Abstract Number: L01 • ACR Convergence 2024
Targeted Exosite Inhibition of STING Activation of TBK1 Selectively Blocks Type I Interferon and NFκB Responses for Treatment of Autoimmune Diseases
Background/Purpose: The cGAS-TBK1-STING pathway senses nucleic acids for innate immunity. Aberrant activation of the pathway is linked to autoimmune diseases including Systemic and Cutaneous Lupus…Abstract Number: 0954 • ACR Convergence 2024
Increased Proliferating Natural Killer Cells Exhibit an Aberrant Pro-Inflammatory Gene Signature in Systemic Sclerosis Patients
Background/Purpose: Recent transcriptomic data suggest a prominent involvement of innate immune cells in the pathogenesis of SSc. In this regard, contrasting data on NK cells…Abstract Number: 1768 • ACR Convergence 2024
A Potential Role of Longstanding IL-18 Stimulation in the Susceptibility for Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis
Background/Purpose: Macrophage Activation Syndrome (MAS) is a pathologic condition of immune hyperactivation, which occurs in 10-30% of cases of Still’s Disease (SD), the spectrum of…Abstract Number: 2397 • ACR Convergence 2024
Increase of IL10 and IFNa2 Are Associated to Clinical Activity in Systemic Lupus Erythematous Patients
Background/Purpose: Systemic lupus erythematous (SLE) is an autoimmune disease characterized by deregulation of cytokine production. Interferon (IFN) is a proinflamatory cytokine considered as a key…Abstract Number: 0970 • ACR Convergence 2024
Deubiquitylase (DUB) BRCC36 Isopeptidase Complex (BRISC) Inhibitors Prevent IFNAR1 Deubiquitination to Restore Natural Type I IFN Response in Autoimmunity
Background/Purpose: Increased Type I IFN activation plays a key role in Scleroderma (SSc), Systemic Lupus Erythematosus and Inflammatory Myositis. Deubiquitylase (DUB) BRCC36 isopeptidase complex (BRISC)…Abstract Number: 1770 • ACR Convergence 2024
Assessing S100A4 and Myofibroblast Phenotypes in the Pathogenesis of Cardiac and Cutaneous Neonatal Lupus
Background/Purpose: Neonatal lupus (NL), driven by fetal exposure to maternal anti-SSA/Ro autoantibodies, typically results in permanent cardiac manifestations in utero and/or a transient rash postnatally.…Abstract Number: 2423 • ACR Convergence 2024
Associations Between Cytometric Interferon Signature and Clinical Response in a Cohort of Anifrolumab-treated Patients with Lupus
Background/Purpose: Anifrolumab, a monoclonal antibody targeting type I interferon (IFN-I) receptor subunit 1, was recently approved for the treatment of systemic lupus erythematosus (SLE) and…Abstract Number: 0001 • ACR Convergence 2024
Type I Interferon and Anifrolumab Effects on Bone Marrow and Blood Plasma Cells in Systemic Lupus Erythematosus
Background/Purpose: Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of pathogenic anti-nuclear antibodies (ANA) by plasma cells (PC). Importantly, long-lived plasma…Abstract Number: 1150 • ACR Convergence 2024
Increased ISG15 Expression in Peripheral Blood from Patients with Positive Dermatomyositis Related Specific Autoantibodies
Background/Purpose: Idiopathic inflammatory myopathies (IIM) are characterised by muscle inflammation (myositis) and weakness, impacting various organ systems. Clinical subtypes include dermatomyositis (DM), antisynthetase syndrome (ASyS),…Abstract Number: 1772 • ACR Convergence 2024
Modeling and Predicting HLH Through Measurement of Immune Synapse Duration, Cytokine Production, and Target Cell Death
Background/Purpose: Hyperinflammation is a life-threatening systemic inflammatory state most commonly associated with Hemophagocytic Lymphohistiocytosis (HLH) and Macrophage Activation Syndrome (MAS), but observed in nearly all inflammatory…Abstract Number: 2456 • ACR Convergence 2024
High Levels of Circulating IFNα Are Associated with Increased Mortality in Patients with Diffuse Cutaneous Systemic Sclerosis
Background/Purpose: Systemic sclerosis (SSc) is a connective tissue disease characterized by skin and organ fibrosis, autoimmunity, and vasculopathy. Although its pathophysiology remains unclear, recent data…Abstract Number: 0020 • ACR Convergence 2024
CD14 Deficiency Protects Against TLR4/LPS-mediated Inhibition of Osteoclastogenesis
Background/Purpose: Osteoarthritis is associated with bone changes such as subchondral sclerosis, bone marrow lesions, and osteophyte formation (Donel S, 2019). Prior work has demonstrated that…Abstract Number: 1266 • ACR Convergence 2024
Anti-Mitochondrial Antibodies Associate with Disease Activity and IFNα Expression in Childhood-onset Systemic Lupus Erythematosus
Background/Purpose: Extracellular mitochondria may represent a source of antigenic burden in autoimmune disorders, such as in systemic lupus erythematosus (SLE), in which those organelles, released…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: 2518 • ACR Convergence 2024
Behçet’s Disease Immune Landscape Exhibits a Universal NF-kB-mediated Hyperactivation Pattern with Cell-specific TNFAIP3 Responses and a Superimposed IFN-regulated Endotype
Background/Purpose: The immune landscape of Behçet’s disease (BD) remains ill-defined, lacking a unifying picture, precise disease phenotype-endotype correlations, and an understanding of immunity at target…
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