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New therapeutic approach for the treatment of sarcoidosis
A research team led by Georg Stary (Medical University of Vienna and CeMM) has identified a new approach to treating the inflammatory disease sarcoidosis. In a clinical study, the inhibition of a specific signaling pathway showed clear success in the treatment of granulomas in the skin. This opens up new therapeutic paths for sarcoidosis and similar inflammatory diseases. The results were recently published in the journal Lancet Rheumatology.
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What do you know about monogenic and polygenic autoinflammatory diseases? Join Drs Liebowitz and Mehta as they review the challenges of these conditions. https://t.co/TXJbpD3yB4 https://t.co/QGDaZdqrqz
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StillsNow Podcast December 2023 - ACR23 Highlights
Dr. Jack Cush reviews selected AOSD & systemic JIA abstracts from the ACR 2023 Annual meeting held in San Diego, November 12th thru 16th.
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Dr. John Cush RheumNow ( View Tweet)
AOSD: Young vs. Elderly Onset AOSD is an autoinflammatory condition characterized by fevers, arthritis, & rash. It is considered an orphan disease because of its low prevalence - reported anywhere between 16 to 40 per ten million. https://t.co/Hw9RECWYg5 https://t.co/A5JMCSpl0D
Best of 2023: EULAR/ACR Guidance on Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome
A EULAR/American College of Rheumatology task force has established evidence based, up-to-date guidance and expert opinion on the evaluation, management and monitoring of patients with Haemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS), with the primary intent to halt disease progression and prevent life-threatening complications from HLH/MAS.
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Best of 2023: LAVLI - A New Autoinflammatory Disorder
NIH researchers have have described a novel autoinflammatory disorder called "Lyn kinase-associated vasculopathy and liver fibrosis" (LAVLI), based on a mutation in the LYN gene (that encodes the Lyn kinase protein). They discovered that increased Lyn kinase activity promotes systemic inflammation, by altering microvascular permeability and neutrophil recruitment, while at the same time promoting hepatic fibrosis.
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DA Approves Canakinumab for Gout Flares The US FDA has approved canakinumab \ Ilaris\ for the treatment of gout flares in adults who cannot be treated with NSAIDs, colchicine, or repeated courses of corticosteroids. https://t.co/OX3wIajQtm https://t.co/tMDMc8ZKE8
IL-18 excess is assoc. w/ autoinflammatory Dz, "IL-18opathies". High IL-18 associated w/ #MAS & PSTPIP1-related diseases (eg, PAPA syndr. Pyogenic arthritis, pyoderma gangrenosum, acne)IL-18 binding protein is a biomarker of IFNγ activity. https://t.co/dy3xHdgTXX https://t.co/qBvCHvtsu7
ICYMI: Here at Last: Treatment Options for VEXAS
We have known about the VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome for nearly 3 years, but there has been relatively little to say about how to treat it. To date there have been over twice as many publications about VEXAS (263 publications) as there have been patients described with respect to treatment strategies (116 patients). One of the late breaking abstracts may finally have rectified this imbalance.
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Hemophagocytic Lymphohistiocytosis in Still's disease Dr. Bella Mehta discusses abstract 1143, presented at the 2023 ACR Convergence meeting in San Diego, CA. https://t.co/AFoKm6kT86 https://t.co/dLTjTOdbmv