CAPS is caused by mutations in NLRP3, a component of the inflammasome. It is thought that NLRP3 mutations lead to the uncontrolled production of the inflammatory cytokine IL-1b.

CAPS (cryopyrin-associated periodic syndrome) is a heterogeneous group of autoinflammatory conditions that causes intermittent fevers (typically after cold exposure), as well as an urticarial-like rash. #ACR19 @RheumNow

New Eurofever / PRINTO criteria for #autoinflammatory diseases. Dr. Ravelli #ACR19 @rheumnow #Abstr  #ACRBest https://t.co/S35Ltegz1F

What temp is a fever? Great article from @WSJ that discusses our @feverprints app! https://t.co/Kixwp1JLB8 #rheum https://t.co/54BmZF5PhC

• At What Temperature Do You Really Have a Fever? - WSJ

I often wonder why my FMF pts are almost all young... At Cerrahpaşa in Istanbul, 180/≈5000 (3.6%) are >40yo, attacks get less frequent/severe after 40 but more comorbidities to deal with. Onset >40yo possible but milder dx (table⬇️) @egeli_bugra #ACR19 ABST1251/1256 @RheumNow https://t.co/JedUmF7KlR

They're both anti-IL-1β agents, but failure of anakinra in adult-onset Still's Disease doesn't mean canakinumab might not work (and work quickly) - see row 7-10 below. Anyone else have that experience? Corrado @Giacomo97811458, San Raffaele Hosp, Milan #ACR19 ABST1245 @RheumNow https://t.co/2qQK08apnT

#ACR19 @rheumnow ABS 1237 OLT1177, oral NLRP3 inflammasome inhibitor- a potential drug for Rx of gout flares: ⬇️ systemic inflamm by ⬇️ WBC count & circulating IL-1β& IL-6 in all3 doses (2000mg, 1000mg or 300mg). ⬇️ ex vivo IL-1β & IL-6 production by cultured PBMCs days 3, 7& 14

The questions that Dr. Amanda Ombrello (@NIH) asks when evaluating patients with recurrent fevers: -Age of onset -Frequency -Duration -Regularity -Height of fever -Prodromal symptoms #ACR19 @rheumnow

IL-1 blockade beneficial in MAS. Ferritin to ESR ratio useful for identifying these patients. Cytokines Storm Syndrome is one way of thinking of the spectrum of conditions like MAS, HLH, auto inflammatory disease which respond to IL-1 and IL-6 blockade. Cron R #ACR19 @RheumNow