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…Abstract Number: 0398 • ACR Convergence 2024
Validation of Patient-Reported Outcomes Measurement Information System® (PROMIS®) Pediatric Measures for Children with Chronic Nonbacterial Osteomyelitis Using the CHOIR Data
Background/Purpose: Chronic nonbacterial osteomyelitis (CNO) is an autoinflammatory bone disease. It is critical to capture the child’s health-related quality of life impact using validated patient-reported…Abstract Number: 0934 • ACR Convergence 2024
Screening NLRP3 Drug Candidates in Clinical Development:Lessons from Existing and Emerging Technologies
Background/Purpose: NLRP3 is emerging as an attractive upstream target of the pathway to down-modulate rather than to completely neutralize IL-1ß levels in both acute and…Abstract Number: 1766 • ACR Convergence 2024
STAT2-Associated Type I Interferonopathy: A Masquerade of Infectious Susceptibility
Background/Purpose: Type I IFN (IFN-I) signaling is a potent inflammatory pathway fundamental to antiviral immunity. In humans, loss of IFN-I activity underlies severe viral disease,…Abstract Number: 2040 • ACR Convergence 2024
Effective Management of Idiopathic Recurrent Pericarditis with Anakinra in Clinical Practice
Background/Purpose: Idiopathic recurrent pericarditis (IRP) is a rare autoinflammatory syndrome marked by recurrent pericardial inflammation after an initial episode of acute pericarditis. Standard treatment involves…Abstract Number: 2651 • ACR Convergence 2024
Vasculitis Associated with VEXAS Syndrome
Background/Purpose: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently defined clinical entity that causes hematologic and autoinflammatory symptoms. Since its initial description…Abstract Number: L03 • ACR Convergence 2023
Efficacy and Safety of Targeted Therapies in VEXAS Syndrome: Retrospective Study from the French VEXAS Group
Background/Purpose: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a monogenic disease of adults due to acquired somatic mutations of the UBA1 gene. Patients…Abstract Number: L01 • ACR Convergence 2023
Analysis of 245,388 Diverse Participants in the NIH All of Us Cohort Identifies VEXAS Resiliency in UBA1 M41L Somatic Mutation Carriers
Background/Purpose: VEXAS syndrome is a recently-discovered systemic auto-inflammatory disease caused by somatic mutation at position 41 in the X-linked gene UBA1.1 First, 25 older men…Abstract Number: 0039.5 • ACR Convergence 2023
Cytokine-induced Transcriptome Modification Elucidates the Similarity and Dissimilarity Across 10 Immune-related Diseases
Background/Purpose: Abnormal cytokine regulation is the fundamental pathology of immune-mediated diseases (IMDs). Clinical transcriptome data has a potential to monitor real-time cytokine pathway activities of…Abstract Number: 0539 • ACR Convergence 2023
Impact of Extreme Baseline BASDAI and/or Maastricht AS Enthesitis Score on Treatment Response to Upadacitinib in Patients with Axial Spondyloarthritis
Background/Purpose: Axial SpA (axSpA) and FM manifest with overlapping clinical features such as pain, fatigue, and stiffness, yet their treatment is distinctly different. FM is…Abstract Number: 1240 • ACR Convergence 2023
Obvious Only in Retrospect: A Cohort of STING Associated Vasculopathy in Infancy (SAVI) Without Typical Rash
Background/Purpose: STING-associated vasculopathy with onset in infancy (SAVI) is characterized by systemic inflammation, skin vasculopathy and interstitial lung disease. However, since the initial description knowledge…Abstract Number: 1936 • ACR Convergence 2023
Higher Rates of Disease Control During the Coronavirus Pandemic in Pediatric Patients with Autoinflammatory Periodic Diseases on Canakinumab Treatment – Interim Data from the RELIANCE Registry
Background/Purpose: Pediatric patients with autoinflammatory diseases (AID) on Canakinumab (CAN) therapy have been affected by the coronavirus pandemic including SARS-CoV-2 infection, SARS-CoV-2 vaccination, and AID…Abstract Number: 0087 • ACR Convergence 2023
Immune Checkpoint Inhibitor Therapy Expands an Activated, anti-PD-1 Drug-bound CD8 T Cell Population That Is Clonally Linked in Blood and Synovial Fluid of ICI-arthritis Patients
Background/Purpose: Immune checkpoint inhibitor (ICI) therapies used to treat cancer can induce immune related Adverse events (irAEs) such as ICI-induced arthritis (ICI-arthritis). We have previously…
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