Pediatric Rheum

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Emapalumab Approved for MAS in Still’s

Susan Shenoi, MD, MS

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

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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

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Dr. John Cush @RheumNow( View Tweet )
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Hmmm Really? (7.11.2025)

Jack Cush, MD

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

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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

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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

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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

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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

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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

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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

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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

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Nelly ZIADE 🍀 @Nellziade( View Tweet )
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MTX Fails Knee OA (6.6.2025)

Jack Cush, MD

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 )

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Autoimmunity Rising in Expectant Mothers

Jack Cush, MD

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 )
teen under covers looking anxious

How Living With Childhood-Onset Lupus Impacts Mental Health

Kaveh Ardalan, MD

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

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Janet Pope @Janetbirdope( View Tweet )

Recent Blogs

Best of 2022: Five Mistakes When Diagnosing Still’s Disease

Jack Cush, MD
Thermometer
Adult-onset Still's presents an interesting and diagnostic challenge when encountered. Here are 5 tips to improve your diagnostic acumen for this febrile disorder. Read Post

From the Archives: Rheumatology Dead Word Cemetery

Jack Cush, MD
CTD.jpg
I recently heard of a secondary school assignment wherein students were challenged to “bury” a word that was no longer useful or appropriate. Their exercise has now evolved into an unofficial RheumNow task force to retire diagnostic terms that have grown into misuse in rheumatology and medicine. How did we decide which words should perish? And by what criteria? Who has the final say? Read Post
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