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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.
Read ArticleAOSD: 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.
Read ArticleBest 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.
Read ArticleDA 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.
Read ArticleHemophagocytic 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
CBT or Naltrexone in Fibromyalgia (12.8.2023)
Dr. Jack Cush reviews the news and journal articles from the past week on RheumNow.com. Be sure to catch the early registration break for 2024 RheumNow Live.
Read ArticlePolands disease study (sarcoidosis 78.6K; #AOSD 3294; systemic sclerosis 35.5K) betw 2009-2018. Avg hospitalization was 5.39 days sarcoidosis, 6.22 days scleroderma, & 7.44 days for Still’s (latter decr 11.7 to 5.8, betw 2008-2014) https://t.co/OcNzt7IKC5 https://t.co/uPcbOyizQN
ICYMI: New Biomarkers and Therapeutics Show Potential in Still's Disease
Adult-onset Still’s disease is a rare complex, sporadic, systemic autoinflammatory disease similar to systemic juvenile idiopathic arthritis characterized by sustained fever, salmon-colored rash, and arthritis. The 2023 ACR convergence featured several abstracts spotlighting developments in understanding and managing this complex disease spectrum.
Read ArticleNEW Health Policy—Diagnosis and investigation of suspected haemophagocytic lymphohistiocytosis in adults: 2023 Hyperinflammation and HLH Across Speciality Collaboration (HiHASC) consensus guideline https://t.co/WYNcVigb7n Free to read with registration on https://t.co/28wkm59DgB https://t.co/Qbm1y76o3E
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.
Read Article