18 levels and IVL pts had significantly higher sIL-2R levels. https://t.co/Ecd6vNJTc4 https://t.co/KdEcpDeskA
Dr. John Cush @RheumNow( View Tweet )
FDA approves new drug for MAS in Still's disease. Gamifant (emapalumab-lzsg) is interferon gamma (IFNγ)–blocking Ab approved For HLH, now for use in adults & children w/ Still's Dz complicated by Macrophage Activation Syndrome. https://t.co/3div1fktWU https://t.co/VvSkuEoki5
Dr. John Cush @RheumNow( View Tweet )
Pregnancy outcomes in autoinflammatory disease
To address this, a French multi-centre prospective pregnancy observational cohort was set up, to analyse disease activity, treatment, pregnancy outcomes, delivery, and neonatal health. The work – presented at the 2025 annual https://t.co/oQ7A2bnDmO
Dr. John Cush @RheumNow( View Tweet )
Nurse-Led Treat-to-Target Clinics
EurekAlert!
New studies shared at the 2025 annual EULAR congress in Barcelona highlight the benefits that nurse-led care can bring for both patients and healthcare systems.
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EULAR 2025 – Day 4 Report
Saturday was the final day of EULAR, and included several lectures, but mostly late breaking abstracts and new EULAR guidelines - notably on Rheumatoid Arthritis and another on Interstitial Lung disease in connective tissue disorders. My favorite presentations included the following.
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Pregnancy outcomes in autoinflammatory disease
EurekAlert!
To address this, a French multi-centre prospective pregnancy observational cohort was set up, to analyse disease activity, treatment, pregnancy outcomes, delivery, and neonatal health. The work – presented at the 2025 annual congress of EULAR – showcased findings from 97 women with an
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EULAR FMF recommendations 2025:
still emphasis on colchicine adherence to maximal dose
+/- bDMARDs if needed for control
+/- csDMARDs for residual chronic MSK involvement
new: consider tapering bDMARD if remission >6mo
See the treatment algorithm below⬇️
#EULAR2025 @RheumNow https://t.co/Hy6DOKzfUL
Links:
David Liew @drdavidliew( View Tweet )
FMF #Recommendations
🔺️Start Colchicine in All (& continue for confirmed pts)
🔺️Single or divided dose, depend on adherence & tolerance
🔺️Max dose 2 mg children, 3 mg adults
🔺️if failure: add IL1i (6 RCT) (2 for IL6i)
🔺️Add c or bDMARd if MSK
#EULAR2025
@RheumNow https://t.co/fPyQrLbb6S
Nelly ZIADE 🍀 @Nellziade( View Tweet )
FMF #Recommendations
During attack:
🔺️No use of increasing Colchicine
🔺️No use for corticosteroids and opioid
🔺️Use NSAIDs
#EULAR2025
@RheumNow https://t.co/jJjWJra7yr
Nelly ZIADE 🍀 @Nellziade( View Tweet )
FMF #Recommendations
#Colchicine can be fatal
🔺️Monitor regularly
#EULAR2025
@RheumNow https://t.co/VJZYPaBrvE
Nelly ZIADE 🍀 @Nellziade( View Tweet )
FMF #Recommendations
#Colchicine can be fatal
🔺️Beware of drug interactions
🔺️Mostly statins in adults
🔺️Macrolides in children
#EULAR2025
@RheumNow https://t.co/SYGLpAJXFu
Nelly ZIADE 🍀 @Nellziade( View Tweet )
Procalcitonin can be used in the monitoring of infection, but is also high in Stills disease. 89 AOSD pts showed pts w high PCT had more pleuritis, MAS,^LFTs, & correlated w/ Dz activity, CRP, LDH, hi ferritin & normalized w bDMARD Rx. #EULAR2025 abstr #POS1118 https://t.co/NpRA9optd3
Dr. John Cush @RheumNow( View Tweet )
"H Syndrome" review of 23 French pts. Autoinflammatory dz due to variants of SLC29A3 gene. Multisystem histiocytic lymphoproliferative disorder of young adults- skin 2/3, sensorineural hearing loss, fever w/ endo & autoimmune dz. Best responses w IL6 inhib. #EULAR2025 Abst https://t.co/F7NGI4TkJS
Dr. John Cush @RheumNow( View Tweet )
The definition of MAS. MAS is secondary HLH. Other causes of secondary HLH are infections, malignancy and iatrogenic.
Georgin-Lavialle S @RheumNow #EULAR2025 https://t.co/CpB78JBlVJ
Links:
Antoni Chan MD (Prof) @synovialjoints( View Tweet )
Total serum IL 18 can help instead of CDC42 or NLCR4 genetic testing in #MAS
Several monogentic mutations in primary HLH- these panels can be available..
#EULAR2025 @RheumNow
Bella Mehta @bella_mehta( View Tweet )
Age of onset is really important when thinking about #MAS
Primary HLH more frequent in pediatric population. But can continue to adulthood.
#EULAR2025 @RheumNow https://t.co/J7xmTiQiwr
Bella Mehta @bella_mehta( View Tweet )
MAS can be triggered by infections in VEXAS syndrome. The threshold is reached leading to a hyperinflammatory state
Georgin-Lavialle S @rheumnow #EULAR2025 https://t.co/NKLL75rOgP
Links:
Antoni Chan MD (Prof) @synovialjoints( View Tweet )
Management of HLH/MAS and modes of action of advanced therapies
Jessica Manson @RheumNow #EULAR2025 https://t.co/Op3u53ZXeK
Links:
Antoni Chan MD (Prof) @synovialjoints( View Tweet )
Stabilize the pts with HLH with steroids when still trying to figure out the diagnosis of #HLH/ #MAS
Many drugs can be used...but important to balance infection risk with immunosuppression
These are critical pts. Be vigilant..
#EULAR2025 @RheumNow https://t.co/id4ae3SWtj
Links:
Bella Mehta @bella_mehta( View Tweet )
Closing the care divide in #JIA treatment plenary session
Overall theme in #EULAR2025 is everyone talking about AI and digital health on day 1
Biologics have changed the game!
@RheumNow https://t.co/qDqbz8JApe
Bella Mehta @bella_mehta( View Tweet )
Treatment of MAS, or the bête noire in Still's disease, presented at the Plenary session by Peter Nigrovic
#Plenary room
#EULAR2025
@RheumNow https://t.co/S4SG2VflNg
Nelly ZIADE 🍀 @Nellziade( View Tweet )
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