Abstract Number: 0237 • ACR Convergence 2025
Monogenic autoinflammatory diseases and undefined autoinflammatory syndrome in Western Mediterranean adults: clinical, genetic, and therapeutic insights
Background/Purpose: Monogenic autoinflammatory diseases are rare innate immunity disorders characterized by recurrent episodes of fever and systemic inflammation. They are most often described in childhood,…Abstract Number: 2154 • ACR Convergence 2025
Whole-Body MRI Findings in a Cohort of Children with Chronic Nonbacterial Osteomyelitis
Background/Purpose: Chronic nonbacterial osteomyelitis (CNO) is an autoinflammatory bone disorder characterized by multifocal, sterile bone inflammation in childhood. Whole-body magnetic resonance imaging (WB-MRI) allows for…Abstract Number: 1889 • ACR Convergence 2025
Navigating Diagnostic Challenges: Insights into the Patient Journey and Delays in Diagnosing Dermatomyositis from a Real-world Survey in the United States and Europe
Background/Purpose: Idiopathic inflammatory myopathies (IIMs) are a heterogenous group of rare autoimmune inflammatory rheumatic diseases, including dermatomyositis (DM).Methods: Data were drawn from the Adelphi Real…Abstract Number: 1305 • ACR Convergence 2025
Clinical outcomes of low-to-moderate–dose glucocorticoids in multisystem inflammatory syndrome in children (MIS-C) – a multicenter study
Background/Purpose: Multisystem Inflammatory Syndrome in Children (MIS-C) is an autoinflammatory disease following SARS-CoV-2 exposure. The optimal treatment strategy remains under investigation, though intravenous immunoglobulin (IVIG)…Abstract Number: 1056 • ACR Convergence 2025
High diagnostic rate of genetic testing in adult patients with autoinflammation: A Single Center Experience
Background/Purpose: Inborn errors of immunity (IEI), including autoinflammatory diseases and primary immune regulation disease (PIRD), are unfamiliar to many adult rheumatologists, leading to potential ascertainment…Abstract Number: 0082 • ACR Convergence 2025
IBI3011, a Humanized anti-IL1RAP Monoclonal Antibody, Inhibits IL1, IL33, IL36-driven Inflammation Pathway, and Attenuates Inflammation in Preclinical Inflammatory Disease Model
Background/Purpose: Interleukin 1 receptor accessory protein (IL1RAP) serves as a co-receptor that forms receptor complexes with IL1R1, ST2, and IL36R and mediates signaling pathways triggered…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…
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