Abstract Number: 0305 • ACR Convergence 2024
Inflammatory T Cell Expansion in Yao Syndrome
Background/Purpose: Yao syndrome (YAOS, #OMIM 617321), formerly known as nucleotide-binding oligomerization protein containing 2 (NOD2)-associated autoinflammatory disease, is associated with specific NOD2 mutations and affects…Abstract Number: 0922 • ACR Convergence 2024
Generation and Pathophysiological Analysis of M694I Variant Knock-in Mice of Human MEFV Gene: Insights from Single-Cell RNA Sequencing
Background/Purpose: The primary objective of this study was to generate knock-in mice with the M694I variant of the human MEFV gene, a critical variant in…Abstract Number: 1705 • ACR Convergence 2024
Deep Phenotyping Characterization of Peripheral Natural Killer Cells Reveals Impaired Cytotoxicity and Exhaustion During VEXAS Syndrome
Background/Purpose: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is a severe auto-inflammatory disorder associated with acquired mutations in the UBA1 gene that occur in hematopoietic stem cells…Abstract Number: 2030 • ACR Convergence 2024
The Development of the EULAR Score for the Definition of Disease Activity in Adult-onset Still’s Disease; The “DAVID” Project
Background/Purpose: Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown aetiology usually affecting young adults. Despite the evidence increasingly reporting the efficacy…Abstract Number: 2509 • ACR Convergence 2024
Bone Marrow Failure in VEXAS Is Associated with the Degree of UBA1b Deficiency
Background/Purpose: VEXAS is a multisystem autoinflammatory disorder due to somatic mutations in UBA1. The disease is characterized by ineffective hematopoiesis with increased risk of transfusion…Abstract Number: 0316 • ACR Convergence 2024
Development and Evaluation of the Adult-onset Still’s Disease Activity Index Based on Whole RNA-seq Analysis: A Novel Approach Independent of Tocilizumab Treatment
Background/Purpose: Tocilizumab (TCZ), an anti-IL-6 receptor antibody, has been shown to be effective in the treatment of adult-onset Still's disease (AOSD), a systemic inflammatory disease.…Abstract Number: 0925 • ACR Convergence 2024
Macrophages Produce Bone Anabolic Factors in Settings of Inflammation-Induced Bone Formation
Background/Purpose: Understanding the pathogenesis of inflammation-induced pathologic bone formation remains a significant challenge in spondyloarthritis patients. We thought to uncover the pivotal events driving the…Abstract Number: 1706 • ACR Convergence 2024
Multi-omic Study in Patients with SITRAME Syndrome
Background/Purpose: The SITRAME syndrome (Systemic Inflammatory Trunk Recurrent Acute Macular Eruption) is a newly described inflammatory entity affecting adult patients with no family history (Soria…Abstract Number: 2032 • ACR Convergence 2024
A Systematic Review of Treatment Strategies in VEXAS Syndrome
Background/Purpose: VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome, first described in 2020, is an autoinflammatory condition characterized by somatic mutations in the UBA1 gene.…Abstract Number: 2569 • ACR Convergence 2024
A Novel Hyperferritinemia Screen to Aid Differentiation of Hyperinflammatory Disorders
Background/Purpose: High ferritin is an important and sensitive biomarker for the diverse and deadly group of cytokine storm syndromes grouped together under the term hemophagocytic…Abstract Number: 0387 • ACR Convergence 2024
Absolute Agreement and Inter-rater Reliability of a Web-based Standardized Scoring Tool for Magnetic Resonance Images from Children with Chronic Nonbacterial Osteomyelitis (CNO) Amongst Radiologists
Background/Purpose: The web-based ChRonic nonbacterial Osteomyelitis Magnetic Resonance Imaging Scoring (CROMRIS) portal was developed to assess specific features of bone and soft tissue inflammation in…Abstract Number: 0927 • ACR Convergence 2024
Synovial Macrophage Heterogeneity and Dynamics in Steady Stateand Rheumatoid Arthritis Mouse Model Time Course
Background/Purpose: Macrophages are vital contributors to both pro-inflammatory signaling and tissue repair processes involved in the pathogenesis and remission of Rheumatoid Arthritis (RA). Recent research…Abstract Number: 1740 • ACR Convergence 2024
Performance of Large Language Models in Rheumatology Board-Like Questions: Accuracy, Quality, and Safety
Background/Purpose: Large language models (LLMs) are increasingly becoming a common source of information for clinicians. We aimed to evaluate the accuracy, quality, and safety of…Abstract Number: 2039 • ACR Convergence 2024
Canakinumab Treatment in Patients with Familial Mediterranean Fever: A Tertiary Center Experience
Background/Purpose: Canakinumab is one of the most commonly used second-line agents in familial Mediterranean fever (FMF) after treatment failure or discontinuation of colchicine treatment. The…Abstract Number: 2627 • ACR Convergence 2024
Development and Validation of a Novel Score System to Guide Diagnostic Procedures in Children with Concerns of Chronic Nonbacterial Osteomyelitis
Background/Purpose: Chronic nonbacterial osteomyelitis (CNO) is an autoinflammatory bone disease. The diagnosis of CNO is made by recognizing typical clinical and imaging presentation and excluding…
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