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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Hyperferritinemia is common in Hyperinflammatory syndromes but is a hallmark of Macrophage activation syndrome and Sepsis; and is a poor prognostic sign too. MAS is excessive activation of T-lymphocytes & macrophages leading to cytokine storm & multi-organ damage. https://t.co/MUKXC0Tw3M
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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/8IWXWZmWgE
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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
Dr. John Cush @RheumNow( View Tweet )
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
Dr. John Cush @RheumNow( View Tweet )
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
Dr. John Cush @RheumNow( View Tweet )
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
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 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 )
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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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 )
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
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 )
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
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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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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 )
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
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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
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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