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Risks associated with mortality in VEXAS

Md Yuzaiful Md Yusof, MRCP(UK), PhD

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome (VEXAS) took the centre stage when it was first introduced at ACR20 last year. It got everyone’s thinking that they might have encountered one or two patients in their rheumatology lifetime who might have this condition.

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Telemedicine

Telemedicine Enriched Care Model for Autoinflammatory Diseases

Bella Mehta, MBBS, MS

Traditionally telemedicine is thought to be a technology where a patient directly talks to a provider or a specialist; however, another concept of having a patient with a local primary care who in turn accesses specialists via video conferencing, is gaining some momentum.  

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ACR Best

ACR21 Best Abstracts We Saw - Day 3 (Monday)

Jack Cush, MD

The third day of ACR 2021 took a big leap in online content.  Here is a compilation (with links) of presentations were the “ACRBest” as seen by our RheumNow faculty. 

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Lab work

Infection or Disease Flare: What Tools Can Help Differentiate?

Olga Petryna, MD

Differentiation between a flare of disease and infection in patients with autoinflammatory (AI) conditions, where fever is the hallmark, can be extremely difficult. Few studies have evaluated potential differentiators; and having such tools would meet a huge unmet need. 

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Having an auto-inflammatory disease specialist available via telemedicine/video for near home providers for pts. Well received by primary care, pediatricians and internal medicine physicians. Will improve the care of auto inflammatory patients! #ACR21 @rheumnow #abt1062

Bella Mehta bella_mehta ( View Tweet)

#ACR21 #AbstrL20 A promising new player on the block. A single-arm study of 14 pts with sJIA-associated MAS, Emapalumab (anti-IFNγ) induced remission in all pts with median 25 days, neutralised CXCL9 and allowed rapid steroid reduction @RheumNow #ACRBest https://t.co/7tnaS5tPJ0 https://t.co/G1IoFxLQoX

Links:

Md Yuzaiful Md Yusof Yuz6Yusof ( View Tweet)

Emapalumab (IFNg inhibitor) Effective in severe MAS (Ferritin 25K, failed anakinra, GC)- 14 pts (2-25 yrs; 4/14 sJIA)-13/14 remission, w rapid drops in CXCL9, Ferritin, ALT, DDimers & GC dose #ACR21 ABst LB20 https://t.co/05oW5C8Waf https://t.co/ecEkt3MY83

Links:

Dr. John Cush RheumNow ( View Tweet)

The plague protection genetically can lead to Pyrin induced inflammasome activation. Theory of more FMF in Mediterranean / Turkey. So high protection gene in population Dan Kastner - a genetic detective 9T119 #ACR21 #ACRBest @RheumNow https://t.co/qzdc12fT0j

Janet Pope Janetbirdope ( View Tweet)
Genes for oral aphthous ulcers common in gen pop’n, Behcets and w auto inflammatory PFAPA - kids w fevers ⬆️IL12 Kastner practical gene stories @RheumNow #ACR21 9T119. Have a listen. Bench to bedside & back https://t.co/UkPzoYbBNb
Janet Pope Janetbirdope ( View Tweet)
New dimensions in cellular immunology. Great session, some which I understood. Kids with #JIA have more cytolytic pathway missense - some leads to macrophage activation syndrome #MAS Low NK cells in Juvenile Dermatomyositis. 9T112 #ACR21 @RheumNow https://t.co/5lUkqr65UM
Janet Pope Janetbirdope ( View Tweet)
Abst1632 #ACR21 #ACRBest @RheumNow 25 children w/ uSAID & MEFV mutations: E148Q mutation standouts-32% heterozygous for E148Q, Asian ancestry seen in 37% kids w/ E148Q. E148Q assoc w/longer febrile episodes& less likely to have a full response to colchicine (25% vs 70%; p = 0.03) https://t.co/Rst6KHntPD
Olga Petryna DrPetryna ( View Tweet)
#ACR21 #Abstr1541 Any tools to help differentiate MAS vs #COVID inflammatory response? Preliminary criteria showed Sensitivity: 79.6%; Specificity: 81.3% within 6 days admission. No Triglyceride/Fibrinogen compared to 2016 ACR-EULAR & 2004 HLH @RheumNow https://t.co/c7pHaVCr5S https://t.co/6irF64qizI

Links:

Md Yuzaiful Md Yusof Yuz6Yusof ( View Tweet)
Telemedicine for AID patients! A fortunate pandemic outcome that PCPs shared "local" care benefit of specialists for these patients due to telemed. Abs 1062 #ACR21 @RheumNow #RheumNow https://t.co/zb0kZbzxsK

Links:

Dr. Rachel Tate uptoTate ( View Tweet)

A quick recap slide on VEXAS syndrome presentation. #ACR21 @RheumNow @petercgrayson https://t.co/4x8hBEFWg2
swethaann23 swethaann23 ( View Tweet)
CARRA survey in SAID pts reported no change in flare frequency w/ COVID19 but many had reduced intensity of fevers during flares. Majority of pts reported mild to moderate symptoms w/ 1/3 had lingering COVID19 symptoms. Abs 1083 #RheumNow #ACR21 @RheumNow https://t.co/qbfsqvXutt https://t.co/6u33VDIexY

Links:

Dr. Rachel Tate uptoTate ( View Tweet)
#ACR21 #Abstr1457 Nine more well-phenotyped patients with VEXAS were presented. New info: 💠Peritubular capillaritis & cryo vasculitis 💠Most cs- & bDMARDs had limited efficacy 💠Potential therapy with plasma cell blockade & stem cell transplant @RheumNow https://t.co/iDwJAdbf4M https://t.co/X4ENz4M91u

Links:

Md Yuzaiful Md Yusof Yuz6Yusof ( View Tweet)
Great Q&A re:VEXAS w/Marcela Ferrada When to suspect VEXAS? Male, MCV >100, Plt <200 Can you order genetics yourself? No. 😆 Any IMT that works? Require high dose steroid, partial response to TCZ, needs close f/u d/t possible neutropenia! How to refer to NIH? Slide!!! ⬇️⬇️ https://t.co/UvUCFbvX2r
Mike Putman EBRheum ( View Tweet)
Pulmonary Arterial Hypertension in #AOSD Rare but severe complication - life threatening French study of 13 pts - network dataset. ALL FEMALES! Age 32, mean f/u 34 mths, 5 pts (38.5%) DIED @RheumNow #ACRBest abst#1100 https://t.co/5LIzHagesP
Bella Mehta bella_mehta ( View Tweet)
You know you made it when you name appears on #ACRknowledgebowl @RheumNow #ACR21 on a question about who deveoped criteria for #AOSD https://t.co/VR5NQOfS3r
TheDaoIndex KDAO2011 ( View Tweet)

GOSH and NAC pedi CAPS pts study since 2005: anti-IL1 treatment has major impact on prevention and tx of CAPS symptoms. Observed by improved CAPS clinical dz activity scores; and normalized inflammatory markers. Abs 1110 #ACR21 #RheumNow @RheumNow https://t.co/xXrnVeRAq5

Links:

Dr. Rachel Tate uptoTate ( View Tweet)

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