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Autoinflammatory

"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
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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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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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
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
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

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

EurekAlert!
Jan 10, 2023

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. They have thus revealed several previously

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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

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
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

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

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

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
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
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
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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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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Best of

ACR Best Abstracts - Day 3

Nov 14, 2022

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



 

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#abst1679 #acr22 @rheumnow CARRA STOP-JIA early introduction of bDMARD beneficial in polyarticular JIA: in early combination of cDMARD+biologic group > pts able to achieve Clinical Remission& significantly longer T spent in CID& cJADAS10 ID during the 3 year study vs step up Rx https://t.co/xwQJnSeCNE
#ACR22 Abstr#1830 Short attack of arthritis episode is common in Familial Mediterranean Fever. Other pointers? A cohort study in Turkey showed to consider FMF in pts with red mono-oligo arthritis in lower limbs & spondyloarthropathy. 20% were colchicine resistant @RheumNow https://t.co/JXGcgCrhPu
#ACR22 Abstr#1839 "Doctor, I have fever & recurrent right-sided tummy pain, is it my appendix?" A case-control study in Turkey reported 16% of unindicated appendectomies prior to FMF diagnosis. This group had late diagnosis and associated with M694V homozygous mutation @RheumNow https://t.co/QtxjuHDXYC
Out work showing JIA pts with sharp decline in needing TKAs, however increased TKA utilizations for tumors!! We are treating inflammatory arthritis better over timein the pediatric population...Natiowide data over 15 years! #ACR22 @Rheumnow abst# 0843 https://t.co/inw41Y1o9r
Moura et al. 22 case series of VEXAS. Lung involvement common, Symptoms in 95%. HRCT abnormal 73%. PFTs abnormal 20%. Changes non specific inflammatory parenchymal, steroid responsive. @RheumNow #ACR22 Abstr#1569 https://t.co/E6vUnvxSdQ https://t.co/l4aEmpy8OX
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