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Rare Diseases

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Cytokine Inhibitor Lung Disease in Still's Linked to HLA-DRB1 Alleles

Dec 01, 2021

Pulmonary complications in the setting of Still's disease (juvenile and adult) has seldom but consistently been reported, including reports of pneumonitis, pulmonary hypertension and a severe or fatal lung disease, often associated with hypersensitivity to anticytokine therapy. A multicenter

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ACR21 – Day 3 Report

Nov 09, 2021

Hitting the home stretch, day 3 presentations were big! Here are a few of my favorites: secukinumab efficacy in juvenile PsA; VEXAS - predicting poor outcomes; and the FDA Safety Update session.

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#ACR21 #IgG4 Q&A [Drs Stone/Perugino] 📌܄Igg4 in the blood/tissue is NOT diagnostic bec it can also be seen in others (malignancy..etc) 📌its more the presentation/ organ involvement see acr/eular guide: https://t.co/xBvu4r0TM3 https://t.co/WkiF6KyxGq
LiSa ◡̈︎ @rheumarhyme( View Tweet )
Nov 08, 2021
Calcium hyperproficiency is a rare complication of vitamin D deficiency, where excess calcium from bone resorption forms complex structures resembling primary teeth. Neoformed teeth grow on different bones, such as this patient's hand, causing fractures and joint dysfunction. https://t.co/oGYCYkh1WD
Eduardo Valdés-Hevia 🦟 @Valdevia_Art( View Tweet )
Nov 08, 2021
Very high serum IgG4 does not equal IgG4-related disease. Most of the time (3/4 in this sample), IgG4>5xULN is IgG4-RD. Some of the time, it's not. (Could be a long list of things) Criteria are useful High serum IgG4 alone isn't enough @zach_wallace_md #ACR21 ABST1103 @RheumNow https://t.co/oxMgnnpIce
Nov 08, 2021
Completely new one for me, Dr Taniguchi presenting on using ear ultrasound to diagnose and monitor disease activity in relapsing polychondritis. Appears useful, I suspect in clinical practice more so for diagnosis. Abstr#0169 #ACR21 @RheumNow https://t.co/PLJtkYkP3A
Richard Conway @RichardPAConway( View Tweet )
Nov 08, 2021
Lab work

Infection or Disease Flare: What Tools Can Help Differentiate?

Nov 08, 2021

Differentiation between a flare of disease and infection in patients with autoinflammatory (AI) conditions, where fever is the hallmark, can be extremely difficult. Few studies have evaluated potential differentiators; and having such tools would meet a huge unmet need. 

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Aware of COPA syndrome? ➡️Rare cause of immune-mediated lung dz ➡️Mimics RA-ILD, SLE, & vasculitis ➡️Adult-onset cases reported Abst#0529 determined that mutations in the COPA gene underlie some cases of severe pulmonary fibrosis. #ACR21 @RheumNow https://t.co/mCx3eT7Pv8
Meral K. El Ramahi, MD @MeralElRamahiMD( View Tweet )
Nov 07, 2021
Clinical pearls on Erdheim-Chester disease: ➕"Hairy Kidneys" on CT ➕"Coated" aorta or periaortitis ➕PET scan can confirm diagnosis - affects distal long bones ➕Refer to Oncology for treatment - it's a histiocytic neoplasm!🤓 @RheumNow #ACR21 #MedTwitter https://t.co/MsIqk1rWvE
Nov 07, 2021
The strawberry🍓gums of granulomatosis with polyangiitis! (Note: not always so florid) @RheumNow #ACR21 #RheumTwitter #MedTwitter https://t.co/qDfGJ5JeWz
Nov 07, 2021
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Opening Day Report

Nov 06, 2021

The opening of ACR2 Convergence was a hit for all who signed up and viewed in. The day included the presidential address by outgoing president Dr. David Karp (UT Southwestern) and a keynote talk and interview with Dr. Seema Yasmin (Stanford).



The Year in Review

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Dr. Marvisi presented a case: refractory MPA relapsed w/prednisone < 25 mg/day. Genetic studies c/w VEXAS. Is MPA/vasculitis part of VEXAS? The experts say: Maybe- VEXAS is such a new entity, there may be a common pathway (e.g NFkB affected by ubiquination, IL-6 role) #ACR21 https://t.co/kEOsruJ8DE
TheDaoIndex @KDAO2011( View Tweet )
Nov 06, 2021
VEXAS a new genetic disorder, (V) Vacuoles, (E) E1-enzyme, (X) X-linked, (A) Autoinflammatory, (S) Somatic. These are the important clinical features to be looking out for in clinic @RheumNow #ACR21 Session6S410 L Wilson, DO Cordona https://t.co/dBBLox6vVg
Dr. Antoni Chan @synovialjoints( View Tweet )
Nov 06, 2021
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Blacks with Systemic Sclerosis May Have More Severe Disease

ACR
Nov 02, 2021

New research presented this week at ACR Convergence, the American College of Rheumatology’s annual meeting, shows that Black people living with systemic sclerosis may have more severe disease and worse prognosis than patients in other racial or ethnic groups, and these worrying disparities may

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Management of Immune-Related Adverse Events - ASCO Guideline

Nov 02, 2021

The American Society of Clinical Oncology, Inc (ASCO) has updated its guidelines and recommendations for the management of immune-related adverse events (irAEs) in patients treated with immune checkpoint inhibitor (ICPi) therapy. Below are select statements from this guideline.

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Clues to Survival and Mortality with VEXAS Syndrome

ACR
Nov 01, 2021

New research to be presented this week at ACR Convergence, the American College of Rheumatology’s annual meeting, shows a relationship between genotype, bone marrow failure and survival in patients with the rare autoinflammatory disease VEXAS syndrome, which was only identified in 2020.

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Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Insights Read the full article on #RheumNowhttps://t.co/294Au0Q1c3 https://t.co/c1X2T5G4jW
Dr. John Cush @RheumNow( View Tweet )
Oct 22, 2021
Kikuchi’s Dz (AKA Kikuchi-Fujimoto dz) is rare, affects young women >men w/ recurrent necrotizing lymphadenitis (may assoc w/ SLE, MAS, TB); Sx included fever, flu-Sxs, Wt loss, night sweats, Hi CRP/LFTs, lymphopenia, uncommon arthritis, organomegaly https://t.co/LpEOgbcfpP

Dr. John Cush @RheumNow( View Tweet )

Oct 18, 2021
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RheumNow Podcast – Quackademia (10.15.2021)

Oct 14, 2021

Dr. Jack Cush reviews the FDA approvals, news and the latest journal articles from the past week on RheumNow.com.

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Decline in the incidence of Behcets Dz? Between 2004 to 2017 Korea has seen BD decreased from 8.15/100K (2004) to 1.51/100K (2017), an 81.5% decrease, greater for women and middle-aged people, but no change survival or mortality https://t.co/tvZSSGMOH7

Dr. John Cush @RheumNow( View Tweet )

Oct 14, 2021
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Still’s Disease Following COVID-19 Vaccination

Oct 14, 2021

Both systemic onset juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease are acquired autoinflammatory disorders of uncertain etiology. Yet recent reports of AOSD onset and flare following COVID-19 vaccinations may have important mechanistic implications.

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Clinical Associations with Myositis Antibodies

Oct 07, 2021

While myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies (MAAs) may have prognostic value through their clinical associations, many patients will not have them and overall, they are poorly predictive of a malignancy risk. 

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Lupus Frailty Index Predicts Organ Damage

MedPage Today
Oct 05, 2021

Patients with longstanding systemic lupus erythematosus (SLE) who were considered frail were more likely to accrue disease damage over time, a secondary analysis of data from a Chicago lupus cohort found.



On a multivariate analysis that controlled for age, race, and duration of

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Early Antibiotic Use Increases JIA Risk

Sep 28, 2021

A population based Swedish study of birth records shows that antibiotic exposure in first three years of life to be associated with a future risk of juvenile idiopathic arthritis (JIA), although infections during fetal life or childhood did not increase risk of JIA.

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Post-Arthroscopic Increase in Septic Arthritis

Sep 27, 2021

A longitudinal study from Iceland shows an increasing number of native joint infections (NJI), with iatrogenic, postarthroscopic infections being of particular concern.



Between 2003-2017 they identified 299 NJI (40 children and 259 adults) with more infections in men (33% women vs 67

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