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Rare Diseases

Overview of the VEXAS Syndrome A current systematic review in Rheumatology addresses the clinical features seen in the VEXAS syndrome. https://t.co/45qeSb14GB https://t.co/2mHEHh7BmU
Dr. John Cush @RheumNow( View Tweet )
EUSTAR Study of 893 SSc-ILD pts - 94 (10.5%) died (mean F/U 39 mos). Predictors of mortality: FVC deterioration >10% w/in 12 mos (HR 3.81) & >10% FVC decline or mRSS worsening (HR 2.82); but not changes in DLCO, mRSS or digital ulcer burden alone. https://t.co/28wSQc1dUS https://t.co/dourQ6WGDq
Dr. John Cush @RheumNow( View Tweet )
Study of 74 systemic sclerosis pts shows nailfold videocapillaroscopy (capillary disorganization, late pattern changes) predicts ILD progression, worsening PFTs & mRSS in SSc over 2 yrs. KL-6 correlates w/ NVC findings & PFT decline. ILD incr from 38 to 51% in 2yrs https://t.co/h2Js6JXl2H
Dr. John Cush @RheumNow( View Tweet )
Is the term polymyositis obsolete?.. now being replaced by dermatomyositis, necrotizing myopathy, inclusion body myositis, antisynthetase syndrome & overlap myositis. Full read overview of inflammatory myositis & new treatments from Joint Bone & Spine https://t.co/X46sptp1Ax https://t.co/QqyMv6fkVS
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
Dr. John Cush @RheumNow( View Tweet )

Overview of the VEXAS Syndrome

A current systematic review in Rheumatology addresses the clinical features seen in the VEXAS syndrome.

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BMJ - UK population study of 1.5 million adults finds pts w/ autoimmune dz have twice the risk of affective disorders (depression, bipolar dz, anxiety) significantly higher (p<0.001) (28.8% vs 17.9% in gen. population. Signif regardless of age, sex, ethnicity, income, Fam Hx, https://t.co/dsJxkvD4Nu
Dr. John Cush @RheumNow( View Tweet )
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
Dr. John Cush @RheumNow( View Tweet )
First FcRn inhibitor in #IIM: In #ALKIVIA, SC efgartigimod PH20 improved TIS vs placebo at 24wks (50.45 vs 35.65, p=0.0004), with faster time to TIS≥20 (30 vs 71.5d) & TIS≥40 (113d vs NE). More major responders too (TIS>60: 34% vs 9.5%). #EULAR2025 #OP0002 https://t.co/ulxtL1NX2Y
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
Dr. John Cush @RheumNow( View Tweet )
Rheumatologists can’t ignore hypereosinopilic syndrome anymore, not when it borders on our diseases and therapies. Great takeaways on subtypes and therapies from Giacomo Emmi in an elegant talk #EULAR2025 @RheumNow https://t.co/yzQVt1fLKW https://t.co/kiX6kPPQP5
Dr. John Cush @RheumNow( View Tweet )
A Review of Intracranial GCA A comprehensive review in Rheumatology on intracranial giant cell arteritis (icGCA) stresses that GCA is both an intracranial and extracranial large vessel vasculitis, with the former having unique presentations, and outcomes. https://t.co/NkN4gfcq3n
Dr. John Cush @RheumNow( View Tweet )
Full read overview of MDA-5 Dermatomyositis (DM) - pathogenesis, findings, & Rx. MDA-5 DM is a rare, aggressive amyopathic form with distinctive skin findings & rapidly progressive interstitial lung dz. Rx includes Hi dose steroids, calcineurin inhib & IV CTX (maybe JAKi, MMF)

Dr. John Cush @RheumNow( View Tweet )

PCR: Prevalence, Cost, & Risk (6.27.2025)

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

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A Review of Intracranial GCA

A comprehensive review in Rheumatology on intracranial giant cell arteritis (icGCA) stresses that GCA is both an intracranial and extracranial large vessel vasculitis, with the former having unique presentations, and outcomes.

A systematic review included 102

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Social Determinants of Health in SLE Dr. Mrinalini Dey interviews Dr. Elena Nikiphorou about the topic of social determinants of health in systemic lupus erythematosus, and data being presented at the EULAR 2025 meeting in Barcelona, Spain. https://t.co/gSFIV8uIuE https://t.co/VpqzVRSwh9
Dr. John Cush @RheumNow( View Tweet )
The Value of Kidney Biopsy in Autoimmune Diseases Dr. Andrea Fava shares his perspective from the EULAR 2025 meeting in Barcelona, Spain. https://t.co/CA6Ak2scKb https://t.co/EK5wdpbzrl
Dr. John Cush @RheumNow( View Tweet )
Predictors and Outcomes in Systemic Sclerosis The VEDOSS project has previously shown that 70% of patients with Raynaud’s phenomenon and at least one red flag – systemic sclerosis (SSc)-specific antibodies, puffy fingers, or an SSc pattern on nailfold capillaroscopy –will https://t.co/9dJn1qMQfy
Dr. John Cush @RheumNow( View Tweet )
Nailfold Capillaroscopy correlated w/ cardiac ECHO in Systemic Sclerosis. NFC & ECHOs studied in 30 SSc pts. Systolic & diastolic dysfunction most common in late SSc NFC patterns. Cardiac dysfunction seen w/ low capillary density, avascular areas, cap neoangiogenesis https://t.co/M6uU6i5cW4
Dr. John Cush @RheumNow( View Tweet )
#EULAR2025 Abstr#OP0202 Phase 1/2 of Resel-cel, a 4-1BB-containing human CD19-CAR T cell (weight-based 1 x infusion) reported efficacy in 8 patients (#SLE and LN) at WK28. 2 x Grade 1 CRS & 1 x Grade 4 ICANS. 1 x HLH, not attributed to SLE flare. Need longterm data @RheumNow https://t.co/COCKlqw687
Md Yuzaiful Md Yusof @Yuz6Yusof( View Tweet )
#WIN in systemic sclerosis (SSc): -Ongoing trials now targeting multiple pathways. -CONQUEST platform may reshape research in Ssc and other autoimmune diseases. -Revised CRISS could replace single organ endpoints for approval. #EULAR2025 @RheumNow https://t.co/kxSeFb5lAU
Adela Castro @AdelaCastro222( View Tweet )
What ‘CAR’ to drive? #CAR-T buzz was at #EULAR2025 In multiple #rheumatic #diseases #SLE #Scleroderma #Sjogrens #myositis Even #axial #SpA Who - active, not a lot of damage Which target 🎯 Which priming regiment What will results be compared to? Who will pay? @RheumNow

Janet Pope @Janetbirdope( View Tweet )

#CAR-T in active #scleroderma #diffuse open label @sclerodermaUM presented improvement in #nailfold #capillary patterns Suggesting #vascular recovery Hope is for lungs, skin and survival to improve but These are ▶️ Early days ?risk OP0338 #EULAR2025. @RheumNow @eular_org https://t.co/QhmO6gekXk
Janet Pope @Janetbirdope( View Tweet )
Despite the evolution of anti-synthetase antibodies, there’s a clear need for better ways to define the disease. Enter the ACR/EULAR CLASS initiative - classification criteria for anti-synthetase syndrome ⬇️ see the next tweet #EULAR2025 @RheumNow https://t.co/Z3rPOt1vlx
David Liew @drdavidliew( View Tweet )
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