Emapalumab (IFNg inhibitor) Effective in severe MAS (Ferritin 25K, failed anakinra, GC)- 14 pts (2-25 yrs; 4/14 sJIA)-13/14 remission, w rapid drops in CXCL9, Ferritin, ALT, DDimers & GC dose #ACR21 ABst LB20 https://t.co/05oW5C8Waf https://t.co/ecEkt3MY83

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The plague protection genetically can lead to Pyrin induced inflammasome activation. Theory of more FMF in Mediterranean / Turkey. So high protection gene in population Dan Kastner - a genetic detective 9T119 #ACR21 #ACRBest @RheumNow https://t.co/qzdc12fT0j

Genes for oral aphthous ulcers common in gen pop’n, Behcets and w auto inflammatory PFAPA - kids w fevers ⬆️IL12 Kastner practical gene stories @RheumNow #ACR21 9T119. Have a listen. Bench to bedside & back https://t.co/UkPzoYbBNb

New dimensions in cellular immunology. Great session, some which I understood. Kids with #JIA have more cytolytic pathway missense - some leads to macrophage activation syndrome #MAS Low NK cells in Juvenile Dermatomyositis. 9T112 #ACR21 @RheumNow https://t.co/5lUkqr65UM

Abst1632 #ACR21 #ACRBest @RheumNow 25 children w/ uSAID & MEFV mutations: E148Q mutation standouts-32% heterozygous for E148Q, Asian ancestry seen in 37% kids w/ E148Q. E148Q assoc w/longer febrile episodes& less likely to have a full response to colchicine (25% vs 70%; p = 0.03) https://t.co/Rst6KHntPD

#ACR21 #Abstr1541 Any tools to help differentiate MAS vs #COVID inflammatory response? Preliminary criteria showed Sensitivity: 79.6%; Specificity: 81.3% within 6 days admission. No Triglyceride/Fibrinogen compared to 2016 ACR-EULAR & 2004 HLH @RheumNow https://t.co/c7pHaVCr5S https://t.co/6irF64qizI

• Preliminary Criteria for Macrophage Activation Syndrome Associated with Coronav…