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: 2152 • ACR Convergence 2025
Blau Syndrome: features beyond the classic triad and proposal for clinical criteria
Background/Purpose: This study describes the characteristic clinical and radiological findings and outcomes of patients with Blau syndrome from a single tertiary care hospital.Methods: A retrospective…Abstract Number: 1877 • ACR Convergence 2025
Demographics and trends of Inflammatory Arthritis-Related Mortality in the United States, 1999–2019
Background/Purpose: The study aims to evaluate the racial, sex-related and urbanization-related trends of deaths in patients with inflammatory polyarthropathies in the United States between 1999…Abstract Number: 1303 • ACR Convergence 2025
Impact of Fruits and Vegetables Intake on Immune-mediated Inflammatory Diseases:a Two-sample Mendelian Randomization Study
Background/Purpose: A balanced diet can prevent diseases and promote physical and mental health. Accumulating evidence shows that fruits and vegetables (F&Vs) intake is associated with…Abstract Number: 0929 • ACR Convergence 2025
SP2H, a Targeted Degrader of STimulator of INterferon Genes (STING), selectively inhibits STING-driven inflammation in vitro and in vivo and improves survival in Trex1-/- mice
Background/Purpose: STING plays a critical role at the intersection of innate and adaptive immunity and has been linked to the pathogenesis of SSc and SLE.…Abstract Number: 0050 • ACR Convergence 2025
TL1A expression is upregulated in rheumatic diseases and anti-TL1A antibody reduces disease symptoms and pathological changes in rat collagen-induced arthritis
Background/Purpose: The development of disease-modifying anti-rheumatic drugs (DMARDs) has reduced disease activity in patients living with rheumatic diseases. Despite this, there is continued unmet need…Abstract Number: 2151 • ACR Convergence 2025
Real-World Experience with IL-1 Blockade in Children with Undifferentiated Systemic Autoinflammatory Diseases (uSAIDs)
Background/Purpose: Systemic Autoinflammatory Diseases (SAIDs) are a group of rare disorders caused by dysregulation of the innate immune system. Over 50 genes have been identified…Abstract Number: 1827 • ACR Convergence 2025
Transcriptomics and Machine Learning Unraveling the Molecular Drivers of PFAPA Flares
Background/Purpose: Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) is the most common periodic fever presenting with frequent episodes of pain and impaired function. While…Abstract Number: 1302 • ACR Convergence 2025
Dose Adjustment was Necessary in Patients with Cryopyrin-Associated Periodic Syndrome Switching from Anakinra to Canakinumab
Background/Purpose: Cryopyrin-associated periodic syndrome (CAPS) is a spectrum of rare autoinflammatory disorders caused by mutations in the NLRP3 gene, leading to excessive interleukin-1β (IL-1 β)…Abstract Number: 0920 • ACR Convergence 2025
Functional Characterization of NEMO-NDAS Causing Variants in Patients’ PBMCs and in Wildtype and Mutant U937 Cells
Background/Purpose: NEMO-deleted 5 autoinflammatory syndrome (NEMO-NDAS) is an inflammatory disease caused by mosaic splice-site variants that lead to exon 5 skipping in IKBKG, encoding NEMO.…
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