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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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About 1 in 7 systemic JIA are "refractory" to standard Rx. Could the definition be active systemic or arthritic disease, despite IL-1 or IL-6 inhibitors? It could also include pts developing MAS, ILD or amyloidosis. Still, there are many Rx options. https://t.co/VBeHhHEtqW https://t.co/67c5Emo337
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Dr. John Cush RheumNow ( View Tweet)
#AOSD cause is unknown, but matched case control study suggests signif link to Stressful life events (OR 2.56) & nonsignificant risk trending towards coal dust exposure (OR 3.0), allergy prior to onset (OR 2.67) & oral contraceptive use (OR 2.0) https://t.co/Saa4gEbzKS https://t.co/87RKgojmGZ
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Dr. John Cush RheumNow ( View Tweet)
Proposed investigations (findings) in the diagnosis of Stills disease:
- CRP, ESR, Ferritin (high)
- W/U for infection, cancer (negative)
- Serologies (negative)
- consider Imaging (CXR, CT, US, PET)
https://t.co/l3yqAx6PA0 https://t.co/Sg7okwKHaw
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Dr. John Cush RheumNow ( View Tweet)
Guidelines for MAS complicating Systemic JIA: Dx is based on:
- Fever in sJIA with ^ ferritin > 684, plus
- Any 2 of these: elevated PLTs, AST, Triglycerides or low Fibrinogen
https://t.co/aIEvxRYZca https://t.co/DYpLHxRCzI
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Dr. John Cush RheumNow ( View Tweet)
Cyclic GMP–AMP synthase (cGAS) engages stimulator of interferon genes (STING) to trigger inflammatory cytokines & type I interferons. cGAS–STING activation by genomic or mitochondrial self DNA implicated in autoinflammatory & autoimmune dz https://t.co/7CEFt9wLrl https://t.co/HpHhjJOATd
Dr. John Cush RheumNow ( View Tweet)
Five Mistakes When Diagnosing Still’s Disease
AOSD is likely to be the adult continuum of systemic-onset juvenile arthritis (sJIA) and hence, frequently affects young men and women below the age of 35 years.
https://t.co/C9kUOpFIlc https://t.co/82GIA7hSJl
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Dr. John Cush RheumNow ( View Tweet)
Solving Still’s Disease
Still's disease is the most common rheumatic cause of unknown fever in adults. This series covers the definition, history, epidemiology, key manifestations and labs, diagnostic criteria, and more.
https://t.co/YqpSW6T3El https://t.co/vwehTCm4aN
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Dr. John Cush RheumNow ( View Tweet)
QD Clinic - Stills (or Not) MAS off of Tocilizumab
https://t.co/YGEifF5X8K https://t.co/719RjYzbE3
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Dr. John Cush RheumNow ( View Tweet)
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)
