All News
Hemophagocytic Lymphohistiocytosis in Still's Disease
Dr. Bella Mehta discusses abstract 1143 presented at the 2023 ACR Convergence meeting in San Diego, CA.
https://t.co/bFeGK6MTcc https://t.co/cxJg6rQ7gm
Nov 24, 2023
New Biomarkers and Therapeutics Show Potential in Still's Disease
AOSD is a rare complex, sporadic, systemic autoinflammatory disease similar to sJIA characterized by sustained fever, salmon-colored rash, and arthritis
https://t.co/cGBBHsBdK7 https://t.co/a0EwBiTJSu
Nov 17, 2023
Here at Last: Treatment Options for VEXAS
Nov 15, 2023
We have known about the VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome for nearly 3 years, but there has been relatively little to say about how to treat it. To date there have been over twice as many publications about VEXAS (263 publications) as there have been patients described with respect to treatment strategies (116 patients). One of the late breaking abstracts may finally have rectified this imbalance.
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Systematic review and meta-analysis of efficacy and and safety or therapies in Adult Onset Still's Disease and sJIA
IL-1i and IL-6i - highest evidence for treatment
Early (<3 months) intervention = higher rate of dz inactivity
@RheumNow #ACR23 Abs#2569 https://t.co/XzzwbLFpo7
Nov 15, 2023
New Biomarkers and Therapeutics Show Potential in Still's Disease
Nov 14, 2023
Adult-onset Still’s disease (AOSD) is a rare complex, sporadic, systemic autoinflammatory disease similar to systemic juvenile idiopathic arthritis (sJIA) characterized by sustained fever, salmon-colored rash, and arthritis.
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TM61 at #ACR23
23 yo M p/w ~3mo of sternal swelling
- US probable abscess, drained + multiple abx w/ no improvement
- XR R shoulder + L elbow nl but MRI abn
- MRI L elbow: possible osteomyelitis (OM)
- MRI R shoulder; clavicular osteolysis
- Bone Bx clavicle + L Ulna: Chronic… https://t.co/7s9PIwByTs https://t.co/wKaKfnncP5
Nov 14, 2023
https://t.co/ft1wNmMxGx
Nov 14, 2023
#VEXAS is still hot at #ACR23 - 245K whole genome sequencing data from #NIH All of US - 74 with somatic mutation UBA1
Increased asthma, Tietze’s syndrome and neutropenia
Hemoglobin similar in cases and controls
@rheumnow #abstL03 https://t.co/A374w60e6H
Nov 14, 2023
How do we treat #VEXAS - UBA1 mutation
Large multicenter cohort - 110 pts, 99% M
Confirms the benefit of JAKi and tocilizumab
Others not so much
@RheumNow #ACR23 #ACRBest
#abstL03 https://t.co/tRjO31IHiD
Nov 14, 2023
Defining accuracy of Administrative Codes for Autoinflammatory Syndromes is important to lay groundwork!
AOSD, SJIA, BD, FMF, CAPS and SAPHO-CNO ICD codes validated as Autoinflammatory syndromes (AIS)- PPV low
@RheumNow #ACR23 #abst1129 https://t.co/XqIrUmUCVc
Nov 13, 2023
Nationwide analysis of #AOSD pts with and without #HLH -
HLH Much higher in Hispanics
Much more in younger pts
Abst1143 @rheumnow #ACR23 https://t.co/fluZ4X2HHi
Nov 13, 2023
Assessment of MAP kinase pathway gene expression prior to tx may aid in identification of polyarticular course JIA patients that could exhibit poor response to tofacitinib. It may be helpful in personalized tx algorithm! #ACR23 Abs #0830 https://t.co/Z8pykbG7x5 @rheumnow https://t.co/ANZr8aVrVB
Nov 13, 2023
STOP-JIA study data from Abs #0831 suggest improved effectiveness with early combination tx vs step up and biologic first tx at the 2-3 yr timeframe for pJIA pts. More data needed to optimize outcomes/tx for these children. https://t.co/vWL5J4FPbo #ACR23 @rheumnow https://t.co/5iJpwglhPu
Nov 13, 2023
Efficacy and Safety of Targeted Therapies in #VEXAS Syndrome: Retrospective Study from the French VEXAS Group :)
#ACR23 late breaking poster - the figure says it all
https://t.co/qahDDPLois https://t.co/ueb4aG8t77
Nov 12, 2023
Is there a difference in secukinumab vs. adalimumab biosimilar on radiographic progression in a subgroup of axSpA pts with syndesmophytes or CRP levels?
Radiographic progression equally low in both drugs
NO difference in syndesmophytes or CRP
@RheumNow #ACR23 Abs#0522 https://t.co/1TBKAxuKPE
Nov 12, 2023
Good Treatment review of biologics (IL-1, IL-6) in systemic JIA (Stills) including complications of #MAS and lung disease; also info on horizon therapies like JAK inhibitors and IL-18 targeting and novel biomarkers (calgranulins, IL-18 and CXCL9) https://t.co/Cbd7bKdIbb https://t.co/d9tn9fmSAy
Nov 12, 2023
Who knew? VEXAS =cluster of findings from a somatic mutation N=110 studying retention of advanced Rx. #JAKi had far better retention than other #Rx. Although there could be channeling bias, I am impressed! Access for off label is a different issue #L03 #ACR23 @RheumNow @ACRheum https://t.co/RE4BVdZeWt
Nov 12, 2023
💡RP is a heterogeneous disease.
💡Think VEXAS in RP with MDS.
💡Think MAGIC if oral/genital ulcers.
💡Pts are experts on their own disease! Listen and acknowledge their symptoms. Shout out to Dr @maferradastrong
#ACR23 #ImNotYellingImColombian🇨🇴
@ACRheum @RheumNow https://t.co/nTZ96pJh09
Nov 12, 2023
Type III relapsing polychondritis has the MAGIC and VEXAS groups, with genital ulcers and haematological manifestations, 8% of RP patients have VEXAS, Ferrada M, #ACR23 @RheumNow https://t.co/FsmBK0F7ZD
Nov 11, 2023
Things for me to take back to the clinic in looking after JIA patients, Sadun R, #ACR23 @RheumNow https://t.co/YkiP4GZG19
Nov 11, 2023