Abstract Number: 1491 • ACR Convergence 2021
Single-cell Analysis of Paired Skin and Blood Samples from Patients with SLE and Cutaneous Lupus Suggests CD16+ DCs Arise from Non-classical Monocytes That Enter Nonlesional Skin, Undergo Type I IFN Education, and Engage in Extensive Crosstalk with Diverse Immune and Stromal Cell Types
Background/Purpose: Cutaneous lupus erythematosus (CLE) is an incompletely understood autoimmune disease that can occur in isolation or in the context of SLE. CLE is often…Abstract Number: L10 • ACR Convergence 2020
Targeting Plasmacytoid Dendritic Cells Improves Cutaneous Lupus Erythematosus Skin Lesions and Reduces Type I Interferon Levels: Results of a Phase 1 Study of VIB7734
Background/Purpose: Plasmacytoid dendritic cells (pDCs) secrete large amounts of type I interferon (IFN) and other cytokines upon activation. pDCs migrate to sites of active disease…Abstract Number: 0286 • ACR Convergence 2020
An SLE-linked ITGAM Gene Variant Changes Mac-1 Structure, Signaling, and Surface Expression and Enhances IFNg Production and Antigen Presentation by B Cells
Background/Purpose: SLE is a chronic and debilitating disease; in the USA with an estimated incidence of 3-10 per 100,000 people and currently affecting an estimated…Abstract Number: 1150 • ACR Convergence 2020
Traditional Laboratory Parameters and New Biomarkers in Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis
Background/Purpose: Macrophage Activation Syndrome (MAS) and secondary Hemophagocytic Lymphohistiocytosis (sHLH) are hyperinflammatory conditions caused by a cytokine storm, in which IFNγ plays a pivotal role.…Abstract Number: 0287 • ACR Convergence 2020
RNA Externalized by Neutrophil Extracellular Traps Promotes Inflammatory Pathways in Endothelial Cells
Background/Purpose: Neutrophil extracellular traps (NETs) are extracellular lattices composed of nucleic material bound to neutrophil granule proteins. NETs may play pathogenic roles in development and…Abstract Number: 1158 • ACR Convergence 2020
Clinical Features and Outcomes in STING-Associated Vasculopathy with Onset in Infancy (SAVI)
Background/Purpose: STING-Associated Vasculopathy with Onset in Infancy (SAVI) is an autoinflammatory interferonopathy caused by gain-of-function mutations in STING1, characterized by peripheral vasculopathy and interstitial lung…Abstract Number: 0289 • ACR Convergence 2020
Endogenous Interferon-β and Low IL-4R on Transitional B Cells Promotes Lupus Nephritis
Background/Purpose: We previously showed that B-cell endogenous interferon-beta (IFNβ) at the transitional (Tr) stage correlates with development of anti-Smith (anti-Sm) and renal disease as well…Abstract Number: 1159 • ACR Convergence 2020
Novel STING1 Mutations Including in the Transmembrane Linker Region Cause STING-associated Vasculopathy with Onset in Infancy (SAVI)
Background/Purpose: STING-associated vasculopathy with onset in infancy (SAVI) is an autoinflammatory disease caused by gain-of-function (GOF) mutations in STING1/TMEM173 that encodes stimulator of interferon genes,…Abstract Number: 0292 • ACR Convergence 2020
Exhausted pSTAT5-IFNα Signaling Pathways in SLE Patients Are Correlated with Age-associated B Cells and Disease Activity
Background/Purpose: Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by periods of elevated and suppressed clinical symptoms. Specific cell subsets, such as CD11c+ age-associated…Abstract Number: 1399 • ACR Convergence 2020
Differential Impacts of TNFa Inhibitors on the Expression of Th Cytokines
Background/Purpose: Inhibition of TNFα has emerged as an effective therapeutic approach for many autoimmune/inflammatory diseases.While the efficacy and safety profile of the five FDA-approved TNFis…Abstract Number: 0297 • ACR Convergence 2020
Towards a Glucocorticoid Exposure Signature in SLE: Effects of Type I Interferon
Background/Purpose: Glucocorticoids (GC), utilised in SLE for their broad immunosuppressive actions, predominantly mediate these effects by interaction with the cytoplasmic GC receptor (GR) to modulate…Abstract Number: 1405 • ACR Convergence 2020
Evaluating the Cellular Composition of Anti-synthetase Syndrome and Dermatomyositis Skin Lesions Using Image Mass Cytometry
Background/Purpose: Antisynthetase syndrome (AS) is a systemic autoimmune disorder characterized by the presence of anti-aminoacyl-tRNA synthetase antibodies, myositis, interstitial lung disease (ILD), mechanics hands, and…Abstract Number: 0304 • ACR Convergence 2020
Type I Interferon Inhibits Glucocorticoid-Induced Leucine Zipper (GILZ) Expression and Upregulation by Glucocorticoids
Background/Purpose: Glucocorticoids (GC) are broadly used in the treatment of inflammatory diseases, including systemic lupus erythematosus (SLE). Despite their widespread use, most SLE patients do…Abstract Number: 1443 • ACR Convergence 2020
High-dimensional Analyses of Checkpoint-inhibitor Related Arthritis Synovial Fluid Cells Reveal a Unique, Proliferating CD38hi Cytotoxic CD8 T Cell Population Induced by Type I IFN
Background/Purpose: Checkpoint inhibitors (CI) used to treat cancer frequently trigger immune-related adverse events, including inflammatory arthritis. CI-related arthritis (CIrA) occurs in ~5% of treated patients,…Abstract Number: 0462 • ACR Convergence 2020
Lupus-like Autoimmunity and Increased Interferon Response in Patients with STAT3-deficient Hyper-IgE Syndrome
Background/Purpose: Autosomal dominant hyper-IgE syndrome (AD-HIES), also known as Job’s syndrome, is a rare primary immunodeficiency caused by dominant-negative loss-of-function (LOF) mutations in signal transducer…