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Rheumatic Associated Macrophage Activation Syndrome
Macrophage Activation Syndrome (MAS) is a hyperinflammatory condition that has a significant mortality risk and may arise in patients with rheumatic disease.
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Two Week Twitter (6.17.2022)
Dr. Jack Cush does a 2-week review of RheumNow Tweets on news and journal citations worth noting, along with some opinions on Telehealth, manpower, monkeypox and the price of drugs.
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Topical therapies take centre stage
Topical treatments have so often been the low level “sure you can try this” option. However there are exciting data that these approaches may become part of our main armamentarium.
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Here's a reference for the Salmon-pink skin rashes in adult-onset Still's disease; distinctive in color (faint, salmon-pink), distribution (trunk, neck extremities), and evanescence (comes and goes within the same day). These are not static eruptions! https://t.co/BgzyQeiLyW https://t.co/c3aolVfpav
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Dr. John Cush RheumNow ( View Tweet)
Single center comparison of MAS (n 18) to malignancy related mHLH (n = 16). Notable differences seen in platelet (lower in mHLH 29k v 50K), soluble IL-2R (6814 vs 27972), but more hepatomegaly (25% v 0). Less mortality with MAS (22 v 44%, p 0.18) https://t.co/gYdRfxDM2c https://t.co/ekMJfnzElC
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Dr. John Cush RheumNow ( View Tweet)
IL-18, Iike IL-1, is a produced by activation of the inflammasome. IL-18 is a potential bioimarker for #AOSD, closely linked to Dz activity & could be a Tx target, either as IL-18 binding protein (Tadekinig alfa) or mAb against IL-18 (phase 1b) https://t.co/ex231f27rg https://t.co/tjsra4blBi
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Dr. John Cush RheumNow ( View Tweet)
FDG-PET/CTscans were used in 58 for FUO evaluations. Dx was Rheumatic (44.5%), malignancy (34.5%), or infectious (10.3%). Most prevalent Rheum dx was vasculitis (17%), especially LG vessel vasculitis. FDG PET/CT is a useful FUO pts https://t.co/NEZDB20OXN https://t.co/4123Gh2v2Y
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Dr. John Cush RheumNow ( View Tweet)
Adults with JIA: It ain’t all child’s play
https://t.co/z3xDjKH2ru https://t.co/9DDGYdpMSr
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Dr. John Cush RheumNow ( View Tweet)
Are you an Expert in Still's disease, Periodic Fevers, FMF or Autoinflammatory Dz? We will add you to our expert referral listing. DM me to get on the list! https://t.co/hCkC1Yu9Nh
Dr. John Cush RheumNow ( View Tweet)
Multinational recs. on Rx of JIA assoc. Uveitis:
- Topical steroids are 1st line Rx
- Systemic Rx if above fails or w/ poor prognosis or persistence
- Options MTX, MMF then Humira EOW, then weekly ADA
- Consider non-ETN TNFi's, then TCZ, ABA, JAKi or RTX https://t.co/ZfVeWf5sVg https://t.co/yu3to0LdEd
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Dr. John Cush RheumNow ( View Tweet)
31 systemic JIA pts: w/ systemic sxs (n=8), chronic arthritis (7), remission on meds (10) & remission off meds (6). High IL-18 levels & impaired IL-18 signaling in NK cells correlated with systemic Dz activity. High IL-18 impairs NK phosphorylation https://t.co/hKTRdhuQVN https://t.co/qM5jYB3yi5
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Dr. John Cush RheumNow ( View Tweet)
#EULAR2022 has concluded but you can find all our coverage here https://t.co/s62OVJPQ86 https://t.co/QAvPQYCABj
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Dr. John Cush RheumNow ( View Tweet)
#LB0002 #EULAR2022 An interesting study design looking at flare rate following withdrawal of drug after WK12. Phase 3 RCT of Baricitinib in paediatric JIA showed 76% met ACR30 at WK12. The Double-blind withdrawal showed less patients on Bari flared vs PBO (17% vs 51%) @RheumNow https://t.co/T0mSDkoHJo
Md Yuzaiful Md Yusof Yuz6Yusof ( View Tweet)
#EULAR2022 LB002
P3 Double-Blind W/d study: Baricitinib for JIA
⭐️All pts start in open label w good benefit (1/2 with ACR70)
⭐️PBO W/d led to 50% flare vs 17% in BARI group, flares earlier
@RheumNow https://t.co/apSSLqu1mp
Eric Dein ericdeinmd ( View Tweet)
JUVE-BASIS Baricitinib in JIA
Phase 3 RCT wk12
Cs or BioDMARDs IR
⚡️Time to disease flare HR 0.24
⚡️JIA-ACR50% 63%
⚡️% flares 17% vs. 50% in PBO
Safety profile similar than adults
@RheumNow
#EULAR2022 LB0002 https://t.co/dX3DpB4QHT
Aurelie Najm AurelieRheumo ( View Tweet)
Quote of the day “Don’t be too quick to call it negative” by Marco Gattorno on looking for systemic Autoinflammatory disease (SAID) when there is the clinical suspicion #EULAR2022 @RheumNow https://t.co/SjARIlDjtR
Dr. Antoni Chan synovialjoints ( View Tweet)
So what diet to follow with autoimmunity in mind?
So many, all with different instructions.
Let’s concentrate on where they agree:
yes to fruits & vegetables
no to emulsifiers & processed foods
#EULAR2022 @RheumNow https://t.co/I9abweQOyZ
David Liew drdavidliew ( View Tweet)
JAK will quiet Still’s disease? 7 w Still’s 4 refractory disease had benefit w #JAKi POS0014. Needs more data but looks promising most w partial response . 5 on #Tofacitinib and 2 w #Baricitinib. @eular_org #EULAR2022 POS0014 @RheumNow https://t.co/j9IRgT9XJf
Janet Pope Janetbirdope ( View Tweet)
Brevet et al. SAPHO syndrome. Divided into bone group and joint/mixed group. Better response to bisphosphonate in bone group 60% vs 6%. Better response to DMARD in joint/mixed group 94% vs 40% @RheumNow #EULAR2022 POS1333 https://t.co/FfYBIEGGgJ
Richard Conway RichardPAConway ( View Tweet)
#EULAR2022 – Day 2 Report
A full day at EULAR with oral and poster presentations of abstracts in the morning and a plethora of scientific (review) sessions in the afternoon. The latter covering topics like sarcoidosis, Still’s disease, and fibromyalgia.
https://t.co/dpq5Het2rN https://t.co/NIfkjhHw86
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Dr. John Cush RheumNow ( View Tweet)
