Autoinflammatory

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 https://t.co/431PQlzBsV
Dr. John Cush @RheumNow( View Tweet )
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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 )
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NHANES, Numbers, & Risk (7.18.2025)

Jack Cush, MD

Dr. Jack Cush reviews the news and journal reports from this past week on RheumNow.com along with interesting case questions from "Ask Cush Anything".

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French Teratology Service study of 57 pregnant women exposed to anti-IL-1 (47% for Stills Dz) found no evidence of adverse pregnancy outcomes with IL-1 inhibition (43 anakinra, 5 canakinumab) 85% livebirths, 11% early miscarriages. Major congenital malformations 3% https://t.co/iyLvaNKA2o
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 )
Martinque, Afro-Caribbean population (n 354 800) study(2004-2022) of 33 AOSD pts (71%F; 79% systemic; 21% chr articular); 91% met Yamaguchi criteria; features as expected but more Pulmonary Dz(51%; 94% w/ a systemic pattern). Half Dx < age 35 yrs. Prevalence 7.6 /100 000 mean https://t.co/QWyfNuvhkl
Dr. John Cush @RheumNow( View Tweet )
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Manifestations and Treatment in Relapsing Polychondritis

Jack Cush, MD

A large multicenter cohort study of Relapsing Polychondritis (RP) describes the clinical manifestations and treatment approaches to this rare, heterogeneous, multisystem disease

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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/ZjuOO08sKx https://t.co/rNVBFxgjZY

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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 )
Vexas

Overview of the VEXAS Syndrome

Jack Cush, MD

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

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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

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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 )
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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

Jack Cush, MD

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

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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

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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

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Nelly ZIADE 🍀 @Nellziade( View Tweet )
FMF #Recommendations #Colchicine can be fatal 🔺️Monitor regularly #EULAR2025 @RheumNow https://t.co/VJZYPaBrvE

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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

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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 )

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

Best of 2022: Myths & Misconceptions with Febrile Disorders

Rachel Tate, DO
Myth vs reality
Still’s disease and autoinflammatory disorders are rare conditions unified by recurrent fevers and inflammation of skin, joints, and other organs. While labs often support the diagnosis, these conditions are best diagnosed by genetic testing, or by specific criteria (in the absence of a genetic test). The following is a table that lists common misconceptions (myths) and their clarifying explanations. Read Post
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