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Autoinflammatory

UTSW Rheum Grand Rounds New and concerning: sJIA associated lung disease - rare and associated w/ pulmonary HTN, alveolar proteinosis, high mortality. Unique features: clubbing, eosinophilia, TCZ anaphylaxis, Trisomy 21. Most were Rx w/ IL-1 > IL-6 inhibition @UTSWRheum https://t.co/aJaPurDSmJ
Dr. John Cush @RheumNow( View Tweet )
Jan 20, 2022
UTSW Rheum Grand Rounds Treating STill's (sJIA) - early early use of anakinra yields best results (in remission and off steroids) - 91% if Anakinra started within the first 3 mos @UTSWRheum https://t.co/neozpYhJtM
Dr. John Cush @RheumNow( View Tweet )
Jan 20, 2022
UTSW Rheum Grand Rounds MAS occurs in 2-8% of Systemic JIA pts and has a high mortality (<40%); It is more frequent w/ onset in Years 1-4. Treatment - steroids, anakinra, etoposide or CsA & Rx infxn if present. Oh...dont stop the anakinra! @UTSWRheum https://t.co/09uQm3LURC
Dr. John Cush @RheumNow( View Tweet )
Jan 20, 2022
UTSW Rheum Grand Rounds IL-18 levels are elevated in systemic JIA, MAS and AOSD @UTSWRheum https://t.co/pIYKf4pods
Dr. John Cush @RheumNow( View Tweet )
Jan 20, 2022
Dr. Peter Nigrovic asserts the division between pediatric and adult arthritis is senseless and has no firm biologic basis. Instead we should consider pts as sero- positive or negative, Systemci or Spondyloarthropathy. @UTSWRheum https://t.co/zx5oQmUQ6Y
Dr. John Cush @RheumNow( View Tweet )
Jan 20, 2022
UTSW Rheum Grand Rounds with Dr. Peter Nigrovic on systemic JIA in children and adults; yes, its the same disease with the same features. @UTSWRheum https://t.co/C7eBFjnDIi
Dr. John Cush @RheumNow( View Tweet )
Jan 20, 2022
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The Overpricing of Drugs (1.14.2022)

Jan 14, 2022

Dr. Jack Cush reviews the news and journal articles from the past week on RheumNow.com. This week we discuss amputations, a $57 billion RA market, an FDA approved drug for Cat arthritis and drug that works in Sjogren's.




  1. Study 126 rheumatic disease (RMD) patients showed that

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Biomarker study of 21 systemic juvenile idiopathic arthritis (sJIA) shows you can differentiate between active & inactive disease. From 69 biomarkers, these were predictive: IL-6, MMP1, S100A12 (p < 0.0001), HMGB1; but IL-18 elevated in both groups! https://t.co/m2yXTITzwY

Dr. John Cush @RheumNow( View Tweet )

Jan 12, 2022
Single center comparison of pts with MAS vs malignancy assoc. HLH (mHLH) - overall MAS had less hepatomegaly (0 v 25%), double platelet #s (50 v 29Kµ/L), but lower sIL-2R (6814 vs. 27,972.0) and less mortality (22 v 44%, p=0.18). https://t.co/NGIaPTU0yT

Dr. John Cush @RheumNow( View Tweet )

Jan 12, 2022
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Clinical Practice Guidelines for Multisystem Inflammatory Syndrome in Children

Jan 05, 2022

Multisystem inflammatory syndrome in children (MIS-C) is new and serious complication of COVID-19 that preferentially affects adolescents. A review of proposed management guidelines is compiled in this report, suggesting a unified approach to therapeutic management of MIS-C.

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There have been reports of COVID-19 vaccination causing adult-onset Still’s dz onset. Now a case of AOSD, given the BNT162b2 vax and developing Macrophage activation syndrome w/ ferritin levels of 136,680 µg/l https://t.co/caKef9BGDR

Dr. John Cush @RheumNow( View Tweet )

Jan 05, 2022
Of 173 autoimmune inflammatory rheumatic Dz pts w/ respiratory Sx, 46 had Pneumocystis jirovecii. Higher risk of PJP w/ high-dose steroids, recent immunosuppressives, combo w/ CsA, hx of chr lung dz, high LDH, ground glass CXR https://t.co/P8KtC2Hab8

Dr. John Cush @RheumNow( View Tweet )

Jan 04, 2022
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When to Stop Biologics in Systemic JIA?

Dec 13, 2021

Systemic onset juvenile idiopathic arthritis (sJIA) is a dramatic onset inflammatory condition marked by spiking fevers, intermittent rash, polyarthritis and a host of other hyperinflammatory manifestations. The condition may be well controlled in many through the use of high dose

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ICYMI: Risks associated with mortality in VEXAShttps://t.co/mpAc5shaMq https://t.co/FdOQcfwusp
Dr. John Cush @RheumNow( View Tweet )
Dec 11, 2021
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Risks associated with mortality in VEXAS

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. One

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Download Available! AOSD Disease Patterns Over Time https://t.co/qZBtCQ2QAg https://t.co/fMmN1PAzFG

Dr. John Cush @RheumNow( View Tweet )
Dec 06, 2021
ICYMI: Diagnosing (Bad) VEXAS: The Experts Speak Dr. Cush discusses Dr. Marcela Ferrada and Dr. David Beck to discuss VEXAS syndrome and abstract 1426, which was presented at the ACR21 annual meeting.https://t.co/oafDX0rJIl https://t.co/UoNpjABLNd
Dr. John Cush @RheumNow( View Tweet )
Dec 05, 2021
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Cytokine Inhibitor Lung Disease in Still's Linked to HLA-DRB1 Alleles

Dec 01, 2021

Pulmonary complications in the setting of Still's disease (juvenile and adult) has seldom but consistently been reported, including reports of pneumonitis, pulmonary hypertension and a severe or fatal lung disease, often associated with hypersensitivity to anticytokine therapy. A multicenter

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New Download Available! AOSD Disease Patterns Over Time https://t.co/IYePF20Nkn https://t.co/fOnc6jUl1b

Dr. John Cush @RheumNow( View Tweet )
Nov 29, 2021
Flare Patterns in Autoinflammatory Syndrome During Pandemic: Dr. Yuz Yusof ( @Yuz6Yusof) discusses abstract #1083 presented at the #ACR21 annual meeting.https://t.co/i0VU3NEe7F https://t.co/DZkB5TgG3N
Dr. John Cush @RheumNow( View Tweet )
Nov 24, 2021

Autoinflammatory: PAH in AOSD, VEXAS, JAKi in Still's, Role of labs in infections vs. flares Drs.Cush, Yusof, Mehta, Petryna and Tate review autoinflammatory data presented at #ACR21. @Yuz6Yusof @bella_mehta @DrPetryna @uptoTate https://t.co/oeAprkSBWZ https://t.co/1g9yRRFJqf

Dr. John Cush @RheumNow( View Tweet )
Nov 24, 2021
#ACR21 is complete--view all our RheumNow's coverage.https://t.co/wkZ66lcB44

Dr. John Cush @RheumNow( View Tweet )

Nov 23, 2021
Pulmonary Hypertension in Adult Onset Still's Disease: Dr. Jack Cush reviews poster #1100 presented at the #ACR21 annual meeting.https://t.co/2YGPHT3AAO https://t.co/lLRKnxB04a
Dr. John Cush @RheumNow( View Tweet )
Nov 22, 2021
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RheumNow Podcast - ACR 2021 Rehash (the good stuff..)

Nov 12, 2021

The ACR 2021 meeting has concluded. Dr. Jack Cush recounts a few more great abstracts from this past week’s virtual meeting.

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Emapalumab for the treatment of macrophage activation syndrome

Macrophage activation syndrome (MAS) is a term commonly used by rheumatologists to describe a potentially life-threatening complication of systemic inflammatory disorders, most commonly occurring in people with systemic juvenile idiopathic arthritis (sJIA) and systemic lupus erythematosus (

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