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Diseases associated with NLRP3 mutations Dr Hal Hoffman #RNL2020 https://t.co/pN2c2LhjJe
Earlier Anakinra Initiation Warranted in Still's Disease
An Italian study examined the outcomes in adult onset Still's disease (AOSD) based on whether they received early or delayed treatment with anakinra (ANK).
A total of 141 AOSD patients were retrospectively studied.
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CAPS, best outcomes with triple therapy that includes Apheresis. Dr Compton #Utsw Rheum Grand Rounds https://t.co/MVesTmSNQK
Criteria for CAPS. Dr. Compton #Utsw Rheum Grand Rounds https://t.co/tdVWhMEII3
Serum aldolase serves as a useful marker for diagnosis and assessment of disease activity in patients with adult-onset Still's disease. https://t.co/K1Z8uqLwML
Dr. John Cush RheumNow ( View Tweet)
Methotrexate and the Risk of Lung Disease
Rheumatology has a comprehensive overview of methotrexate (MTX) and the risk of lung injury, MTX-related pneumonitis and interstitial lung disease (RA-ILD) with rheumatoid arthritis (RA). Past reports suggest the frequence of MTX-pneumonitis to be between 0.3 and 11.6%; r
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Tofacitinib Effective in Refractory Still's Disease
Fourteen cases reported from mainland China suggest that tofacitinib (TOFA) may be effective in patients with refractory adult-onset Still’s disease (AOSD), further adding to a growing list of potential use of JAK inhibition in managing inflammatory, and now autoinflammatory, disease.
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#UTSW Dr Nicole Bittencourt on fever and autoinflammatory workup. Here are sources for genetic testing. I rec the Invitae autoinflammatory panel (or cash pay ~$100) https://t.co/XsJ0k7q0ex
Study of 182 hospitalized adult-onset Still's disease patients shows predictive factors for developing macrophage activation syndrome to include: 1. Splenomegaly (OR 5.7), 2. pericarditis (OR 6.5), and 3. ferritin >2000 (OR 4.7). https://t.co/CNcIDrbU77
Dr. John Cush RheumNow ( View Tweet)
Systemic JIA-Associated Lung Disease
A disease you’ve never heard of is becoming increasingly common and carries a very poor prognosis.
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Autoinflammatory Diseases at the ACR Annual Meeting
It has been a wonderful year for autoinflammatory diseases at the ACR Annual Meeting.
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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.
Jonathan Hausmann MD hausmannMD ( View Tweet)
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
Jonathan Hausmann MD hausmannMD ( View Tweet)
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
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
Olga Petryna DrPetryna ( View Tweet)
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
Jonathan Hausmann MD hausmannMD ( View Tweet)
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
Dr. Antoni Chan synovialjoints ( View Tweet)