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ILD Begins (8.29.2025)
Dr. Jack Cush reviews the news and journal reports from RheumNow.com. This week news on vaccines, safety of acetaminophen and more.
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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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NHANES, Numbers, & Risk (7.18.2025)
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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Manifestations and Treatment in Relapsing Polychondritis
A large multicenter cohort study of Relapsing Polychondritis (RP) describes the clinical manifestations and treatment approaches to this rare, heterogeneous, multisystem disease.
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.
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Turkish study of Childhood-onset arthritis (n 1713; age 6.8 yrs). Most Dx: JIA 50% (most oligo), FMF 16% (recurrent oligo), vasculitis 14% (most IgA vasculitis; 94% oligo), reactive arthritis 9% (monarthritis) & acute rheumatic fever 5% (polyarticular& migratory), SLE 5% (oligo https://t.co/HkiPT0hsr7
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Elevated serum levels of interleukin-18 discriminate Still's disease from other autoinflammatory conditions. Sera from 53 mSAID, 220 guSAID, 49 controls show IL-18 signif increased in Still's Dz; free IL-18 levels were selectively higher in Still's disease. https://t.co/oAd3JwDR5D
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Advances in Still's disease Rx:
- using IL-1 or IL-6 inhibitors 1st line
- New FDA approval of emapalumab (IFNg) for MAS
- unclear if biologics can cause lung probs in Stills https://t.co/zSqTcOSVGE https://t.co/8GhoamkV4N
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Dermatomyositis Reviewed
Researchers from the University of Pennsylvania have published an open-access review of dermatomyositis (DM) - its manifestations, hallmark cutaneous features, etiopathogenesis and treatment options.
https://t.co/Dm85RL99UU https://t.co/SbaVBWC3Wv
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Advances in Still's disease Rx:
- using IL-1 or IL-6 inhibitors 1st line
- New FDA approval of emapalumab (IFNg) for MAS
- unclear if biologics can cause lung probs in Stills https://t.co/zSqTcOSVGE https://t.co/qefCt5b7qh
Dr. John Cush RheumNow ( View Tweet)
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
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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
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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
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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/IDeEvitIrB
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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
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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
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
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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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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)
