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Rare Diseases

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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EGPA: Benralizumab or Mepolizumab

A multicenter, double-blind, phase 3, randomized, active-controlled trial has shown that Benralizumab (BEN) was noninferior to mepolizumab (MEP) for the induction of remission of patients with relapsing or refractory Eosinophilic granulomatosis with polyangiitis (EGPA).



Adults with

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Behçet’s Syndrome

A comprehensive overview of Behcet's syndrome appeared in last week's NEJM, reviewing the numerous advances in the immunogenetics, syndrome recognition, and targeted therapeutic approaches. Despite certain advances, there remains a need for better clinical trials, better diagnostic tools, and

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Immunosuppression Increasing (2.23.2024)

Dr. Jack Cush reviews the news and journal reports from the past 2 weeks on RheumNow.com; but begins with a discussion of what constitutes "immunosuppression" and how it applies to our patients.





  1. 2021 NHIS Survey data suggests the prevalence of immunosuppression in the USA

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CD19 CAR T-Cell Therapy in Autoimmune Disease

The NEJM has published the results of CD19 B cell depletion (by CAR-T cell therapy) in autoimmune disease patients, demonstrating it to be feasible, safe, and efficacious.



The rationale to CD19 CAR-T cell transfer is to reset aberrant autoimmunity via deep depletion of B cells with

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Curbside Consults (2.16.2024)

Dr. Jack Cush answers audience clinical case questions sent by email or "ask Cush anything". Cases and questions from:




  • Dr. William Hong - RA management in the setting of active TB or latent TB (LTBI)

  • Dr. Vrishali Dalvi - relapsing polychondritis monitoring

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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)".





  1. Many #RA pts remain on Steroids despite b/tsDMARD use! Study 228 #RA pts

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