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Autoinflammatory

Pyoderma gangrenosum review in BMJ - an ulcerative neutrophilic autoinflammatory disorder; often associated with underlying systemic disease - IBD, seronegative RA, hematological malignancies or monoclonal gammopathies, etc. https://t.co/AGorrWNfAQ https://t.co/hawksCEHRA
Prospective cohort study of 41 Stills Dz pts w/ ILD - 85% had 1+MAS & 41% with adverse reactions to a biologic. 93% were alive w/ median F/U 3 years, but 37% progressed to chronic lung dz (O2, or vent) & 84% were HLA-DRB1*15 https://t.co/fo44fgKh8m https://t.co/xa9g4Slr1H

FDA Approves Canakinumab for Gout Flares

Medscape
Aug 30, 2023

The US Food and Drug Administration (FDA) has approved canakinumab (Ilaris) for the treatment of gout flares in adults who cannot be treated with NSAIDs, colchicine, or repeated courses of corticosteroids. The drug is also indicated for people who could not tolerate or had an inadequate response

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Japanese study of 98 pts w/ VEXAS syndrome [81M: 8F; age 69.3 yrs) found 45% (40pts) with pathogenic UBA1 variants. VEXAS Dx Score based on: age >50 yrs, skin lesions, lung Sxs, chondritis, macrocytic anaemia (AUC=0.908) https://t.co/W2bSmuGf6l https://t.co/EwHuRk4GCr
Cytokine comparison of Kikuchi-Fujimoto disease (KFD) & Stills dz (pts w/ FUO and LN) shows, after excluding HLH/MAS pts, KFD pts had higher serum IFN-γ (> 8.56 pg/mL) & IFN-γ/IL-6 ratio > 0.45. Stills pts had higher serum IL-6 levels than KFD https://t.co/KYPa9OQaa5 https://t.co/70S0M9nGhp

Mono- and Oligo- are Different (8.18.2023)

Aug 18, 2023

Dr. Jack Cush reviews the news and journal reports from RheumNow.com this past week.




  1. Glucocorticoid Affects Weight, but not Blood Pressure in RA A pooled analysis of 5 RCTs shows that the effects of 2 years of low-dose glucocorticoid (GC) treatment in RA can significantly

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10% of a Single center cohort (n 38) Systemic JIA pts developed MAS. Only1 had recurrent MAS(3 times), mostly Male (3M:1F) w/ mean Dx age=8 ± 6 yrs. Stills w/ MAS had less arthritis (51 v 91%), more Rash (75 v 55%), HSM (50 v 5%), serositis (25 v 5%) https://t.co/QgdSJW03wj https://t.co/L5MTnmgWDn
Patient Perspectives on Telemedicine Use During the Pandemic Research from the COVID-19 Global Rheumatology Alliance included surveys and data gathering from rheumatology patients. https://t.co/EJ5TkiDMcJ https://t.co/xBx7OIxf9h

EULAR/ACR Guidance on Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome

Jul 26, 2023

A EULAR/American College of Rheumatology task force has established evidence based, up-to-date guidance and expert opinion on the evaluation, management and monitoring of patients with Haemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS), with the primary intent to 

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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/vGt12ul6iy https://t.co/044BScmSYc
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
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
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
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
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

ICYMI: LAVLI - A New Autoinflammatory Disorder

Jul 06, 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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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
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
Still's Disease Abstracts at EULAR 2023 https://t.co/1gNf8RUDLi https://t.co/1f1ERUOS03
Still's Disease Abstracts at EULAR 2023 https://t.co/lb1QRcsa9G https://t.co/JIBszS7s7D

EULAR 2023 Notes from Milan (6.09.2023)

Jun 09, 2023

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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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

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

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
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