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JAK Inhibitors in Refractory Adult and Childhood-Onset Still’s Disease

Bella Mehta, MBBS, MS

Still's disease is an autoinflammatory disease characterized by spiking fever, rash, polyarthralgia, sore throat and even life-threatening complications, such as macrophage activation syndrome. It was first described by George Still in the late 1800s.

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ACR Best

ACR21 Best Abstracts - Day 1

Jack Cush, MD

The RheumNow faculty have been glued to their monitors all day, watching video, and running down abstract presentations to find the best – several of these stood out as #ACRBests. Here is a listing of “Best” they saw on Day 1.

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acr_session2.jpeg

Opening Day Report

Jack Cush, MD

The opening of ACR2 Convergence was a hit for all who signed up and viewed in. The day included the presidential address by outgoing president Dr. David Karp (UT Southwestern) and a keynote talk and interview with Dr. Seema Yasmin (Stanford).

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Hip involvement/TJR assoc'd w/ MEFV+ vs HLA-B27+ in FMF/SpA pts vs SpA. FMF/SpA: more severe disease, worse outcomes, & destructive hip involvement vs SpA, particularly in pts with MEFV variants & M694V homozygosity. Abs 1108 #ACR21 #RheumNow @RheumNow https://t.co/hlnN26AYgE https://t.co/1jHeVxKs7O

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Dr. Rachel Tate uptoTate ( View Tweet)
Fautrel describes a series of 13 #AOSD (Stills) pts with Pulmonary arterial HTN (cath confirmed) - seen exclusively with women, 85% and all were on DMARDs - 31% MTX, 69% IL-1i, all on steroids- 5 deaths, 3 yr Survival 74% (same as PAH) #ACR21 Abstract 1100 https://t.co/qR21MdYG3A https://t.co/WN38ifgtT6

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Dr. John Cush RheumNow ( View Tweet)

#ACR21 #Abstr1100 Pulmonary Hypertension is a rare complication of AOSD. What can we learn from case-series of N=13🧐 💠All were female 💠Severe at diagnosis: mean PAP 41mmHg; 85% had NYHA III&IV 💠38.5% mortality rate So, plz be aware of PAH @RheumNow https://t.co/gmXF3AzH2n https://t.co/s5CXrHYUor

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Md Yuzaiful Md Yusof Yuz6Yusof ( View Tweet)
#ACR21 #Abstr1083 Do pts with Autoinflammatory disease flare differently during #COVID19? Survey of N=647 (mostly CAPS): 💠56% No change 💠Of pts who flared, 50% had low/absent fever 💠7% had COVID; mostly mild Shielding could influence results @RheumNow https://t.co/g7VazLfiT9 https://t.co/lzaSoMEzyR

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Md Yuzaiful Md Yusof Yuz6Yusof ( View Tweet)
#ACR21 #Abstr1011 Can we differentiate NEMO-deleted exon 5 autoinflammatory syndrome (NEMO-NDAS) vs CANDLE? Bioinformatics to mask the IKBKG pseudogene is promising and identified IL-6, 8,18, IFN-I and TNF-induced cell death as future targets @RheumNow https://t.co/tR2PNymMVn https://t.co/yCKHQx6R3E

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Md Yuzaiful Md Yusof Yuz6Yusof ( View Tweet)
Daniela Ospina Cardona presenting at the Down and Dirty 30 on VEXAS. Marked differences in mortality depending on exact mutation. Looking forward to hearing more about this at Plenary session III today #ACR21 @RheumNow https://t.co/R3gXpxOfSk
Richard Conway RichardPAConway ( View Tweet)
Clinical Pearl at #ACR21 from Dr. J. Stone: ⭐️Ferritin:ESR ratio helps distinguishes systemic JIA flares from macrophage activation syndrome (MAS). ⭐️During MAS: *️⃣Ferritin rapidly rises as an acute phase reactant *️⃣ESR declines as fibrinogen is consumed. #ACRBest @RheumNow

Meral K. El Ramahi, MD MeralElRamahiMD ( View Tweet)

Treatment for VEXAS syndrome. It is steroid responsive. Avoid IL-1 inhibitors severe skin reactions at injection site, bone marrow transplant may be curative @RheumNow #ACR21 Session6S410 L Wilson, DO Cordona https://t.co/eQ1KVDiKKV
Dr. Antoni Chan synovialjoints ( View Tweet)
VEXAS a new genetic disorder, (V) Vacuoles, (E) E1-enzyme, (X) X-linked, (A) Autoinflammatory, (S) Somatic. These are the important clinical features to be looking out for in clinic @RheumNow #ACR21 Session6S410 L Wilson, DO Cordona https://t.co/dBBLox6vVg
Dr. Antoni Chan synovialjoints ( View Tweet)
IL-18 in autoinflammatory Disorders session talk by Raphaela Goldbach-Mansky - Discovered in 1995 - IL18 expressed in epithelial cells, keratinocyts, macrophages - IL-18R neg regulated by IL-18 binding protein https://t.co/U6TTDqEb7L https://t.co/R2lIo188ac

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Dr. John Cush RheumNow ( View Tweet)
It is always difficult to distinguish infection and disease flares in autoinflammatory syndromes = 25 pt sJIA study at #ACR21 showing serum procalcitonin levels are useful when >0.95ng/ml @StillsDis_411 @rheumnow #abst0252

Bella Mehta bella_mehta ( View Tweet)

Fautrel reports mixed response to JAK Inhib. in 8 refractory Adult (6) & juvenile (2)-Onset Still's Dz w/ dz Dz duration 86 mos (7 Systemic, 1 Chr articular) w/ JAKi (Bari 4, Upa1, Tofa 2, Ruxo 2). There were no remissions, 50% had partial response @ 11 mos #ACR21 Abstr#0195 https://t.co/Ww8ONBrRqq

Dr. John Cush RheumNow ( View Tweet)

#ACR21 #Abstr0252 Let's examine our patients than relying on tests. In sJIA pts presenting with fever, clinical features (fever pattern, rash and MSK symptoms) distinguished inflammation vs infection but not CRP, ferritin and procalcitonin @RheumNow https://t.co/s61WGBVK73 https://t.co/qhN95GdwgJ

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Md Yuzaiful Md Yusof Yuz6Yusof ( View Tweet)
@RheumNow #acr21 abst 195 prospective study of JAKi for refractory Still’s in 7 pts suggests JAKs can be useful as an add on but not in monotherapy : partial response 57%& failure in43%. No patient achieved complete remission. steroids could be decreased by63%~ https://t.co/rYHVReRHvL
Olga Petryna DrPetryna ( View Tweet)
Abst0254 #ACR21 @RheumNow CARRA SJIA cohort defines factors associated with medication free remission for ≥6 month-period: younger age, normal CRP levels & shorter time btw diagnosis and start of Rx. none of pts who developed MAS (3.9%) able to d/c Rx for ≥6 months

Olga Petryna DrPetryna ( View Tweet)

Polyautoimmunity: axSpA and Familial Mediterranean Fever by Dr. Rachel Tate ( @uptoTate ) Mysteries behind polyautoimmunity have confounded rheumatologists for decades. One such association, the co-existence of FMF and axSpA, remains rare. https://t.co/GbU1ikeyQw #ACR21 https://t.co/dRd6yxjieS

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Dr. John Cush RheumNow ( View Tweet)

Rare co-existence of FMF & axSpA significantly lowered age of axSpA symptom onset. Radiographic changes in FMF+axSpA suggested worse disease. Rate of amyloidosis and hip involvement were higher in FMF+axSpA pts. Abs 0378 #ACR21 #RheumNow @RheumNow https://t.co/lUmn3A90Ob https://t.co/n3kj8GcV1i

Links:

Dr. Rachel Tate uptoTate ( View Tweet)
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