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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

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Md Yuzaiful Md Yusof Yuz6Yusof ( View Tweet)
ACR Best

ACR21 Best Abstracts We Saw - Day 3 (Monday)

d 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?

d 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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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

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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

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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

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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

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Dr. Rachel Tate uptoTate ( View Tweet)

Hip involvement/TJR assoc'd w/ MEFV+ vs HLA-B27+ in FMF/SpA pts vs SpA. FMF/SpA: more severe disease, worse outcomes, & destructive hip involvement vs SpA, particularly in pts with MEFV variants & M694V homozygosity. Abs 1108 #ACR21 #RheumNow @RheumNow https://t.co/hlnN26AYgE https://t.co/1jHeVxKs7O

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Dr. Rachel Tate uptoTate ( View Tweet)
Fautrel describes a series of 13 #AOSD (Stills) pts with Pulmonary arterial HTN (cath confirmed) - seen exclusively with women, 85% and all were on DMARDs - 31% MTX, 69% IL-1i, all on steroids- 5 deaths, 3 yr Survival 74% (same as PAH) #ACR21 Abstract 1100 https://t.co/qR21MdYG3A https://t.co/WN38ifgtT6

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Dr. John Cush RheumNow ( View Tweet)

#ACR21 #Abstr1100 Pulmonary Hypertension is a rare complication of AOSD. What can we learn from case-series of N=13🧐 💠All were female 💠Severe at diagnosis: mean PAP 41mmHg; 85% had NYHA III&IV 💠38.5% mortality rate So, plz be aware of PAH @RheumNow https://t.co/gmXF3AzH2n https://t.co/s5CXrHYUor

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Md Yuzaiful Md Yusof Yuz6Yusof ( View Tweet)
#ACR21 #Abstr1083 Do pts with Autoinflammatory disease flare differently during #COVID19? Survey of N=647 (mostly CAPS): 💠56% No change 💠Of pts who flared, 50% had low/absent fever 💠7% had COVID; mostly mild Shielding could influence results @RheumNow https://t.co/g7VazLfiT9 https://t.co/lzaSoMEzyR

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Md Yuzaiful Md Yusof Yuz6Yusof ( View Tweet)
#ACR21 #Abstr1011 Can we differentiate NEMO-deleted exon 5 autoinflammatory syndrome (NEMO-NDAS) vs CANDLE? Bioinformatics to mask the IKBKG pseudogene is promising and identified IL-6, 8,18, IFN-I and TNF-induced cell death as future targets @RheumNow https://t.co/tR2PNymMVn https://t.co/yCKHQx6R3E

Links:

Md Yuzaiful Md Yusof Yuz6Yusof ( View Tweet)
Daniela Ospina Cardona presenting at the Down and Dirty 30 on VEXAS. Marked differences in mortality depending on exact mutation. Looking forward to hearing more about this at Plenary session III today #ACR21 @RheumNow https://t.co/R3gXpxOfSk
Richard Conway RichardPAConway ( View Tweet)
Thermometer

JAK Inhibitors in Refractory Adult and Childhood-Onset Still’s Disease

d Bella Mehta, MBBS, MS

Still's disease is an autoinflammatory disease characterized by spiking fever, rash, polyarthralgia, sore throat and even life-threatening complications, such as macrophage activation syndrome. It was first described by George Still in the late 1800s.

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Clinical Pearl at #ACR21 from Dr. J. Stone: ⭐️Ferritin:ESR ratio helps distinguishes systemic JIA flares from macrophage activation syndrome (MAS). ⭐️During MAS: *️⃣Ferritin rapidly rises as an acute phase reactant *️⃣ESR declines as fibrinogen is consumed. #ACRBest @RheumNow

Meral K. El Ramahi, MD MeralElRamahiMD ( View Tweet)

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