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

d Jack Cush, MD

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 study reports that drug hypersensitivity and pulmonary reactions in Still's patients receiving IL-1 or IL-6 inhibitors is strongly associated with HLA-DRB1*15 haplotypes.

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ACR21 – Day 3 Report

d Jack Cush, MD

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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Infection or Disease Flare: What Tools Can Help Differentiate?

d Olga Petryna, MD

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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#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

Links:

LiSa ◡̈︎ rheumarhyme ( View Tweet)
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)
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
David Liew drdavidliew ( View Tweet)
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)
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)
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
sheila RHEUMarampa ( View Tweet)
The strawberry🍓gums of granulomatosis with polyangiitis! (Note: not always so florid) @RheumNow #ACR21 #RheumTwitter #MedTwitter https://t.co/qDfGJ5JeWz
sheila RHEUMarampa ( View Tweet)
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Opening Day Report

d Jack Cush, MD

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).

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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)
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)
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Blacks with Systemic Sclerosis May Have More Severe Disease

d ACR

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 be driven by several socioeconomic factors (Abstract #1854). This poster will be presented Tuesday morning,  November 9, 2021 in the Clinical Poster III (1836–1861) section on Systemic Sclerosis & Related Disorders.

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Management of Immune-Related Adverse Events - ASCO Guideline

d Jack Cush, MD

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

d ACR

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 #RheumNow https://t.co/294Au0Q1c3 https://t.co/c1X2T5G4jW

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Dr. John Cush RheumNow ( View Tweet)
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

Links:

Dr. John Cush RheumNow ( View Tweet)

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RheumNow Podcast – Quackademia (10.15.2021)

d Jack Cush, MD

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

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Dr. John Cush RheumNow ( View Tweet)

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