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: 0400 • ACR Convergence 2024
Osteoclastogenesis from Peripheral Blood Mononuclear Cells in Children with Chronic Nonbacterial Osteomyelitis Are Similar to Those from Healthy Children
Background/Purpose: Chronic nonbacterial osteomyelitis (CNO) is an autoinflammatory bone disease in which osteoclastogenesis may play a critical role. Cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) was shown…Abstract Number: 0999 • ACR Convergence 2024
Clinical and Biological Features of VEXAS Syndrome in Women: Study of 9 French Cases Compared with 263 Men
Background/Purpose: VEXAS syndrome is an autoinflammatory disease associated with somatic mutations in the UBA1 gene, which is located on the X chromosome, which explains the…Abstract Number: 1773 • ACR Convergence 2024
Divergent Genetic Architecture in Boys and Girls with NEMO-deleted Exon 5 Autoinflammatory Syndrome (NEMO-NDAS) Implies Role for Wildtype Effector Cells
Background/Purpose: Splice-site variants in X-linked IKBKG cause NEMO-deleted exon5 autoinflammatory syndrome (NEMO-NDAS); a pseudogene (IKBKGP1) complicates genetic diagnosis. NEMO-NDAS is four times more common in…Abstract Number: 2044 • ACR Convergence 2024
Clinical Practice Patterns, Rilonacept Appropriate Use for Recurrent Pericarditis
Background/Purpose: Pericarditis is inflammation of the pericardial sac which can be of infectious or non-infectious etiology, commonly associated with rheumatic diseases. Pericarditis is defined as…Abstract Number: 2659 • ACR Convergence 2024
A Comprehensive Approach Utilizing a Combination of Genetics, Cytological Analysis, and Imaging Techniques to Speed up the Diagnostic Process in VEXAS Patients
Background/Purpose: VEXAS is a newly described “haemato-autoinflammatory” condition resulting from somatic mutations in the UBA-1 gene. By integrating genetics, cytology and imaging, this work seeks…Abstract Number: 0006 • ACR Convergence 2024
NX-5948, a Clinical-Stage BTK Degrader, Achieves Deep Suppression of BCR, TLR, and FcR Signaling in Immune Cells and Demonstrates Efficacy in Preclinical Models of Arthritis and Other Inflammatory Diseases
Background/Purpose: Bruton’s tyrosine kinase (BTK) mediates signaling downstream of the B cell receptor (BCR), toll-like receptors (TLRs), and Fc receptors (FcRs). This makes BTK an…Abstract Number: 0408 • ACR Convergence 2024
Analysis of Protein Biomarkers for the Prediction of IL-1 Inhibitor Treatment Response in the CARRA First-line Options for Systemic Juvenile Idiopathic Arthritis Treatment (FROST) Study
Background/Purpose: IL-1 inhibitors are both widely used and highly effective as first-line therapy for children with systemic juvenile idiopathic arthritis (sJIA); however, the mechanisms underlying…Abstract Number: 1062 • ACR Convergence 2024
Assessing Musculoskeletal Symptom Burden in a Cohort of Patients with Hidradenitis Suppurativa Using the IDEOM MSK-Q
Background/Purpose: Hidradenitis suppurativa (HS) has been associated with inflammatory joint diseases such as psoriatic arthritis and ankylosing spondylitis. Despite this correlation, dermatologists do not routinely…Abstract Number: 1778 • ACR Convergence 2024
A Novel Variant in IRAK2 Results in Immune Dysregulation in Systemic Juvenile Idiopathic Arthritis (sJIA)
Background/Purpose: Lung disease (LD) is poorly understood complication of Still’s disease with high fatality. HLA-DRB1*15 is a strong risk factor for the development of this…Abstract Number: 2046 • ACR Convergence 2024
Duration of Disease and Long-term Outcomes in Patients with Difficult-to-treat Recurrent Pericarditis: A Chronic Condition Treated with NSAIDs, Colchicine, Corticosteroids, and Anti-IL-1 Agents
Background/Purpose: Recurrent pericarditis (RP) challenges pts and clinicians in terms of long-term management. Objective of this study was to evaluate the remission rate (drug-free for…
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