Autoinflammatory

Stills,continuum

Pharmacologic Efficacy in Still's Disease

Jack Cush, MD

A systematic review of the effectiveness and safety of pharmacological treatments for adult-onset Still’s disease (AOSD) supports the use of either IL-1 or IL-6 inhibitors.

A metanalysis was performed using databases, trial

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Cutoffs for Systemic Juvenile Arthritis Disease Activity Score

Jack Cush, MD

An international cohort of children with systemic Juvenile idiopathic arthritis (Still's disease) validated the systemic Juvenile Arthritis Disease Activity Score 10 (sJADAS10), a disease activity measure distinguish patients with inactive disease, minimal disease activity, moderate disease

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BSR presented (at the 2024 annual BSR meeting) new guidelines for the management of Behçet's disease - how to manage skin lesions, mouth ulcers, genital ulcers, serious eye, CNS & vascular involvement https://t.co/SFM4NJ48fH

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Dr. John Cush @RheumNow( View Tweet )

British Society for Rheumatology (BSR) presented (at the 2024 annual BSR meeting) new guidelines for the management of Behçet's disease - how to manage skin lesions, mouth ulcers, genital ulcers, serious eye, CNS & vascular involvement https://t.co/d0bjKuubym https://t.co/lqoTeTGqZ7

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Dr. John Cush @RheumNow( View Tweet )
'Drug Rediscovery for Rare Immune Mediated Inflammatory Diseases’ - a 6 center consortium will study if JAKi (filgotinib) is effective and safe in 3 rare disorders -- Behcets, inflammatory myositis & IgG4 related disease. https://t.co/UyZM4VNSY2 https://t.co/D2D1tdygS4

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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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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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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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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 )
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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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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

Links:

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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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

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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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