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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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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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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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The RheumNow faculty have been glued to their monitors all day, watching video, and running down abstract presentations to find the best – several of these stood out as #ACRBests. Here is a listing of “Best” they saw on Day 1.
Dr. Rachel Tate
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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
swethaann23
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A quick recap slide on VEXAS syndrome presentation. #ACR21 @RheumNow @petercgrayson https://t.co/4x8hBEFWg2
Dr. Rachel Tate
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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
Md Yuzaiful Md Yusof
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#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
Mike Putman
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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
Bella Mehta
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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
TheDaoIndex
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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
Dr. Rachel Tate
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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
Dr. Rachel Tate
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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
Dr. John Cush
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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
Md Yuzaiful Md Yusof
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#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
Md Yuzaiful Md Yusof
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#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
Md Yuzaiful Md Yusof
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#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
Richard Conway
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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
Meral K. El Ramahi, MD
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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
Dr. Antoni Chan
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Treatment for VEXAS syndrome. It is steroid responsive. Avoid IL-1 inhibitors severe skin reactions at injection site, bone marrow transplant may be curative @RheumNow #ACR21 Session6S410 L Wilson, DO Cordona https://t.co/eQ1KVDiKKV
