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Autoinflammatory

AOSD: Young vs. Elderly Onset AOSDis an autoinflammatory condition characterized by fevers, arthritis, and rash. It is considered an orphan disease because of its low prevalence - it is reported anywhere between 16 to 40 per ten million. https://t.co/PPI1fvwLAf https://t.co/qMXMsbpwl3
@eular_org recommendations session #EULAR2023 - and the first recommendation is one I’m so glad is up front: Adult Onset Still’s Disease (AOSD) and Systemic JIA (sJIA) are the same disease. If anything, it’s the really young sJIA (<18 months old) which is different @RheumNow https://t.co/OCDeeNtNoR
@eular_org recommendation for AOSD/sJIA: An IL-1 or IL-6 inhibitor should be initiated as early as possible when the diagnosis is established #EULAR2023 @RheumNow https://t.co/xNUdpGDxb9
Microvascular visualization in Behcet's disease by ultrasound. Maybe really useful in cases which are difficult to diagnose and may not need biopsies in some pts. #EULAR2023 @RheumNow https://t.co/PbC3tCe7v6
#EULAR2023 Clinical Highlights. Vasculitis: OP0061 - new US imaging showing vessel inflammation differentiating patients with Behcet’s vs No-BD. OP0304: cohort study showed Benralizumab (IL5 RA) given for eosinophilic asthma also improved BVAS. RCT for EGPA is ongoing @RheumNow https://t.co/7uohfjGk49
Tune it at 1pm EST for RheumNow's daily #EULAR23 recaps. You can find us live on Twitter, YouTube and LinkedIn. https://t.co/t531csjXo8
Long term data on Canakinumab - IL-1B antibody in #AOSD @EULAR2023 25 pts with persistent disease activity, f/u 12 mths, remission in all but 2 pts. Improved parameters as below :- @rheumnow abst#OP0257 https://t.co/7jKTp87FlX
Obesity in #AOSD – predicted the development of a chronic disease course and biologic DMARD failure, 139 pts with AOSD- 19% Obese (BMI>30) observational study. abst#AB1503 #EULAR2023 @rheumnow
Pearls in AOSD – from #EULAR2023 Italian study of 64 patients – AOSD/sJIA pts who achieved drug-free remission more often had white blood cells count >15.000/uL at baseline, however those who were treated with cyclosporine likely failed drug free remission. Abst#AB1484 @rheumnow

AOSD: Young vs. Elderly Onset

Adult onset still's disease (AOSD) is an autoinflammatory condition characterized by fevers, arthritis, and rash. It is considered an orphan disease because of its low prevalence - it is reported anywhere between 16 to 40 per ten million. Traditionally, a bimodal distribution by age has been

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An interesting biomarker in #sJIA- helps differentiate it from other diseases, including infections and other inflammatory diseases! - kids with fever! serum calprotectin (MRP8/14) particle enhanced immuno-turbidimeteic assay (sCAL Turbi). 650 pts @rheumnow #EULAR2023 abst#OP0034
Review of the etiology of Polyserositis (14 pts, 23 articles): most common - neoplasia (26.3%; Lung Lymphoma; ovarian; pancreatic), autoimmune (16.7% - SLE, AOSD) & infections (12.3%; coxackie, CMV, mTB). https://t.co/Bubd99ETgu https://t.co/VbiPB0y7pv
Proposed AOSD criteria: 80 AOSD vs 60 autoimmune pts: \Nneed 7 points = AOSD dx (sensitivity 92%, specificity 93%, AUC 93%): - typical rash 3 pts - fever ≥ 39°C 3 pts - pharyngitis 2 pts - arthritis 2 pts - glycosylated ferritin≤ 20% 1 pt https://t.co/iLUryBVa7C https://t.co/HTWgAucBIm
One in Ten has Autoimmune Disease A UK population-based study suggests that autoimmune diseases affect approximately one in ten individuals. https://t.co/a2VbAO0Fc6 https://t.co/69Yl9OTtKY

Orphan drugs target rare dz <200k. New drugs (2008-2016) compared 5 yr sales 86 orphan Rx to 232 non-orphan = similar sales- Orphan ($719 mill) vs nonorphan ($812 mill). But orphan Rx lower volume offset by higher prices (7X higher) than nonorphan drugs https://t.co/I8kb4A0eyG https://t.co/gEQpssRVkg

Have a Rheumatology question or case for Jack Cush? Record it here and we'll feature it on an upcoming podcast. Tell us your name and where you practice rheumatology. https://t.co/0mC7PxF0DU https://t.co/klbnwjcPbM
Join us 4/25 as we conclude our Women in Rheumatology campaign by focusing on what's next. Our panelists will recap the challenges addressed this month and discuss a strategy to suppot women in rheumatology. https://t.co/DP4S2hf5Tz @uptoTate @KDAO2011 @gwenesta_melton https://t.co/rcglaZxqoF
Join us April 18 for: Private Practice & Academia In this session, our panelists will address the big questions of: "How do I choose between private practice and academia?" and "How do I advance in my career?". https://t.co/BrWG57mRRG https://t.co/A0pzEvJ3cu

Anakinra Phase 2/3 study in severe hosp. COVID-19 pts (5/20-3/21) w/ pneumonia & hyperinflammation (incr levels IL-6, ferritin, CRP, LDH). 179 pt Rx w/ ANA or SOC - d15 need for Mech ventilation was 77% ANAK v 86% SoC (NS; RR 0.90; 0.77-1.04) https://t.co/CFcI1qTsU2 https://t.co/wAj4pHN5Dh

Drs. Tate and Dao will be leading discussions on Women in Rheumatology, the XX Factor. Sign up to attend our Tuesday Night Rheumatology series. @uptoTate @KDAO2011 https://t.co/ciEAY1SJdg https://t.co/dlCfXItAxN
Tomorrow! Join us for Tuesday Night Rheumatology. Our panelists will discuss the challenges they have faced trying to manage a career and family life, as well as provide advice and strategies for managing competing priorities and obligations. https://t.co/OlP0pupkMV https://t.co/pZQMASafcG
Open-label Trial of 14 pts with MAS due to steroid refractory systemic JIA or adult-onset Still’s demonstrated the efficacy of IV emapalumab, an anti-IFNγ mAb. Demonstrated signif reductions in LDH, sIL-2R, Ferritin, CXCL9, & steroid dose https://t.co/vgxT5KzmQF https://t.co/gDLmfOlSHG

LAVLI - A New Autoinflammatory Disorder NIH researchers have have described a novel autoinflammatory disorder called "Lyn kinase-associated vasculopathy and liver fibrosis" (LAVLI), based on a mutation in the LYN gene. https://t.co/RYmb3QFB1T https://t.co/sn5hwVxwPk

LAVLI - A New Autoinflammatory Disorder NIH researchers have have described a novel autoinflammatory disorder called "Lyn kinase-associated vasculopathy and liver fibrosis" (LAVLI), based on a mutation in the LYN gene. https://t.co/VWs0YuF4LD https://t.co/7ydA3bb82j

LAVLI - A New Autoinflammatory Disorder

Mar 28, 2023

NIH researchers have have described a novel autoinflammatory disorder called "Lyn kinase-associated vasculopathy and liver fibrosis" (LAVLI), based on a mutation in the LYN gene (that encodes the Lyn kinase protein). They discovered that increased Lyn kinase activity promotes systemic

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