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VEXAS may be more common than expected

Jack Cush, MD

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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Holes in TH17 cells release IL-1α on NLRP3 activation

EurekAlert!

Certain T cells can secrete cytokines that are normally part of the innate immune system, as researchers from the Leibniz Institute for Natural Product Research and Infection Biology (Leibniz-HKI) and an international research team discovered.

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ACR 2022 Appraisal, Praise & Critique (11.18.2022)

Jack Cush, MD

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). 

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Familial Mediterranean Fever mimics

Md Yuzaiful Md Yusof, MRCP(UK), PhD

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.

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Best of

ACR Best Abstracts - Day 3

Jack Cush, MD

The last day was jammed with important reports and research including the late breaking abstracts. 



 

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"Dry Synovitis" exist in juvenile arthritis pts? Study of 12 DS pts, says its different from Poly #JIA; onset 6.1 yrs, w/ polyarthritis, no palpable swelling, progressive LOM/contractures, neg ESR/CRP/RF/CCP, +MRI synovitis w/ osteochondral involvement.https://t.co/okGPCsIPNs https://t.co/XlFhgTtq5b

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Dr. John Cush RheumNow ( View Tweet)
Have a Rheumatology question or case for Jack Cush? Record it here and we may feature it on an upcoming podcast. Tell us your name and where you practice rheumatology. https://t.co/RaFKTqhQjN https://t.co/hH0NcpOoyx

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Dr. John Cush RheumNow ( View Tweet)
Causes of Enthesitis: SpA (AS, reactive arthritis, PsA, enteropathic arthritis, ERA), JIA, but also in RA, leprosy, trauma, OA, DISH, acromegaly, fluorosis, retinoid therapy, hypo- or hyper-PTH, POEMS syndrome, X-linked hypophosphatemia https://t.co/pff2OWBT5C https://t.co/7zm2la4GtD

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Dr. John Cush RheumNow ( View Tweet)

Ferritin may be more than biomarker in Stills, etc - may contribute directly to pathogenesis. Ferritin causes PMN activation & neutrophil extracellular traps (NETs). Could this be targeted therapeutically? https://t.co/yuvMaKqOa7 https://t.co/bjkEJvKH8d

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Dr. John Cush RheumNow ( View Tweet)
Ferritin may be more than biomarker in Stills, etc - may contribute directly to pathogenesis. Ferritin causes PMN activation & neutrophil extracellular traps (NETs). Could this be targeted therapeutically? https://t.co/tHbS6WtNWp https://t.co/rXfqgkij7k

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Dr. John Cush RheumNow ( View Tweet)

Ferritin may be more than biomarker in Stills, etc - may contribute directly to pathogenesis. Ferritin causes PMN activation & neutrophil extracellular traps (NETs). Could this be targeted therapeutically? https://t.co/42KeTbVsQb https://t.co/imLipzmf4R

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Dr. John Cush RheumNow ( View Tweet)

Differential Dx of hyperferritinemia (≥500 μg/) in critically ill ICU pts includes: sepsis, liver disease/failure, hematological malignancy, HLH. Single center study of 2583 patients https://t.co/yuqJcyLIP5 https://t.co/vHLqJGP4mv

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Dr. John Cush RheumNow ( View Tweet)
Nifty little read about dx and tx algorithms in monogenic autoinflammatory disease I think we need to start generating more diagnostic algorithms and "approach to" articles Working on a series for my fellows and have had a hard time finding material! https://t.co/DYmgSyjILi
Mike Putman EBRheum ( View Tweet)
IL-18 is a proinflammatory cytokine involved in innate & adaptive immune responses. IL-18 is a key mediator in autoinflammatory diseases, MAS, Still’s disease. Serum IL-18 levels may be useful in diagnosis and monitoring of disease activity https://t.co/1SXlODZcXc https://t.co/xtauaMMMos

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Dr. John Cush RheumNow ( View Tweet)

Anakinra in Pericarditis: A Systematic Review (comprehensive, full read) -Options include steroids, colchicine, NSAIDs, pleuropericardial window, IL-1 inhibitors. ANAKINRA - 11 studies, 259 pts, all showing significant rapid resolution. https://t.co/i0LRp4E1tH https://t.co/lGLjJRB1G9

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Dr. John Cush RheumNow ( View Tweet)

Review of the Rheumatic causes of Pericarditis and their management. Causes: SLE, RA, MCTD, Myositis, EGPA, GPA, IgG4-rel Dz, FMF, TRAPS, CAPS, AOSD, JIA, Sarcoidosis, Kawasakis, GCA, Takayasu's https://t.co/MQmkUe4Yey https://t.co/FSfrznoeBe

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Dr. John Cush RheumNow ( View Tweet)
Study of 172 febrile pts w skin rash + arthralgia; 112 Dx w/ #AOSD. Use of a "streamlined diagnostic process (looking @ neutrophilia, hyperferritinemia, high IL-18), shortened Dx time (2 vs 4 wks), earlier Dx and more remissions (85% vs 68%, p < 0.05) https://t.co/MSMq2XLeYA https://t.co/ECKkeVqTIW

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Dr. John Cush RheumNow ( View Tweet)
FROST study (First-line options sJIA Tx) - a prospective study to assess treatments in SJIA. 86% Rx w/ biologic (IL-1/IL-6 i) & 14% non-biologic Rx. oral GC used in 54% biologic & 90% of non-biologic. 1/2 non-biologic went onto biologics w/in 4 mos. https://t.co/8TMpEPgzOr https://t.co/N8laSs5rrZ

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Dr. John Cush RheumNow ( View Tweet)
Review of the Rheumatic causes of Pericarditis and their management. Causes: SLE, RA, MCTD, Myositis, EGPA, GPA, IgG4-rel Dz, FMF, TRAPS, CAPS, AOSD, JIA, Sarcoidosis, Kawasakis, GCA, Takayasu's https://t.co/h6zpTOIuyu https://t.co/Gy3Eaqz8sM

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Dr. John Cush RheumNow ( View Tweet)
Impressive free read and review of Neutrophils and Netosis in Autoimmune and Autoinflammatory disorders (Nature Reviews) from Kapland & Wigerblad at the NIH. https://t.co/w4cIwcQMkt https://t.co/ReA9xM2X4j

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Dr. John Cush RheumNow ( View Tweet)
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