Goofy But True

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Dr. Jack Cush discusses declining survival rates in the USA, FDA approvals of new COVID subvariant boosters and other odd and possibly true new research reports from the past week on RheumNow.com.

Genetic Testing for Autoinflammatory Disease

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Not all patients with periodic fevers fit neatly into diagnostic categories. Some can be diagnosed as Still’s disease (based on criteria) while others can be classified as autoinflammatory diseases (AID) and some may be unclassifiable, clinically or genetically.

Disease Activity Scoring in Adult-onset Still's disease

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Still's disease in adults (AOSD) or children (sJIA) can have dramatic symptom severity, making it easy to gauge disease activity and response to therapy, especially at the outset. However, a validated measure of disease activity has not been agreed (for clinical trial and treatment assessments). A new study compares two such activity measuresin a large cohort of Still's patients.

STING/IFN Activation Promotes ANCA-Associated Vasculitis Progression

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Research presented in the Journal of Experimental Medicine shows that ANCA-associated vasculitis (AAV) may be propagated by pathogenic mechanisms triggering cGAS/STING/IRF3-dependent IFN-I release.

Autoimmune Disease Augments Cardiovascular Risk

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A large UK database study suggests that young adults with autoimmune diseases have an associated increased risk for cardiovascular disease.

Influenza Vaccination Recommendations

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The CDC has published an update to the recommendations from the Advisory Committee on Immunization Practices (ACIP) on the use of seasonal influenza vaccines in the United States for the 2022-23 season.

Olokizumab, another IL-6 Inhibitor for RA

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This week's NEJM has published the efficacy results of a large phase 3 trial of olokizumab, a humanized monoclonal antibody that directly targets IL-6 in patients with rheumatoid arthritis.

This is in contrast to two other marketed IL-6 inhibitors (sarilumab, tocilizumab) that bind to the IL-6 receptor.

Bullous Systemic Lupus Erythematosus

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Bullous systemic lupus erythematosus (BSLE) is a rare blistering cutaneous manifestation of systemic lupus erythematosus (SLE).

As goes SLE, bullous disease typically affects women, especially those of African descent.

Managing JDM with Calcinosis

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Dr. Jack Cush reviews the news and journal reports from this past week on RheumNow and discusses a case of refractory juvenile dermatomyositis with calcinosis.

Low Dose IL-2 Therapy in SLE

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A multicentre, proof-of-concept trial of suggests that low-dose IL-2 therapy may be effective in moderate-to-severe systemic lupus erythematosus (SLE).

Less Organ Damage with Remission in SLE

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A cohort study of SLE patients shows that remission and low disease activity (LDA) are associated with less damage accrual over time.

Utility of Repeat ENA Antibody Testing

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Repeat serologic testing is not uncommon, but is it warranted? A retrospective study shows that repeated testing for autoantibodies to extractable nuclear antigens (ENA) rarely changes the result or adds a new diagnosis. 

CDC Reviews Monkeypox

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MMWR recently reviewed the epidemiology and characteristics of U.S. monkeypox cases seen between May and July 2022.

More Bad Safety News for Xeljanz

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Another analysis of the mandatory postmarketing safety study for tofacitinib (Xeljanz) has confirmed what most observers expected: that rates of infection with the drug in rheumatoid arthritis are higher than with tumor necrosis factor (TNF) inhibitors.

ACR Updated Guideline on Vaccinations for Rheumatic Patients

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The ACR has posted a new ACR Clinical Practice Guideline Summary providing recommendations on the use of vaccinations for children and adults with rheumatic and musculoskeletal diseases (RMDs).

The Approach to Difficult to Treat Rheumatoid Arthritis

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Tan and Buch have reviewed the approach to difficult to treat rheumatoid arthritis (D2T-RA), with a new EULAR definiation of D2T-RA to foster an approach rationale and concideration of treatment options.

Plasmacytoid Dendritic Cell Inhibitor in Cutaneous Lupus

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Litifilimab, a humanized monoclonal antibody against BDCA2, targets the BDCA2 receptor on plasmacytoid dendritic cells. When administered to patients with cutaneous lupus erythematosus (CLE) was shown reducing disease activity in CLE patients.

Approach to ILD in Myositis Syndromes

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Mehta et al have published a full read review of managing interstitial lung disease (ILD) in patients with inflammatory myopathies, a heterogeneous group of syndromes connected by ILD and and increased morbidity and mortality risk.

Rheumatic Causes for Fever of Unknown Origin

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Fever of unknown origin (FUO) represents a diagnostic challenge to many physicians and while cancer and infectious causes need to be excluded, rheumatic disorders are amongst the most common causes of FUO.

Mitochondria as Master Regulators of Inflammation

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Mitochondrial constituents and metabolic products may give rise to immune activation; this is especially true for mitochondrial DNA, which can function as damage-associated molecular patterns (DAMPs) leading to inflammasome activation.