Abstract Number: 1394 • ACR Convergence 2024
Nationwide Retrospective Cohort Study on Clinical Outcomes in Rheumatoid Arthritis Patients Treated with JAK Inhibitors
Background/Purpose: Janus kinase inhibitors (JAKi) are a class of disease activity-modifying drugs (DMARDs) that have quickly established a role in managing rheumatoid arthritis. Inhibition of…Abstract Number: 2025 • ACR Convergence 2024
Autonomic Nervous System Dysfunction Common Among Patients with Long COVID:Diagnosis and Treatment Implications
Background/Purpose: Up to 70% of the 236 million people who have been diagnosed with COVID-19 develop some post-COVID symptoms, i.e. post-acute sequalae of SARS-CoV-2 (PASC)…Abstract Number: 2202 • ACR Convergence 2024
Evolving Phenotypic and Genotypic Spectrum of Human ISG15 and USP18 Deficiencies
Background/Purpose: Loss of negative regulator in ISG15 and USP18 results in recently described immunodysregulatory disorders with diversity of clinical characteristics related to enhanced IFN-a/b immunity.…Abstract Number: 0302 • ACR Convergence 2024
TNFAIP3 Loss-of-function and Missense Mutations Demonstrate Clinically Diverse Presentations: A Multi-center Cohort Experience
Background/Purpose: A20 is a critical anti-inflammatory protein encoded by the TNFAIP3 (tumor necrosis factor alpha-induced protein 3) gene. A20 negatively regulates multiple anti-inflammatory pathways including…Abstract Number: 0921 • ACR Convergence 2024
Deep Immunologic Profiling of Trisomy 8-associated Autoinflammatory Disease (TRIAD)
Background/Purpose: Trisomy 8 mosaicism is associated with an inflammatory disease characterized by recurrent fever and oral, genital, and gastrointestinal ulcers, resembling Behçet’s disease. Little is…Abstract Number: 1621 • ACR Convergence 2024
Analysis of Hospitalizations of Adult Patients with Kawasaki’s Disease: A United States Population Based Study
Background/Purpose: Kawasaki disease (KD) is a systemic vasculitis, typically affecting young children, and is the leading cause of acquired heart disease in children in developed…Abstract Number: 2028 • ACR Convergence 2024
Therapeutic Insights in VEXAS Syndrome from a Multicenter Study in Spain: Moving Towards Amore Specific and Effective Treatment
Background/Purpose: Nearly four years since its characterization, management and therapeutic algorithms for VEXAS syndrome remain unclear. This study aims to describe treatment approaches and assess…Abstract Number: 2503 • ACR Convergence 2024
Hypothalamic-Pituitary-Adrenal Axis Suppression by Prednisolone Reversed by the 11β-Hydroxysteroid Dehydrogenase Type 1 Inhibitor Clofutriben
Background/Purpose: Approximately 1% of the population rely on chronic glucocorticoid (GC) prescription to control autoimmune and inflammatory conditions. Risk of GC induced adrenal insufficiency (GC-AI)…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: 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: 0006 • ACR Convergence 2023
Preclinical Characterization of a Novel anti-CD40 Antagonist Antibody-glucocorticoid Conjugate with Superior Preclinical Efficacy and Favorable Safety Profile
Background/Purpose: Glucocorticoids (GCs) are the first-line anti-inflammatory treatment for many autoimmune diseases, but long-term systemic administration of GCs is associated with unwanted side effects, which…Abstract Number: 0275 • ACR Convergence 2023
No Cumulative Effect of Infection Rates in Children Receiving Long-term Canakinumab Treatment in Autoinflammatory Periodic Fever Syndromes − Data from the RELIANCE Registry
Background/Purpose: Autoinflammatory diseases (AID) have been treated safely and effectively with the interleukin-1β inhibitor canakinumab (CAN) in controlled trials and routine clinical practice. The most…Abstract Number: 1136 • ACR Convergence 2023
Challenges in Diagnosing VEXAS Syndrome: Delayed Diagnosis, Misdiagnosis, and Associations with Specific Gene Mutations
Background/Purpose: The newly described VEXAS syndrome is a very heterogenous disease with rheumatologic and hematologic manifestations, caused by somatic mutations affecting UBA1 gene, that still…Abstract Number: 1921 • ACR Convergence 2023
Clinical Spectrum of VEXAS Syndrome in a Rheumatology Department
Background/Purpose: VEXAS (vacuoles, E1 activating enzyme, X-linked, auto-inflammatory, somatic) syndrome is a clinically serious and potentially fatal adult-onset disease caused by somatic mutations in the…Abstract Number: 2519 • ACR Convergence 2023
A Phase II Clinical Study to Investigate the Efficacy and Safety of Hemay005 Tablets in Patients with Active Behçet`sDisease
Background/Purpose: Behçet's disease (BD) is a chronic and recurrent vascular inflammatory disease with major manifestations including oral ulcers, genital ulcers, skin damage and ophthalmitis, and…
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