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ICYMI: Here at Last: Treatment Options for VEXAS

Michael Putman, MD, MSCI

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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CBT or Naltrexone in Fibromyalgia (12.8.2023)

Jack Cush, MD

Dr. Jack Cush reviews the news and journal articles from the past week on RheumNow.com.  Be sure to catch the early registration break for 2024 RheumNow Live.

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Stills,continuum

ICYMI: New Biomarkers and Therapeutics Show Potential in Still's Disease

Bella Mehta, MBBS, MS

Adult-onset Still’s disease is a rare complex, sporadic, systemic autoinflammatory disease similar to systemic juvenile idiopathic arthritis characterized by sustained fever, salmon-colored rash, and arthritis. The 2023 ACR convergence featured several abstracts spotlighting developments in understanding and managing this complex disease spectrum.

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rare

Here at Last: Treatment Options for VEXAS

Michael Putman, MD, MSCI

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.

Read Article
Stills,continuum

New Biomarkers and Therapeutics Show Potential in Still's Disease

Bella Mehta, MBBS, MS

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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ICYMI: Here at Last: Treatment Options for VEXAS We have known about the VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome for nearly 3 years, but prior to #ACR23 there has been relatively little to say about how to treat it. https://t.co/FnygxtDwx8 https://t.co/2j1nbsAAp0

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Dr. John Cush RheumNow ( View Tweet)

IL-18 excess is assoc. w/ autoinflammatory Dz, "IL-18opathies". High IL-18 associated w/ #MAS & PSTPIP1-related diseases (eg, PAPA syndr. Pyogenic arthritis, pyoderma gangrenosum, acne)IL-18 binding protein is a biomarker of IFNγ activity. https://t.co/dy3xHdgTXX https://t.co/qBvCHvtsu7

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Dr. John Cush RheumNow ( View Tweet)

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/AFoKm6kT86 https://t.co/dLTjTOdbmv

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Dr. John Cush RheumNow ( View Tweet)
ICYMI: 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/ZU0XFecPyP https://t.co/GLPsf73Hj0

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Dr. John Cush RheumNow ( View Tweet)

Polands disease study (sarcoidosis 78.6K; #AOSD 3294; systemic sclerosis 35.5K) betw 2009-2018. Avg hospitalization was 5.39 days sarcoidosis, 6.22 days scleroderma, & 7.44 days for Still’s (latter decr 11.7 to 5.8, betw 2008-2014) https://t.co/OcNzt7IKC5 https://t.co/uPcbOyizQN

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Dr. John Cush RheumNow ( View Tweet)

NEW Health Policy—Diagnosis and investigation of suspected haemophagocytic lymphohistiocytosis in adults: 2023 Hyperinflammation and HLH Across Speciality Collaboration (HiHASC) consensus guideline https://t.co/WYNcVigb7n Free to read with registration on https://t.co/28wkm59DgB https://t.co/Qbm1y76o3E

The Lancet Rheumatology TheLancetRheum ( View Tweet)
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

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Dr. John Cush RheumNow ( View Tweet)
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

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Dr. John Cush RheumNow ( View Tweet)
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
Robert B Chao, MD  ( View Tweet)
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
Meral K. El Ramahi, MD MeralElRamahiMD ( View Tweet)
https://t.co/ft1wNmMxGx

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Meral K. El Ramahi, MD MeralElRamahiMD ( View Tweet)
#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
Bella Mehta bella_mehta ( View Tweet)
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
Bella Mehta bella_mehta ( View Tweet)
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
Bella Mehta bella_mehta ( View Tweet)
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
Bella Mehta bella_mehta ( View Tweet)
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