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Rare Diseases

Lung Involvement in Still's Disease

A multicentre registry observational study assessed lung involvement in a cohort of Still’s disease patients, affirming pleuritis as most common and that parenchymal lung inflammatory disease is very uncommon in both children and adults.

A total of 90 Still's patients with lung

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Fallen Angels (1.17.2025)

Dr. Jack Cush reviews the news and journal reports from the past week on RheumNow.com; and pays homage to colleagues who passed away in 2024.

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Childhood Maltreatment and the Risk of Rheumatic Diseases

EurekAlert!

A UK retrospective matched open cohort study shows that children who suffered frmo abuse, neglect or domestic abuse carry a significantly increased risk of developing rheumatoid arthritis or psoriasis in later life.    

Researchers used Dexter software and over 16 million

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Outcomes of Multisystem Inflammatory Syndrome in Children (MIS-C)

A nationwide cohort study of patients with multisystem inflammatory syndrome in children (MIS-C), a life-threatening complication of COVID-19 infection, showed that many had severe disease during the acute phase, but most recovered quickly and had a reassuring midterm prognosis.

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EULAR: Considerations when Transitioning from Pediatric to Adult Rheumatologic Care

EULAR has established guidance on best practices for delivering patient education in physical activity and self-management of pain during transitional care in rheumatology.

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Prevalence of Autoimmune Diseases in the United States

An epidemiologic assessment of the prevalence of autoimmune diseases in the United States (US) finds that nearly 5% of the population has an autoimmune disorder, twice as many in women compared to men.

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ICYMI: VITAL Info on Autoimmune Disease (2.9.2024)

Dr. Jack Cush reviews the journal reports from the past week on RheumNow.com. Highlights are summarized by three songs: "Stairway to Heaven", "You're No Good", and "How Long (has this been going on)".

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Best of 2024: BSR Guidelines for Systemic Sclerosis Management

The British Society for Rheumatology has updated its 2015 guidelines for the management of patients with systemic sclerosis (SSc) based on published evidence, systematic literature review and expert opinion.

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Bah Humbug Vitamin D (12.20.2024)

Dr. Jack Cush reviews the news and journal reports from this past week on RheumNow.com.

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54K Peptic Ulcers Annually (12.13.2024)

Dr. Jack Cush reviews the news and journal reports from the past week on RheumNow.com

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Drug-Free Remission in Giant Cell Arteritis is Uncommon

A Spanish retrospective registry study of patients with giant cell arteritis (GCA) found that 3-4 years after diagnosis, only 21% of patients with GCA successfully reached the sustained drug-free remission (SDFR). But if SDFR was achieved, the likelihood of experiencing recurrence was

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Way Behind in Pediatric Rheumatology

While adult rheumatology fellowships have surged in popularity and become quite competitive, more than half of pediatric rheumatology fellowship positions went unfilled in 2024, according to the National Resident Matching Program (NRMP). 

According to Medscape, across all 39

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Chikungunya marches on, at a cost

A recent article from BMC Global Health suggests that between 2011 and 2020, the mosquito-borne disease chikungunya, has disabled millions and likely amassed close to $50 billion in healthcare and disability-related costs in 110 countries. 

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FDA has granted Rare Pediatric Disease Designation (RPDD) to a new proprietary investigational drug, 101-PCG-005, a type IA prodrug of dexamethasone targetsing CD206+ macrophages. The intended use is in systemic juvenile idiopathic arthritis (sJIA) or Still's disease .… https://t.co/ErNWlva9Nd https://t.co/gc8cPNs3H1
Dr. John Cush @RheumNow( View Tweet )
Inebilizumab for Treatment of IgG4-Related Disease Inebilizumab is a monoclonal antibody that depletes CD19+ B cells and has been studied in adults with IgG4-related disease. In a phase 3, double-blind, randomized, placebo-controlled trial, it reduced IgG4-related disease… https://t.co/7PAmCn9O2I https://t.co/NmNA1p6GmI
Dr. John Cush @RheumNow( View Tweet )

Don’t Buy Guitars (12.6.2024)

This year at ACR24, the RheumNow faculty and reporters were prolific, generating >800 Tweets, 56 written articles, 117 Videos and 28 podcasts – all in 4 days! You can best review and learn ACR24 by A) Rheumatology Roundup; b) Topic Panel videos; and c) Topic podcasts.

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Inebilizumab for Treatment of IgG4-Related Disease

Inebilizumab is an monoclonal antibody that targets and depletes CD19+ B cells and has been studied in adults with IgG4-related disease. A phase 3, double-blind, randomized, placebo-controlled trial, and was shown to reduce IgG4-related disease
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Autoantibodies, found in over half of adult and paediatric patients with idiopathic inflammatory myopathies (IIMs), these correlate with clinical phenotypes, aiding in classification and prognosis. https://t.co/ZRXqtEec6s https://t.co/jkUe9bRlmU
Dr. John Cush @RheumNow( View Tweet )
@DrLisaCS distills the challenges of MCTD 👇 🔵Clinical phenotyping key to improve patient outcomes @jhrheumatology @RheumNow #acr24 https://t.co/APfXPwxvfE

Caoilfhionn Connolly @CaoilfhionnMD( View Tweet )

Sullivan et al. Vasculitis in VEXAS. 81 patients. 27% vasculitis. Cutaneous SVV 20%, AAV 2.5%, cutaneous MVV 2.5%, LVV 2.5%. @RheumNow #ACR24 Abstr#2651 https://t.co/AV3RcuSKpV https://t.co/xrax025TzP
Richard Conway @RichardPAConway( View Tweet )
A#2649 Relapsing Polychondritis Latent Class Analysis to define clinical phenotypes of heterogeneous dx Type 1- ear, nose, subglottic stenosis 2- tracheo/bronchomalacia 3- absence tracheomalacia Decision tree to classify pts in subgroups #ACR24 @RheumNow https://t.co/xpZedPVl7f
Eric Dein @ericdeinmd( View Tweet )
A#2647 DADA2 cohort 101 pts in India Median delay dx 5 years - 5 pts of >20 yr - 39 pts w GI involvement- bad outcomes Skin most common - largely livedo, then ulcers/nodules CNS common w ischemia Many Rx before dx 12 deaths (11.8%) - 8 GI, 3 CVA, 1 suicide #ACR24 @RheumNow https://t.co/Q8kiVbJq8I
Eric Dein @ericdeinmd( View Tweet )
Late-Breaking Abstract L07 The CLASS Project introduces new global classification criteria for Anti-Synthetase Syndrome (ASSD). Using the gold standard dataset, the key findings were: ✅ Definite ASSD: Sensitivity 94.3%, Specificity 99.7% ✅ Probable ASSD: Sensitivity 97.5%,… https://t.co/PtciIMTSsu https://t.co/jvJv2VNvHx
Antoni Chan MD (Prof) @synovialjoints( View Tweet )
On now: The Great Debate - is MCTD a distinct clinical entity? @DrLisaCS reviews the history of MCTD classification criteria and its evolution Kasukawa criteria may be most sensitive in one cohort @RheumNow #ACR24 https://t.co/rDHafZVL1I
Brian Jaros, MD @Dr_Brian_MD( View Tweet )
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