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Rare Diseases

Full read review of Pathogenic autoantibodies in idiopathic inflammatory myopathies: antisynthetase autoAbs, TIF1, MDA5, SRP, HMGCR, TRIM72, cN1A, FHL1, eIF3 autoantibodies and more https://t.co/DSov27xdrT https://t.co/58wKHGMneH
Dr. John Cush @RheumNow( View Tweet )
Pneumocystis Jirovecii Pneumonia Complicating Systemic Vasculitis A retrospecitive analysis of 62 patients with systemic vasculitis complicated by Pneumocystis jirovecii pneumonia (PJP) showed those with MPA had a higher mortality risk, possibly due to a higher prevalence of… https://t.co/vZHZbfqlbB https://t.co/bn2oTAPSnc
Dr. John Cush @RheumNow( View Tweet )

Stratified Cancer Screening in Dermatomyositis

The International Myositis Assessment and Clinical Studies Group (IMACS) have published  evidence and consensus based cancer screening guidelines for patients with idiopathic inflammatory myopathies, including dermatomyositis. 

They proposed 18 final recommendations - 13 were

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Pneumocystis Jirovecii Pneumonia Complicating Systemic Vasculitis

A retrospecitive analysis of 62 patients with systemic vasculitis complicated by Pneumocystis jirovecii pneumonia (PJP) showed those with microscopic polyangiitis (MPA) had a higher mortality risk, possibly due to a higher prevalence of interstitial lung disease (ILD) in MPA.

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British Society Guidelines for Managing Behçet’s Disease

An exhaustive, full read guideline from the British Association of Dermatologists (BAD) and the British Society for Rheumatology (BSR) on the management and treatment of Behçet’s disease (BD) in 2024 has been published in Rheumatology.

This guideline provides up-to-date,

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I Can't Treat Ugly (9.20.2024)

Dr. Jack Cush reviews the news, journal articles and a new FDA approval for EGPA, this week on the Podcast.

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BSR Guidelines for Systemic Sclerosis Management

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

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Different Autoantibody Phenotypes in Juvenile and Adult Myositis

A Kyoto University study compared juvenile-idiopathic inflammatory myopathy (IIM) and adult-IIM patients, demonstrating different autoantibody profiles and a more favorable outcome for n juvenile-IIM patients.

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Systemic Sclerosis Insights (9.13.2024)

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

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Increasing Incidence of Morphea

A large EMR derived cohort study of over 10,000 patients with morphea (localized scleroderma) sheds light on its prevalence and associated features.

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Cross-sectional study 69 Systemic Sclerosis Pts: 33% w/ PPI-refractory esophagitis & 2/3 w/ EGD normal esophageal mucosa. PPI-refractory esophagitis was more commonly linked to diffuse SSc subset (43 vs 17%) and GI dysmotility (66 vs 8%; p = <0.001) https://t.co/givt90C0tE https://t.co/3oN5qUHpUU
Dr. John Cush @RheumNow( View Tweet )

Linger on the Fingers (9.6.2024)

Dr. Jack Cush reviews the news and journal reports from the past week on RheumNow.com. This week with a focus on fingers and better prescriptive follow through.

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Urticarial Vasculitis Overview

Urticarial vasculitis is rare and the causes often go undiagnosed. A Medscape recent overview highlights key features in the diagnosis and management of this rare disorder.

Urticarial vasculitis is often chronic, or recurrent, accompanied by a painful or burning sensation. The wheals or

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Immunomodulatory Prevention of Uveitis Relapse in Behçet's

The results of a randomised, open-label, head-to-head trial shows that adalimumab was superior to ciclosporin in preventing uveitis relapses in patients with severe Behçet's disease.

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VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome - genetic disease with estimated prevalence of 1 in 4269 in men >50 year: systemic inflammation, progressive bone marrow failure, and inflammatory cutaneous manifestations https://t.co/hSmE64xzcU https://t.co/gWh4rvaaS3
Dr. John Cush @RheumNow( View Tweet )

Higher Cancer Rates (8.16.2024)

Dr. Jack Cush reviews the news and journal reports from the past week on RheumNow.com, noting his concerns about diet, cancer, ILD, death and dosing challenges.

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Hispanic Patients With Dermatomyositis

JAMA Dermatology has published a review of dermatomyositis (DM), focusing on similar and different manifestations of DM.

DM affects all races, but it has been suggested that racial and ethnic minority patients may have more severe disease and a different autoantibody profile. This analysis

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Depends on Where You’re Looking (8.9.2024)

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

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Relapses with Rituximab Maintenance in ANCA-associated Vasculitis

Real-world, retrospective study of microscopic-polyangiitis (ΜPA) and granulomatosis-with-polyangiitis (GPA) patients shows that rituximab (RTX) maintenance was associated with 25% risk of relapse and that lower relapse rates was seen with RTX plus cyclophosphamide treatment.

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CARRA Consensus Treatment for Refractory Juvenile Dermatomyositis

CARRA has developed consensus treatment plans for the use of biologic disease-modifying antirheumatic drugs (bDMARDs) in patients with refractory, moderately severe juvenile dermatomyositis. Juvenile dermatomyositis is the most common form of idiopathic, inflammatory myositis in childhood, with

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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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