Abstract Number: 034 • 2023 Pediatric Rheumatology Symposium
STAT3 Gain-of-function Syndrome Mutations Are Susceptible to JAK Inhibition Despite a Spectrum of Potency
Background/Purpose: STAT3 gain-of-function (GOF) syndrome is a rare inborn error of immunity that leads to early-onset lymphoproliferation, autoimmune cytopenias, multi-solid organ autoimmunity, hypogammaglobulinemia, and short…Abstract Number: 037 • 2023 Pediatric Rheumatology Symposium
Transcriptional Analysis of CD14+ Monocytes During Macrophage Activation Syndrome Highlights Role for Interferons and RNA Sensing in Monocytes
Background/Purpose: Macrophage activation syndrome (MAS), a form of secondary hemophagocytic lymphohistiocytosis (HLH), is a potentially fatal complication of rheumatic diseases. MAS is a dysfunctional hyperinflammatory…Abstract Number: 0266 • ACR Convergence 2022
Expression of Cytokine and Chemokine Genes in Patients with Clinically Suspect Arthralgia; A Longitudinal Study During Progression to Inflammatory Arthritis or Non-Progression
Background/Purpose: Autoantibody responses rise years before onset of inflammatory arthritis (IA) and are stable during transitioning from clinically suspect arthralgia (CSA) to IA (1). Cytokine…Abstract Number: 0652 • ACR Convergence 2022
Serum from Patients with SLE and Anti-MDA5 Antibody-Positive Dermatomyositis Induce Shared Monocyte Cytokine Signature Through Type 1 Interferon Pathway
Background/Purpose: Although type 1 interferon (IFN) plays a pivotal role in the pathogenesis of systemic lupus erythematosus (SLE) and polymyositis/dermatomyositis (PM/DM) [1] as typically evidenced…Abstract Number: 1686 • ACR Convergence 2022
Post-translational Modification of Matrix-gla Protein with Malondialdehyde-Acetaldehyde Alters Cellular Responses by Human Fibroblasts
Background/Purpose: Post-translational modifications with malondialdehyde-acetaldehyde (MAA) alter protein structure and function, inducing pro-inflammatory and pro-fibrotic responses characteristic of rheumatoid arthritis (RA). Matrix-gla protein (MGP) chelates…Abstract Number: 0499 • ACR Convergence 2022
Drivers of Heterogeneity in Synovial Fibroblasts in Rheumatoid Arthritis
Background/Purpose: Rheumatoid arthritis (RA) is characterized by hyperplasia of both the synovial lining, which forms the synovial fluid interface, as well as the synovial sublining,…Abstract Number: 0666 • ACR Convergence 2022
HRES-1/Rab4 Controls the Overexpression of CD38 and Depletion of IL-2 in CD4+ T Cells; Potential Involvement in Proinflammatory Lineage Development in SLE
Background/Purpose: HRES-1/Rab4 (Rab4A) is a small GTPase that is overexpressed in SLE patient T cells1,2, mediates the enhanced recycling of CD3 and CD4 cell surface…Abstract Number: 1691 • ACR Convergence 2022
Pentagalloyl Glucose Inhibits MSU-Induced Inflammation via Transforming Growth Factor-β-Activated Kinase 1 (TAK1)
Background/Purpose: Gout disease flares occur when primed macrophages interact with and phagocytose monosodium urate crystals (MSU), activating IL-1β via the NLRP3 inflammasome. Abrogation of inflammasome…Abstract Number: 0559 • ACR Convergence 2022
JAK Inhibitor Withdrawal Causes a Transient Proinflammatory Signaling Cascade in Minor Salivary Gland Mesenchymal Stromal Cells
Background/Purpose: Sjögren's Disease (SjD) has high glandular IFNg levels, associated with disease activity and lymphoma risk. We previously showed IFNg-stimulated minor salivary gland (SG)-mesenchymal stromal…Abstract Number: 0689 • ACR Convergence 2022
Type I Interferon Stimulated Genes Identify Different Phenotypes of Antiphospholipid Syndrome Patients
Background/Purpose: At present, a limited number of evidencesis available on the specific role of Type I Interferons (IFN) activation in antiphospholipid antibodies (aPL) positive patients,…Abstract Number: 1700 • ACR Convergence 2022
IL-21/STAT3 Pathway Mediated Th2-like Vδ2 T Cells Promote Plasmablasts Differentiation in IgG4-Related Disease
Background/Purpose: To explore the phenotype and role of gamma delta (γδ) T cells in the pathogenesis of IgG4-related disease (IgG4-RD), focusing on the effect on…Abstract Number: 0569 • ACR Convergence 2022
Cytokine Storm Susceptibility Factors IL-18 and Cytotoxic Impairment Converge on Pathologic CD8 T-cell Hyperactivation
Background/Purpose: Mounting evidence suggests partial defects in cytotoxicity-related genes may promote hyperinflammation in Macrophage Activation Syndrome (MAS) patients, who uniformly have highly-elevated IL-18. In mice,…Abstract Number: 0806 • ACR Convergence 2022
Long-term Effectiveness of Sublingual Polybacterial Vaccines in Patients with Systemic Autoimmune Disease and Active Pharmacological Immunosuppression
Background/Purpose: Infections in patients with systemic autoimmune disease (SAD) are associated with immune dysfunction, disease activity and immunosuppression. Synthetic and biologic disease-modifying drugs (DMARDs) have…Abstract Number: 1729 • ACR Convergence 2022
SLAMF4+ CCR5+ Effector Memory CD4+ T Cells Are Polyfunctional, Resistant to Exhaustion, and Expanded in Rheumatoid Arthritis
Background/Purpose: CD4+ Foxp3- conventional T cells (Tconv) play a key role in the inflammatory process involved in rheumatoid arthritis (RA). Usually, chronic stimulation of T…Abstract Number: 0581 • ACR Convergence 2022
Mutants Improving Stability Enhance the Potency of Treg-Selective IL-2 Mutein for the Treatment of Autoimmune Disorders
Background/Purpose: Low-dose IL-2 with enhanced sensitivity to Treg cells has been proved to be safe and efficacious in multiple autoimmune disorders (AIDs) including SLE, RA,…
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