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Rare Diseases

"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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1/600 Falls May Die (4.5.2024)

Dr. Jack Cush reviews the news and Journal reports from this past week on RheumNow.com -  including problems w/ falls, pain and treatment of Dupuytren’s contractures.

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Serious Infections in Takayasu arteritis

The large vessel vasculitis, Takayasu arteritis (TAK), carries a significant morbid risk and a cohort analysis shows these patients are also at high risk of serious infection with resultant higher risk of death.

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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 (MAS or death).

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Predicting Vasculitis Relapse

MedPage Today

Scores on a patient-reported sino-nasal symptom questionnaire were associated with subsequent flares of one type of ANCA-associated vasculitis (AAV), researchers said.

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Ides of March (3.15.2024)

Dr. Jack Cush has advice for Caesar and the superstitious, and covers pregnancy, platelets, dialysis and vaccinations in Lupus.





  1. Dutch study of 586 RA pts on bDMARDs found more nonserious infections (NSIE) (77/100PYs) than serious infx (SIE)(1.3/100Pys). Upper respiratory

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MMWR: Vaccination and Multisystem Inflammatory Syndrome in Children

CDC

Multisystem inflammatory syndrome in children (MIS-C) is a rare but serious condition typically occurring 2–6 weeks after SARS-CoV-2 infection and characterized by fever and multiorgan involvement.  The current MMWR report shows that MIS-C case numbers have decreased by 98% since 2021.

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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 

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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).



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

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Lyme Disease 2022

The CDC has updated its Lyme disease surveillance report based on a new case definition. Compared to 2017–2019, the number of cases in 2022 rose by 69%.

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19% Arthritis Prevalence in the USA

According to the National Health Interview Survey (NHIS), in 2022, the prevalence of diagnosed arthritis in adults was 18.9% (women 21.5%, more than men 16.1%). These numbers are down since the CDC's MMWR report of 2019–2021, where is was estimated 21.2% of U.S. adults (53.2 million) had

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Sniffles & Arthritis (3.1.2024)

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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Polyarteritis Nodosa: Clinical Features and Outcomes

A multinational study of polyarteritis nodosa (PAN) through the international GLOBAL-PAN network shows PAN to be a complex, multisystem disorder with a high risk of relapse and low risk of death (11% at 10 years).

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