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Rare Diseases

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Hope for Biologic Treatment of Hidradenitis Suppurativa

Feb 01, 2023

A meta-analysis of trials using biologics [adalimumab (ADA), ustekinumab (UST) and secukinumab (SEC)] in the the management of hidradenitis suppurativa (HS) suggests promising results, but more controlled, rigorous studies are needed.



While only adalimumab is currently FDA approved

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Simon Says “Don’t Do This” (1.27.2022)

Jan 27, 2023

Dr. Jack Cush reviews this week’s journal articles, focuses on studies that should not have been done and responds to viewer questions in Ask Cush Anything.




  1. "Dry Synovitis" exist in juvenile arthritis pts? Study of 12 DS pts, says its different from Poly #JIA; onset 6.1 yrs

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rare

VEXAS may be more common than expected

Jan 25, 2023

A great study of a recently described rheumatologic/hematologic disorder VEXAS (vacuoles, E1-ubiquitin-activating enzyme, X-linked, autoinflammatory, somatic) syndrome recently appeared in JAMA, describing the frequency and presentations of UBA1 variant. 

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2022,wordle

2022 Rheumatology Year in Review

Jan 05, 2023

2022 was a year of recovery, discovery, and some losses.  While still dealing with the aftermath of COVID-19, rheumatologists were resilient as leaders for their patients and research in advancing rheumatologic care.

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GCA,woman

In Giant Cell Arteritis, Hospital Admission Is Bad News

MedPage Today
Jan 03, 2023

One-third of patients admitted with giant cell arteritis (GCA) were rehospitalized within 6 months, largely because of complications potentially related to corticosteroid therapy, researchers found in a retrospective cohort study.



Among the cohort of 1,206 patients hospitalized with

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JAK Inhibitor Potential in Systemic Sclerosis-Associated ILD

Dec 15, 2022

The occurrence of interstitial lung disease (ILD) in Systemic sclerosis portends serious morbid and mortal outcomes for those affected. This review examines the available clinical literature on the potential benefits and outcomes of JAK inhibitor use in SSc=ILD.



Overall the authors

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GCA,woman

2022 ACR/EULAR Giant Cell Arteritis Classification Criteria

Dec 12, 2022

Giant cell arteritis (GCA) has historically been a clinical diagnosis (visual loss, headache, jaw pain, scalp tenderness, etc) supported by labs and histopathology. Yet the diagnosis and classification of GCA has evolved, so much so that the ACR and EULAR have updated the 1990 Classification

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Inflammatory Arthritis and Hidradenitis Suppurativa

Dec 09, 2022

Hidradenitis suppurativa (HS) (also called acne inversa), is a chronic inflammatory skin disorder affecting apocrine gland-bearing skin in the axillae, groin, and under the breasts and is characterised by persistent or 

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RECITAL Trial: RTX vs. CTX in CTD-ILD

Dec 01, 2022

A UK study suggests the equivalent outcomes when patients with interstitial lung disease (ILD) associated with connective tissue disease (CTD) are treated with either intravenous rituximab (RTX) or cyclophosphamide (CTX), but with fewer adverse events with RTX. 

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ACR 2022 Appraisal, Praise & Critique (11.18.2022)

Nov 18, 2022

It was great to be back at an ACR annual meeting, this one in Philadelphia - Philly was great. A walking town, rich in culture, history and good places to meet and mingle. 



I loved the city, but not the convention center (not easy to navigate). 



The meeting itself was very

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Thermometer

Familial Mediterranean Fever mimics

Familial Mediterranean fever (FMF) is an inherited autoinflammatory disease characterized by recurrent attacks of fever and acute inflammation of the membranes lining the abdomen, joints and lungs. These attacks are often short-lasting, around 1-3 days. It is important to recognise these

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Most VEXAS pts have pulm involvement on HRCT. Pulmonary manifestations of VEXAS are nonspecific and characterized predominantly by inflammatory parenchymal involvement. Abs 1569 #ACR22 @RheumNow https://t.co/7AB3b9e7Lt https://t.co/ikhQMEu5GA
VEXAS is more common than you think in men >50 #ACR22 https://t.co/z2T6nBoI9a
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Women with Rheumatic Disease (11.4.2022)

Nov 04, 2022

Dr. Jack Cush reviews the news and journal articles from this past week on RheumNow.com.  New regulatory decisions about JAK inhibitors and why do women with rheumatic diseases have unfair outcomes?




  1. Population study shows among vaccinated gout pts, COVID breakthrough

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DPGN, lupus, nephritis, GN, glomerulonephritis, biopsy, kidney

Increasing Risk of Lupus Nephritis

Oct 25, 2022

Population based study from Minnesota shows that the incidence of lupus nephritis (LN) in the US has increased in the last four decades and that LN patients have poorer outcomes with high rates of ESRD and mortality rates six times that of the general population.



Cases of incident LN

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Spondyloenchondrodysplasia - Very rare genetic skeletal dysplasia w/ short stature, platyspondyly, short broad ilia and enchondromas in long bones or pelvis. Varied phenotype w/ CNS (spasticity, retardation) & autoimmune (ITP, SLE, AIHA, thyroiditis) https://t.co/4DHqohjb2Z https://t.co/Bafh1v0MOA
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ACR Award Winners (10.21.2022)

Oct 21, 2022

Dr. Jack Cush reviews the news and journal reports from the past week on RheumNow.com. Highlighted discussion of implantable stimulators, urine proteomics and gut-immune responses to Prevotella in RA.




  1. Vaccination Cut Long COVID Risk for Rheum Patients Patients with

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Miner,coal,newcastle

Miners Face Increased Risk of Rheumatoid Arthritis

MedPage Today
Oct 17, 2022

Add "hard rock" mining to the list of occupations associated with high rates of rheumatoid arthritis (RA), a new study indicated, with silica exposure the presumed malefactor.



Based on survey data from men ages 50 and older in portions of three Western states, odds of developing RA

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PSS.scleroderma.arthritis.contractures

Tofacitinib shows promise in scleroderma

EurekAlert!
Oct 07, 2022

Systemic sclerosis is one of the rarest autoimmune diseases, affecting roughly 100,000 people (primarily women) in the U.S. With no licensed treatments available for this subset of scleroderma patients, rheumatology researchers are searching for opportunities to use resources and technology that

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RheumNow Podcast – “That’s Not My Name” (10.7.2022)

Oct 07, 2022

Dr. Jack Cush goes off on "Untitling" (not using proper names/titles) in the clinic, in addition to reports on rheumatologist salaries, new drugs in phase 1-3 development and the range of COVID-19 MSK symptoms -- all from the past week on RheumNow.com.




  1. Microbiome analyses of

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death

Causes of Death in Lupus

Oct 03, 2022

The California Lupus Surveillance Project has longitudinally followed included systemic lupus erythematosus (SLE) cases and shown that overall, the leading cause of death was cardiovascular disease (CVD), followed by rheumatic disease and hematologic/oncologic conditions.

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Heavy Metal Rheumatology (9.23.2022)

Sep 23, 2022

Below are this week’s highlights from RheumNow, as discussed by Dr. Jack Cush. Thanks to all of you for your kind comments and great reviews of our weekly podcast. Please let us know how we can improve by emailing me or recording your suggestion using the "Ask Cush Anything" link on our website

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Cancer Risk with Scleroderma

Sep 22, 2022

Spanish Scleroderma Registry examined a large cohort of systemic sclerosis (SSc) patients showing an increased cancer risk, especially breast, lung, hematologic and colorectal cancer.

The registry included 1930 patients with SSc and were compared to a matched general population to derive the

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Is PMR the Next Indication for Tocilizumab?

MedPage Today
Sep 21, 2022

About half of patients with steroid-dependent polymyalgia rheumatica (PMR) were able to get off steroids altogether when they were started on tocilizumab (Actemra) infusions, which also improved disease control in most cases, a randomized phase III trial showed.



In a 24-week study, 49

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antibody (Keep)

Clinical Profiles Seen with NXP-2 Antibodies

Sep 20, 2022

A recent review of the myositis associated autoantibody NXP-2 profiles its clinical associations with dermatomyositis (DM), calcinosis, severe myositis and, in some reports, with cancer.

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