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Risks associated with mortality in VEXAS

Md Yuzaiful Md Yusof, MRCP(UK), PhD Nov 09, 2021

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.

Composite MACE outcomes no different among PsA pts treated w/ biologics and DMARDs. Abs 1833 #ACR21 #RheumNow #ACRbest @RheumNow https://t.co/TxyE4Uqttq https://t.co/Eykxwmcc44

Risk of Major Adverse Cardiovascular Events (MACE) with Biologic and Targeted S…

Secukinumab in GCA, phase 2 (TitAIN): I can't deny this is promising, but phase 2 needs to be interesting both arms did worse than equivalents in GiACTA, TCZ phase 2 (?looser flare def) @drpnash: "Bring on the phase 3 trials" (recruitment started👀) #ACR21 ABSTL19 @RheumNow https://t.co/25OAL9BqIZ

#ACR21 L20- Anti-IFN Ab Emapalumab for MAS/sJIA ⭐️⬆️IFN in models of HLH/MAS, neutralization may be helpful ⭐️Pilot study in 14 pts w inadequate response to high-dose IV GC ⭐️Ok to continue IL1i ▶️13/14 pts remission ▶️ SAEs: 1 CMV reactivation https://t.co/cuM9LcEr4u @RheumNow https://t.co/USYGNtq4eC

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I am still obsessed with the name MAGIC syndrome -mouth and genital ulcers with inflamed cartilage!! 14% of patients with relapsing polychondritis have this! They also have features of aortitis, Raynaud's phenomenon and anticollagen II antibodies @rheumnow #ACR21 abt#1876 https://t.co/UlQUZlBPfx

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

#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

Efficacy of Emapalumab, an Anti-IFNγ Antibody in Patients with Macrophage Activ…

What if a celebrity had #Scleroderma? Would that ⬆️attention & funding for this rare disease? See what Big Bird says @RheumNow #ACR21 ?affected #PetsofACR @RheumNow @scleroderma @SclerodermaCAN audio-13.m4a

#ACR21 L21. REOVAS: RTX for EGPA ⭐️CYC traditionally preferred for 5-factor score>0 ▶️Remission rates and relapse-free survival comparable bw RTX and conventional. RTX not superior for induction ▶️No diff in ANCA+/-, naive v relapse, FFS https://t.co/cuM9LcEr4u @Rheumnow

https://bit.ly/3mUsMUp

#Rituximab NOT different from conventional Rx in #eGPA #churgstrauss abst#L21 v small study, 60% ANCA neg #ACR21 @RheumNow https://t.co/tO1vAF18y9

Miss anything from Day 1? We are sharing the best #ACR21 abstracts from Day 1. https://t.co/YZ3jwW0Abu https://t.co/gvWCrIbWuG

https://bit.ly/2YrDmJ7

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

https://bit.ly/3H9qxEI

Complications of Covid19 in Lupus: Dr. Kathryn Dao ( @KDAO2011) discusses abstract #1933 presented at the #ACR21 annual meeting. https://t.co/jKFJwCBeCo https://t.co/ncleyYZWLn

https://bit.ly/3qj7KBa

Abst1830 #ACR21 @RheumNow d/c of TNFi in PsA/PSO: probability of d/c TNFi ⬆️ by 9.6% for each additional biologic trial. Former smoker &PsA duration correlated w/ discontinuation. most commonly reason for d/c TNFi was secondary failure (33%). infliximab had lower d/c rate https://t.co/BXd2ALX2qo

#ACR21 L17: Heterologous vs Homologous Booster vax ▶️RTX non-responders to mRNA: Randomized to mRNA 3rd dose vs AZ vax ⭐️Majority of pts develop immune response (humoral and/or cellular) after 3rd dose, no statistical diff b/w groups @Rheumnow https://t.co/cuM9LcEr4u https://t.co/cmNBNSIoOl

https://bit.ly/3mUsMUp

What to do w Rituximab users and #COVID19 vaccine? #Rituximab no seroconversion 30% with 2 #COVIDVaccination. With 3rd vaccine dose up to 94% can have immune response! Pts need their 3rd vaccination @RheumNow #ACR21 #ACRBest abstL17 https://t.co/0GHOuoqPKQ

Deucravacitinib is a novel, oral, selective TYK2 inhibitor in PsA and selectively mediates specific inflammatory pathways (IL-23, IL-12, Type I IFN). Listen to my interview with Prof. Oliver Fitzgerald on this and his highlights https://t.co/3UJCwbHsLC @RheumNow #ACR21 Abst#490 https://t.co/OWZDHhcQ9q

Emerging Therapies in Psoriatic Arthritis: Drs. Antoni Chan and Oliver FitzGera…

Racial disparities & Co-morbidities in PsA findings. Amongst important others, increased biologic use in Caucasians w/ PsA vs DMARDs in black PsA pts. View Abs 1780 for other relevant findings. #ACR21 #RheumNow #ACRbest @RheumNow https://t.co/0uKsa8i0bG https://t.co/R9YkxYqXws

Racial Disparities in Comorbidities of Patients with Psoriatic Arthritis - ACR …

👉29% of pts on RTX fail to mount a humoral OR cellular response to #COVIDvaccine & have⬆️hosp,⬆️hosp stay,⬆️severity, persistent viremia,⬆️deaths) 👉study adding booster (AZ or mRNA) overcomes this, enhancing humoral and cellular response #ACR21 @rheumnow Abst#L17 https://t.co/yuX1TT6ZBF

Work participation in patients with axSPA Patients w/ axSpA often have increased absence from work as well as reduced productivity at work, which can lead to wider economic & social burden. Dr. Dey (@DrMiniDey) discusses abstracts from #ACR21. https://t.co/eAMLPR2zoT https://t.co/NuIajGa7gx

https://bit.ly/3BTBqXG

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Risks associated with mortality in VEXAS

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