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A large cohort study of children with febrile disorders has demonstrated the diagnostic utility of Myeloid-related protein 8/14 (MRP8/14) in diagnosing systemic JIA patients (SJIA) in clinical practice.
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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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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.
Dr. John Cush
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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
Dr. John Cush
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#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
Dr. John Cush
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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
Dr. John Cush
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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
Dr. John Cush
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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
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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
Dr. John Cush
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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
Dr. John Cush
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QD Clinic - Stills (or Not) MAS off of Tocilizumab
https://t.co/YGEifF5X8K https://t.co/719RjYzbE3
Dr. John Cush
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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
Dr. John Cush
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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
Dr. John Cush
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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
Dr. John Cush
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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
Dr. John Cush
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Adults with JIA: It ain’t all child’s play
https://t.co/z3xDjKH2ru https://t.co/9DDGYdpMSr
Dr. John Cush
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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
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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
Dr. John Cush
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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
Dr. John Cush
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#EULAR2022 has concluded but you can find all our coverage here https://t.co/s62OVJPQ86 https://t.co/QAvPQYCABj
