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Autoinflammatory

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Great and Not So Great (9.30.2022)

Sep 30, 2022

This week it's the great and not-so-great on gout, chondrocalcinosis, osteoporosis and misdiagnosis. We are good at many of those things - what's not so great? Let's review the news and journal reports from the past week on RheumNow.

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Joint Flares extend disease & show joint specific memory. Study of 95 JIA pts, 12.5 yrs F/U: 95% got to inactive dz, 81% flared 1+ times. 74% flares in prior joints, usual side 83%, contralateral 17%, 40% new Jt; more likely off meds https://t.co/20UEtZXUIW https://t.co/yDt7WXvkB0
Is there a link between PsA & autoinflammatory disease? Dr. Cush reviews a recent study of 120 #PsA patients in our latest podcast.https://t.co/b15zmfXACa https://t.co/aCY08xXtPw
IL-18 is a proinflammatory cytokine involved in innate &adaptive immune responses. IL-18 is a key mediator in autoinflammatory diseases, MAS, Still’s disease. Serum IL-18 levels may be useful in diagnosis and monitoring of disease activity https://t.co/IB9CwbILD9 https://t.co/TWNnjJyR2Z
Worldwide, IL-6 inhibitors are approved for RA, poly JIA, systemic JIA, AOSD, GCA, Takayasu arteritis, Castleman’s dz, CAR-T-cell-induced cytokine release syndrome, neuromyelitis optica spectrum disorder and severe COVID-19 https://t.co/SRPjerXnGS
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Heavy Metal Rheumatology (9.23.2022)

Sep 23, 2022

Below are this week’s highlights from RheumNow, as discussed by Dr. Jack Cush. Thanks to all of you for your kind comments and great reviews of our weekly podcast. Please let us know how we can improve by emailing me or recording your suggestion using the "Ask Cush Anything" link on our website

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NIH: 27 pts w/ ROSAH (retinal dystrophy, optic N. edema, splenomegaly, anhidrosis, headache) syndrome, due to dominant mutation ALPK1 - an autoinflammatory Dz w fevers, HA, Abnl CNS MRI, deforming arthritis, AA amyloidosis . https://t.co/Pyb3QNrPnB https://t.co/wxtwOGpKFr

Colchicine vs cimetidine in PFAPA (Periodic fever, aphthous stomatitis, pharyngitis, adenitis) syndrome. 72 pts given COL vs CIM x 3 mos: # of febrile attacks was significantly decreased by both Rx (noninferior to each other) https://t.co/MO7IQ0e8gf

The 2022 ACR abstracts are published and posted for you. Let the Learning begin! https://t.co/ZqMAc8jV3U https://t.co/3iCGnzsPI5
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ACR Questions Medicare Proposed Cuts to Critical Services Reimbursement by Cognitive Specialists

Sep 08, 2022

In comments submitted to the Centers for Medicare and Medicaid Services (CMS) in response to the CY 2023 Medicare Physician Fee Schedule and Quality Payment Program proposed rule, the American College of Rheumatology applauded proposals that would provide more flexibility and improve care

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Rheums! Have a Rheumatology question or case for Jack Cush? Record it here and we may feature it on an upcoming podcast. Tell us your name and where you practice rheumatology. https://t.co/1aSrRsLkel https://t.co/gmh155ty6w
StillsNow Podcast August 2022 - Genetic Testing for Autoinflammatory Diseases Featuring a discussion with Dr. Susan Shenoi from Seattle Childrens Hospital https://t.co/W9e6r83R6B https://t.co/rVvDurZz2g
Genetic Testing for Autoinflammatory Disease Not all patients with periodic fevers fit neatly into diagnostic categories. Some can be diagnosed as Still’s disease (based on criteria) while others can be classified as AID and some may be unclassifiable.https://t.co/E5qZS1JyAY https://t.co/PYgUbiduPF

Review of IL-1 inhibitors in recurrent idiopathic pericarditis. While Rilonacept is FDA approved (320 mg x1 then 160 mg sc/wk), anakinra (2 mg/kg/d or 100 mg/d), canakinumab and colchicine are used https://t.co/j00yWpBbq3 https://t.co/7BK2mopssc

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Goofy But True

Sep 02, 2022

Dr. Jack Cush discusses declining survival rates in the USA, FDA approvals of new COVID subvariant boosters and other odd and possibly true new research reports from the past week on RheumNow.com.




  1. After historic drop in 2020 (COVID), life expectancy in USA dropped again,

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Genetic Testing for Autoinflammatory Disease

Sep 01, 2022

Not all patients with periodic fevers fit neatly into diagnostic categories. Some can be diagnosed as Still’s disease (based on criteria) while others can be classified as autoinflammatory diseases (AID) and some may be unclassifiable, clinically or genetically.

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Recent review of AOSD suggests: - AOSD & SJIA are the same - Classify dz as systemic or articular - #MAS occurs in up to 23% - Yamaguchi & Fautrel criteria are widely used - Cytokines important: IL-1, IL-6, IL-18, IL-37 - only Canakinumab is FDA-approved https://t.co/4ERA56Wqcb https://t.co/FkIWxIrwxX

Disease Activity Scoring in AOSD AOSD or sJIA can have dramatic symptom severity, making it easy to gauge disease activity and response to therapy, especially at the outset. However, a validated measure of disease activity has not been agreed upon.https://t.co/oD6uDD6W8m https://t.co/KxNJeJ31h8
Tapering biologics in Still's/Systemic JIA? 4 studies suggests after sustained inactive disease achieved, options include QOD (anakinra) dosing or dose reduction or interval prolongation (CAN & TCZ). KEY: early biologic starts https://t.co/33tjr65Gi0 https://t.co/fi6fc4NPgG
Study of Adult-onset Still's in Western Australia. Incidence = 0.22/100 000, prevalence = 2.4/100 000. AOSD had higher risk of previous liver disease (OR 2.67), serious infx (OR 4.36), joint replacement (OR 45.5), mortality not increased @ 1 & 5 yrs https://t.co/Wu0SQIMjWT https://t.co/Zvsdb2OR0y
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Disease Activity Scoring in Adult-onset Still's disease

Aug 30, 2022

Still's disease in adults (AOSD) or children (sJIA) can have dramatic symptom severity, making it easy to gauge disease activity and response to therapy, especially at the outset. However, a validated measure of disease activity has not been agreed (for clinical trial and treatment assessments

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Myths & Misconceptions with Febrile Disorders Still’s disease and autoinflammatory disorders are rare conditions unified by recurrent fevers and inflammation of skin, joints, and other organs.https://t.co/vdZWR1cwDx https://t.co/OoGIBjpZGn
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Axial Psoriatic Arthritis and Ankylosing Spondylitis with Psoriasis

Aug 22, 2022

A cohort analysis from Toronto suggests that axial psoriatic arthritis (PsA) is distinctly different from axial ankylosing spondylitis (AS) with psoriasis.



Researchers examined two PsA and AS cohorts -  patients with PsA with axial disease and isolated axial patients with AS with

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Rheums! Do you have a rheumatology question or case for Jack Cush? Record it here and we may feature it on an upcoming podcast. Tell us your name and where you practice rheumatology. https://t.co/cvmbzknQqA https://t.co/6mX5eRUNuU
Intensity and longevity of SARS-CoV-2 vaccination response in patients with immune-mediated inflammatory disease: a prospective cohort study https://t.co/1fiNgBQUbr looks like lower ab response and drops over 6 mths - boosters important in our patients
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