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ICYMI: LAVLI - A New Autoinflammatory Disorder

Jack Cush, MD

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 inflammation, by altering microvascular permeability and neutrophil recruitment, while at the same time promoting hepatic fibrosis.

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EULAR 2023 Notes from Milan (6.09.2023)

Jack Cush, MD

Dr. Jack Cush reviews highlight abstracts and sessions - covering lupus, RA, gout, Still's disease, safety data, AI in medicine and more - from last week's EULAR 2023 Congress held in Milan, Italy.

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Post hoc analysis 2 phase III RCTs of abatacept (Sc & IV) in 409 polyarticular JIA pcJIA. ABA was effective (by JIA-ACR and JADAS27 responses) & well tolerated, regardless of concomitant or prior MTX use. https://t.co/cTn52V5Fuu https://t.co/CUqin00LZh

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Dr. John Cush RheumNow ( View Tweet)
Multicenter open label trial of 30 refract. Behcets (BD) pts treated w/ tocilizumab (TCZ) x 6 mos; TCZ was effective in 83% (18 complete & 7 partial resp). Complete responses seen w/ uveitis 67%, CNS 60%, mucosal 42%, articular 21%; plus steroid sparing https://t.co/PfrAoQxC2F https://t.co/ocAFv0Y2kr

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Dr. John Cush RheumNow ( View Tweet)
In Memoriam: Dr. K. Frank Austen (1928-2023) Rheumatology mourns the loss of a historic leader in the field, Dr. K. Frank Austen, who died at his home in Maine on June 23, 2023, at the age of 95. https://t.co/mtRQ8D5bWt https://t.co/fAQ9ebK95R

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Dr. John Cush RheumNow ( View Tweet)
RT @bella_mehta : 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 #EULAR2023

Dr. John Cush RheumNow ( View Tweet)

RT @bella_mehta : 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

Dr. John Cush RheumNow ( View Tweet)

Why Patients Don't Take Medicines This report examines the characteristics of adults aged 18–64 who took prescription medication in the past 12 months and did not take medication as prescribed due to cost. https://t.co/zuPgMjKYbh https://t.co/62cG9LDYWi

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Dr. John Cush RheumNow ( View Tweet)
Rheums! Have a rheumatology question or case for Jack Cush? Record it here and we may feature it on an upcoming podcast. Tell us your name and where you practice rheumatology. https://t.co/3atPdJwb1m https://t.co/MKbgzEi9pd

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Dr. John Cush RheumNow ( View Tweet)
Still's Disease Abstracts at EULAR 2023 https://t.co/1gNf8RUDLi https://t.co/1f1ERUOS03

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Dr. John Cush RheumNow ( View Tweet)
Still's Disease Abstracts at EULAR 2023 https://t.co/lb1QRcsa9G https://t.co/JIBszS7s7D

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Dr. John Cush RheumNow ( View Tweet)
EULAR/PRES recos for sJIA & AOSD 🔹sJIA = AOSD l 👉 Unique name: Still’s disease 🔹Start IL1i or IL6i as soon as diagnosis made 🔹MAS ⚠️ 🔹Emerging issue: lung disease 👉Screening (HRCT if suspicion) #EULAR2023 Pr B Fautrel & F deBenedetti Key slides 👇🏼 NZ https://t.co/x1Snf8WI99
ARD & RMD Open ARD_BMJ ( View Tweet)

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/bB2mpul3j7 https://t.co/ic6mY04ddF

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Dr. John Cush RheumNow ( View Tweet)
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

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Dr. John Cush RheumNow ( View Tweet)
@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
David Liew drdavidliew ( View Tweet)
@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
David Liew drdavidliew ( View Tweet)
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
Bella Mehta bella_mehta ( View Tweet)
#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
Md Yuzaiful Md Yusof Yuz6Yusof ( View Tweet)
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
Dr. John Cush RheumNow ( View Tweet)
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
Bella Mehta bella_mehta ( View Tweet)
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