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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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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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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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Anakinra studied in COVID(+) ARDS (nonICU) pts w/ high CRP L or ferritin; 29 high dose Anakinra vs 17 controls. At day 21, more survival with anakinra 90% vs. Controls 56% (p=0·009). Anakinra also more improved CRP+resp function (72 vs 50%) https://t.co/wxjlQG44Oc
Links:
Dr. John Cush RheumNow ( View Tweet)
Inzomelid, a selective, orall small molecule inhibitor of the NLRP3 inflammasome, has been tested in a phase I trial of CAPS (Cryopyrin-Associated Periodic Syndrome) https://t.co/8gO63xO9bC
Dr. John Cush RheumNow ( View Tweet)
Hyper IgD syndrome. Dr. Dan Kastner #RNL2020 https://t.co/PF5TxDzvdt
Features of TRAPS. Episodes last 4 to 6 weeks. Amyloid in 10 to 15%. Dr. Dan Kastner #RNL2020 https://t.co/E3CjPa7EVA
Key features of FMF Dr. Dan Kastner #RNL2020 https://t.co/8BmXxRMvEi
5 known inflammasomes and what activates them Dr. Hal Hoffman #RNL2020 https://t.co/3qxs1c13YN
Diseases associated with NLRP3 mutations Dr Hal Hoffman #RNL2020 https://t.co/pN2c2LhjJe
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)
#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)
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
