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Rare Diseases

PEXIVAS: Diffuse Alveolar Hemorrhage in ANCA-Associated Vasculitis Patients

A secondary analysis of the PEXIVAS study showed antineutrophil cytoplasmic antibody–associated vasculitis (AAV) patients complicated by diffuse alveolar hemorrhage (DAH) may have improved with plasma exchange and glucocorticoids, the results did not achieve significance. 

Diffuse

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Biologics Prevent AA amyloidosis progression to ESRD

MedPage Today

One of the most serious complications of rheumat0logic and autoimmune diseases can be prevented with biologic drugs, a single-center chart review suggested, and some such products may be more effective than others.

Among 83 patients receiving biologic agents for their underlying

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Best Rheumatologists (7.19.2024)

This week on the RheumNow Podcast, Dr. Jack Cush reviews the news and journal reports of interesting, including irAE, pollution and Psoriasis, microwave therapy, scleroderma without scleroderma that only the best rheumatologists could discern.

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Updated EULAR Recommendations on the Treatment of Systemic Sclerosis

Medscape

Medscape has published an overview to the 2024 updated recommendations for the treatment of systemic sclerosis (SSc) presented in Vienna at EULAR 2024 by Professor Francesco Del Galdo, MD, PhD on behalf of a 27 member task force.

The new recommendations (n=20)

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Systemic sclerosis sine scleroderma

Systemic sclerosis sine scleroderma (ssSSc), which accounts for nearly 10% of systemic sclerosis (SSc) patients and was first described in 1962, is a subset of SSc.

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PMR - a Risk Factor for Recalcitrant Giant Cell Arteritis?

Despite the clinical similarities and treatment differences between  giant cell arteritis (GCA polymyalgia rheumatica (PMR), confusion exists regarding the impact of overlapping disorders. A large cohort study shows that GCA patients with PMR symptoms are

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ICYMI: Best Imaging in Giant Cell Arteritis - US, PET, MRI?

Imaging is often instrumental in diagnosing and staging patients diagnosed with giant cell arteritis. A new study compared the diagnostic performance of Colour Duplex Ultrasound (CDUS), Fluor-18-deoxyglucose Positron Emission Tomography Computed Tomography and Magnetic Resonance Imaging in

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Review of Amyloidosis The New England Journal of Medicine has published a review of systemic amyloidosis. https://t.co/4507N4DwLk https://t.co/Q0MP5XXUHr
Dr. John Cush @RheumNow( View Tweet )

ICYMI: Hidradenitis Suppurativa-Related Autoinflammatory Syndromes

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 pyoderma

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New Treatments for Systemic Sclerosis

EurekAlert!

Autologous hematopoietic stem cell transplantation (aHSCT) for SSc has been proven the most effective treatment strategy with regard to overall and event free survival in selected patients.2 But a key limitation is its toxicity, and new treatment options are needed. Two abstracts

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Is Cannabis Safe? (6.7.2024)

Dr. Jack Cush reviews FDA approval, news and journal articles -- and it’s the week before EULAR 2024 in Vienna!!

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The Window of Opportunity (5.24.2024)

Dr. Jack Cush reviews the journal reports and news from the past week on RheumNow.com. This week: blockbuster drugs, opioids in rheumatology and nontreatment of inflammatory arthritis?

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"Don't You Know Who I am?" (5.17.2024)

Dr. Jack Cush reviews the news and journal reports from the past 2 weeks. This week's question: can we prevent gout, ILD or psoriasis?

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Pharmacologic Efficacy in Still's Disease

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

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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Imaging-Only Diagnosis of Giant Cell Arteritis Is Feasible

Medpage Today

Positive findings with color Doppler ultrasound were enough to diagnose giant cell arteritis (GCA) accurately without need for confirmation with temporal artery biopsy (TAB), a prospective study indicated.

Out of 165 older patients in whom GCA was strongly suspected, 73 (44%) had the

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Featured BSR Abstracts (5.2.2024)

Dr. Jack Cush muses on the news, journal reports, FDA announcements and the 2024 BSR abstracts just released.

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Methotrexate Does Not Increase ILD Risk in Dermatomyositis

A cohort analysis of dermatomyositis (DM) patients starting immunomodulators finds that methotrexate use was not associated with an increased risk of interstitial lung disease (ILD).

ILD affects ~23% of DM patients and the oft used MTX is has a low risk of pneumonitis and pulmonary

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Rheum Chapter Notes (4.26.2024)

Dr. Jack Cush reviews this past week’s news and journal articles from RheumNow.com.

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Canary in a Coal Mine (4.19.2024)

Dr. Jack Cush reviews regulatory reports, news and novel journal articles - this week focusing on Sjogren's, ILD, Gout and Uveitis.

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Burnout or Retire (4.12.2024)

Dr. Jack Cush reviews the news and journal reports from this past week on RheumNow.com. Is it time to worry about burnout and those who are leaving healthcare?

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ILD Onset in MDA-5 Dermatomyositis

Anti–melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis (DM) is a rare, aggressive and progressive subtype of DM. Its' severity is dependent on the development of progressive interstitial lung disease (ILD), manifesting as either rapidly progressive ILD (RPILD

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Neuropsychiatric SLE - 2/3 Improved at 12 Months

The management and outcomes of neuropsychiatric systemic lupus erythematosus (NPSLE) can be challenging as there is limited data. 

A retrospective cohort study identified 350 NPSLE events (between 1999-2015) defined by ACR definitions and categorized as being:

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Best Imaging in Giant Cell Arteritis - US, PET, MRI?

Imaging is often instrumental in diagnosing and staging patients diagnosed with giant cell arteritis (GCA).  A new study compared the diagnostic performance of Colour Duplex Ultrasound (CDUS), Fluor-18-deoxyglucose Positron Emission Tomography Computed Tomography (FDG-PET/CT) and

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Seasonal Pathogens and Henoch-Schönlein Purpura

Epidemiologic studies of Henoch-Schönlein purpura (HSP) have suggested seasonal variation in occurrence rates (higher from September-April, lower in June-August), suggesting a role for infectious triggers. The importance of infection was recently shown with nonpharmaceutical interventions (

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