Autoinflammatory

Renal Dz in 44 AOSD (Stills)pts-mean age 38; 28 w/ renal onset after Dx. 36 renal Bx showed amyloidosis (25%), collapsing glomerulopathy (11%), thrombotic microangiopathy (11%), IgA nephropathy (9%), minimal change (7%). Renal assoc w/ 6 deaths; 4 ESRD https://t.co/tQykmjNIIb https://t.co/n0xgi6uF6D

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Dr. John Cush @RheumNow( View Tweet )
Systemic Score Predicts Still's Disease Severe Complications A multinational study of adults with Still's disease found that calculating their systemic score at baseline significantly predicted the patients risk for life-threatening outcomes. https://t.co/YB7EcWFruE https://t.co/Vcql5tZHOk

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Dr. John Cush @RheumNow( View Tweet )
Stills,continuum

Systemic Score Predicts Still's Disease Severe Complications

Jack Cush, MD

A multinational study of adults with Still's disease  found that calculating their systemic score at baseline significantly predicted the patients risk for life-threatening outcomes (MAS or death).

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Stills pts w/ remission on Tocilizumab who stop TCZ, 50% will relapse in 1 yr. Lower relapse rate w/ longer TCZ use w/ prednisolone lowering. 38/48 AOSD Rx w/ TCZ were in remission after 6 mos. & 12 D/C TCZ - half flared after mean of 5.5 mos.https://t.co/L9XbUX8lXn https://t.co/Z4oixQR2oX

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Dr. John Cush @RheumNow( View Tweet )
Retro study 109 oligoarticular JIA pts (~8 yrs) Rx w/ intraarticular steroids w/ 4.3 yrs F/U: 38% needed no Rx, 15.5% needed more IAS; 46% systemic Rx(MTX; 8 mos) &22% biologics(2.2 yrs later). @FUV 89% were inactive. Persistence predicted by ANA+ & B27+ https://t.co/5YLkYyA84l https://t.co/wClhEiKOIw

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Dr. John Cush @RheumNow( View Tweet )
HS-Related Autoinflammatory Syndromes HS is an autoinflammatory skin disorder of the terminal hair follicle, manifesting with painful nodules, abscesses, draining tunnels, and hypertrophic scarring, typically occurring in apocrine gland bearing skin. https://t.co/02Svwj99Bm https://t.co/9oy1UngII7

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Dr. John Cush @RheumNow( View Tweet )
hidradenitis.jpg

Hidradenitis Suppurativa-Related Autoinflammatory Syndromes

Jack Cush, MD

Hidradenitis suppurativa (HS) is a suppurative inflammatory skin disorder that is considered to be autoinflammatory by many. HS includes a specturm of disorders, linked by suppurative (or psoriatic) skin disease and potentially genetics. These HS-related disorders may include 

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Effective Treatments for Still's Disease A systematic review of pharmacological treatments for adult-onset Still disease (AOSD) supports the efficacy of tocilizumab (TCZ), anakinra (ANK), and canakinumab (CNK). https://t.co/Qi98OXyoLJ https://t.co/DCgXfs0Bot

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Dr. John Cush @RheumNow( View Tweet )
Stills,continuum

Effective Treatments for Still's Disease

Jack Cush, MD

A systematic review of pharmacological treatments for adult-onset Still disease (AOSD) supports the efficacy of tocilizumab (TCZ), anakinra (ANK), and canakinumab (CNK).



Data and results from 44 studies abstracts evaluated treatments, including nonsteroidal antiinflammatory drugs (

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Depression and RA (3.8.2024)

Jack Cush, MD

Dr. Jack Cush reviews the news, journal reports and regulatory approvals from the past week.

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Stills Dz Rx w/ IL-1 or IL-6 inhib may mask Dx of MAS! Study of 162 systemic JIA & MAS (62 on biologics). Biologic Rx pts had lower Plt, Ferritin (1107 vs 2863(, CRP (15 vs 90), ESR, fever duration. 27% on IL1/IL6i did not meetMAS classification criteria https://t.co/RmAzxh459R https://t.co/SXroqNBLSZ

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Dr. John Cush @RheumNow( View Tweet )
Fever of Unknown Origin: Not Always an Infection Roel Sanchez Baez, MD presents his poster at RWCS 2024. https://t.co/WiDfcNHuNv https://t.co/gTs78QmE2A

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Dr. John Cush @RheumNow( View Tweet )
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Sniffles & Arthritis (3.1.2024)

Jack Cush, MD

Dr. Jack Cush reviews the news and journal reports from the past week on RheumNow.com. Are these associations with infection, biomarkers and weather real or imaginary? Linked or luck? Causal or casual?  You decide....





  1. No clear link between weather & MSK symptoms

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PAPA (pyogenic arthritis, pyoderma gangrenosum & acne) syndrome is a hereditary autoinflammatory Dz caused by dominant mutation in PSTPIP1 (triggers IFN-γ-mediated pyrin induction & IL-18 release; can be Tx by JAK inhibition) https://t.co/fy0MnwDA5U https://t.co/l8n2EwgV0t

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Dr. John Cush @RheumNow( View Tweet )
Join our experts, Drs Liebowitz and Mehta, as they provide insights on the differential diagnosis and management concepts in autoinflammatory diseases. https://t.co/r5WjYIdmaT https://t.co/gGdhyDKqtC

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Dr. John Cush @RheumNow( View Tweet )
Transcriptome analysis (of PBMCs) looked for differential gene expression btwn 125 SJIA & 92 healthy controls identified 4 key genes (ALDH1A1, CEACAM1, YBX3 and SLC6A8) distinguishing SJIA; & may aid in diagnosis. https://t.co/M6pfajw3U3 https://t.co/VVwrYUJcBH

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Dr. John Cush @RheumNow( View Tweet )
Systematic review of 44 studies looking at treatment of Stills Dz (AOSD), showing that tocilizumab, anakinra & canakinumab therapy are effective in AOSD. Complete remission: - TCZ 80% (57% Steroid D/C) - ANAK 73% (47% GC DC) - CAN 77% (34% GC DC) https://t.co/bFil81GGlJ https://t.co/F5NJ6m1gVF

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Dr. John Cush @RheumNow( View Tweet )
JAMA Clinical Challenge - Recurrent Fever, Rash, and Shortness of Breath in a 69-Year-Old. Admited with fever, fatigue, rash, right periorbital swelling, SOB, Palpable purpural, Serositis previously - whats your diagnosis? https://t.co/j6aSGffpPv https://t.co/du6pEo74xp

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Dr. John Cush @RheumNow( View Tweet )
human figures on blue circle

New therapeutic approach for the treatment of sarcoidosis

Eureka Alert!

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

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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

Links:

Dr. John Cush @RheumNow( View Tweet )
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/3ssM9fMGCm https://t.co/FWZPx9Cpn7

Links:

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/gvbPkeq2sH https://t.co/J25U6rWNTy

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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/vY1yZUX25S

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Dr. John Cush @RheumNow( View Tweet )

Stillsnow, Stills, podcast

StillsNow Podcast December 2023 - ACR23 Highlights

Jack Cush, MD

Dr. Jack Cush reviews selected AOSD & systemic JIA abstracts from the ACR 2023 Annual meeting held in San Diego, November 12th thru 16th.





  1. Abstract 0761. EULAR / PreS Recommendations for the Diagnosis and Management of Systemic Juvenile Idiopathic Arthritis (sJIA) and

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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

Links:

Dr. John Cush @RheumNow( View Tweet )

Recent Blogs

Best of 2022: Five Mistakes When Diagnosing Still’s Disease

Jack Cush, MD
Thermometer
Adult-onset Still's presents an interesting and diagnostic challenge when encountered. Here are 5 tips to improve your diagnostic acumen for this febrile disorder. Read Post

Best of 2022: Myths & Misconceptions with Febrile Disorders

Rachel Tate, DO
Myth vs reality
Still’s disease and autoinflammatory disorders are rare conditions unified by recurrent fevers and inflammation of skin, joints, and other organs. While labs often support the diagnosis, these conditions are best diagnosed by genetic testing, or by specific criteria (in the absence of a genetic test). The following is a table that lists common misconceptions (myths) and their clarifying explanations. Read Post

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