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Pediatric Rheum

Emapalumab Approved for MAS in Still’s

On June 27th, 2025, the FDA approved emapalumab for macrophage activation syndrome (MAS) in known or suspected Still’s disease (including both adult-onset Still’s disease (AOSD) and systemic juvenile idiopathic arthritis (sJIA)) with inadequate response or intolerance to glucocorticoids.

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Study of a Chinese systemic JIA cohort using scRNA-seq and Bulk RNA-seq, found a potential biomarker w. UBE2D1 expression closely related to Dz activity levels, reflecting enhanced monocyte activity in sJIA patients. UBE2D1 may complement others S100A8/A9, IL-18, ferritin https://t.co/hp7sFR6kLa
Dr. John Cush @RheumNow( View Tweet )
Metanalysis of Uveitis & JIA - 28 studies, 22,834 JIA pts w/ 3,381dx w/ uveitis - prevalence = 12.7%,, highest in Europeans (14.3%), lowest in Asian (6.5%). Uveitis risk factors included early age JIA onset, ANA-positive, and increased ESR. https://t.co/MxffJKvbik https://t.co/LDz60uwQTM
Dr. John Cush @RheumNow( View Tweet )
Study of a Chinese systemic JIA cohort using scRNA-seq and Bulk RNA-seq, found a potential biomarker w. UBE2D1 expression closely related to Dz activity levels, reflecting enhanced monocyte activity in sJIA patients. UBE2D1 may complement others S100A8/A9, IL-18, ferritin https://t.co/IDeEvitIrB
Dr. John Cush @RheumNow( View Tweet )
Don't Stop the DMARDS!! Retrospective study of 91 JIA pts (all types; 36% oligo, 27%poly) all Rx w/ cycles of DMARD therapy. Consistently two-thirds relapsed and flared. Only one-third of JIA patients were able to remain off DMARDs. https://t.co/ifqnMKfhAe https://t.co/8JAbpO1IVe
Dr. John Cush @RheumNow( View Tweet )

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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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 )
Don't Stop the DMARDS!! Retrospective study of 91 JIA pts (all types; 36% oligo, 27%poly) all Rx w/ cycles of DMARD therapy. Consistently two-thirds relapsed and flared. Only one-third of JIA patients were able to remain off DMARDs. https://t.co/ifqnMKfhAe https://t.co/bEqAxzRoq6
Dr. John Cush @RheumNow( View Tweet )
FDA has approved a SC autoinjector of Benlysta (BEL) for use in pediatric lupus nephritis & pts > 5rs of age for SLE and active LN. BEL SC dosing for Pediatric SLE: 200 mg qwk (>40 kg) or 200 mg EOW (q2wk) if 15-40 kg. https://t.co/zrskHdw9Jx https://t.co/jkqygmfJPv
Dr. John Cush @RheumNow( View Tweet )
FDA has approved a SC autoinjector of Benlysta (BEL) for use in pediatric lupus nephritis & pts > 5rs of age for SLE and active LN. BEL SC dosing for Pediatric SLE: 200 mg qwk (>40 kg) or 200 mg EOW (q2wk) if 15-40 kg. https://t.co/xrMJUgdSWv https://t.co/UJ2LZ3dU5p
Dr. John Cush @RheumNow( 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 )
🚨 Type I IFN in Pediatric SLE: Expanded Insights! •🩺 IFN-I Impact: Fuels pediatric SLE; drives T-cell autoreactivity, autoantibody production, proinflammatory cytokines. •📈 Key Pathways: Plasmacytoid dendritic cells overproduce IFN-I via cGAS-STING-TBK1, TLR4/TLR9; https://t.co/4i3MktOoVd
Dr.Mukesh , MD , DM @dr_immuno29( 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 )
Young Patients’ AREA empowers teens/young adults w/ autoimmune disease to shape research. US-based, youth-led & grounded in empowerment, they co-design studies, train peers & advise researchers. A truly positive and meaningful initiative esp for PPIE @RheumNow #EULAR2025 #OP0008

Mrinalini Dey @DrMiniDey( View Tweet )

Muscle biopsies form 3 treatment-naïve juvenile dermatomyositis (JDM) finds transcriptomic evidence of mitochondrial dysfunction consistently in JDM muscle, independent of interferon-driven inflammation. Treating mitochondrial dysfunction may improve JDM outcomes https://t.co/u5tMhT23si
Dr. John Cush @RheumNow( 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 )

MTX Fails Knee OA (6.6.2025)

Dr. Jack Cush reviews the news and journal reports from this past week on RheumNow.com

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Single-centre retrospec study of 39 Juvenile Dermatomyositis pts Rx w/ JAKi. Partial/complete Clin inactive Dz achieved in 32/39 w/in 6 months w/ steroid reduction and W/D of other DMARDs. Only TIF1γ) Ab (+) was assoc w/ nonresponse to JAKi (P < 0.001). 5 w/ H. Zoster

Dr. John Cush @RheumNow( View Tweet )

Blau syndrome -a rare autoinflammatory Dz w/ triad of granulomatous dermatitis, arthritis, & uveitis (may cause blindness); from a NOD2 gain-of-function mutations. Onset age 2-4 yrs. Early biologics (esp TNFi) may prevent joint complications; but metanalysis shows no preferred https://t.co/IIJbJ7x2WB
Dr. John Cush @RheumNow( View Tweet )
Single-centre retrospec study of 39 Juvenile Dermatomyositis pts Rx w/ JAKi. Partial/complete Clin inactive Dz achieved in 32/39 w/in 6 months w/ steroid reduction and W/D of other DMARDs. Only TIF1γ) Ab (+) was assoc w/ nonresponse to JAKi (P < 0.001). 5 w/ H. Zoster

Dr. John Cush @RheumNow( View Tweet )

Autoimmunity Rising in Expectant Mothers

Lancet Rheumatology has published a UK study showing a significant increased in the number of expectant mothers beginning their pregnancies with autoimmune conditions.

The MuM-PreDiCT project run by the University of Birmingham, analyzed the electronic healthcare records from

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How Living With Childhood-Onset Lupus Impacts Mental Health Children, teens, and young adults with childhood-onset systemic lupus erythematosus (cSLE) require intense immunosuppressive treatments to prevent organ damage. Recently, the mental health burden for cSLE patients has https://t.co/JXkdYiAO9o
Dr. John Cush @RheumNow( View Tweet )

How Living With Childhood-Onset Lupus Impacts Mental Health

Children, teens, and young adults living with childhood-onset systemic lupus erythematosus (cSLE) require intense immunosuppressive treatments to prevent serious organ damage. By the mid-2010’s, multiple studies were published that showed high rates of depression, anxiety, and suicidal

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🍋 or ‘Lyme’ - is it Lyme disease? Dr Elizabeth Stringer discusses Eastern Canada 🇨🇦 Epidemic of #Lyme #arthritis Which mimics #pauciarticular #JIA When it is #chronic Work up of all oligoarthritis in endemic areas in kids Includes Validated Lyme test @ORAexec #ORA25 https://t.co/WxU1vaOa7z
Janet Pope @Janetbirdope( View Tweet )
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