Abstract Number: 1001 • ACR Convergence 2021
Exosomes Mediate a Cooperative Mechanism of Macrophage/Fibroblast Activation in Systemic Sclerosis
Background/Purpose: Prior work demonstrates that macrophages (MØs) from patients with Systemic Sclerosis (SSc) produce fibrotic and pro-inflammatory mediators and that cocultured MØs and SSc fibroblasts…Abstract Number: 1455 • ACR Convergence 2020
Functional Characterization of PLCG2 Mutations Found in Subjects with Autoinflammation and PLCG2-Associated Antibody Deficiency and Immune Dysregulation (APLAID) Reveals Both Hypermorphic and Hypomorphic Mutants
Background/Purpose: PLCG2-associated antibody deficiency and immune dysregulation (PLAID) and autoinflammatory PLAID (APLAID) are autosomal dominant diseases caused by mutations of PLCG2. APLAID is clinically characterized by episodic…Abstract Number: 0062 • ACR Convergence 2020
The Role of PGLYRP1 in the Pathogenesis of Lyme Disease
Background/Purpose: Lyme Disease is caused by the spirochete Borrelia Burgdorferi (Bb). The infection often begins in the skin, following a tick bite, and spreads to…Abstract Number: 1517 • ACR Convergence 2020
Metabolic Regulation of Type 3 Innate Lymphoid Cells by Intestinal Bacteria-Derived Indoles in Ankylosing Spondylitis
Background/Purpose: Intestinal microbial dysbiosis, intestinal inflammation, and Th17 immunity are all linked to the pathophysiology of ankylosing spondylitis (AS); however, the mechanisms linking them remain…Abstract Number: 0063 • ACR Convergence 2020
Novel Repurposed Drugs Against Joint Inflammation Reveal Potential Use for Gout Treatment: An In Silico, In Vitro and Clinical Study
Background/Purpose: Joint inflammation is a common feature across multiple rheumatic diseases. To deal with the induction of innate immune factors, targeting therapeutic targets such as…Abstract Number: 1531 • ACR Convergence 2020
Characterization of Cytokine/chemokine Profile in Patient-derived M1/ M2 Macrophages to Identify Biomarkers for Genetically-defined Systemic Autoinflammatory Diseases
Background/Purpose: Genetic mutations in key regulatory molecules of the innate immune system cause autoinflammatory diseases through propagation of hyperinflammatory responses. Monocytes/ macrophages regulate inflammatory processes…Abstract Number: 0064 • ACR Convergence 2020
Neutrophil Extracellular Traps Are Sufficient to Activate the Alternative Pathway of Complement
Background/Purpose: Systemic lupus erythematosus (SLE) replies on complement activation to drive many of the pathophysiologic features of disease. We and others have noted that SLE…Abstract Number: 1636 • ACR Convergence 2020
8 Years Follow-Up of a Novel Autoinflammatory Disease: CD59 Malfunction Causes Hemolytic Anemia, Recurrent Guillain-Barre Syndrome, and Strokes in Pediatric Populations and Respond Well to Eculizumab and Pozelimab
Background/Purpose: In 2013 we have described the first patients with a novel autoinflammatory disease manifested in 4 children with recurrent Guillain-Barre syndrome and hemolytic anemia…Abstract Number: 0074 • ACR Convergence 2020
The Intracellular DNA Sensor STING Protects Against Bone Loss Through Regulation of Type I Interferons
Background/Purpose: The intracellular DNA sensor Stimulator of Interferon Genes (STING) is essential for detection of viral and bacterial pathogen DNA. As with other pathways in…Abstract Number: 1810 • ACR Convergence 2020
Complement Activation in Systemic Lupus Erythematosus Patients with Low Disease Activity Is Not Inhibited by Hydroxychloroquine
Background/Purpose: Mortality in patients with systemic lupus erythematosus (SLE) is significantly higher than in the general population. Treatment of SLE patients has improved, however, a…Abstract Number: 0176 • ACR Convergence 2020
Characterization and Molecular Mechanism Underlying NEMO Deleted Exon 5 Autoinflammatory Syndrome (NDAS)
Background/Purpose: The NF-kB essential modulator (NEMO) is a scaffolding protein with a broad immune cell and tissue expression profile. Hypomorphic mutations in IKBKG encoding NEMO…Abstract Number: 1861 • ACR Convergence 2020
T Cells with IL-17A+ Signature in Psoriatic Arthritis Are of Different Subpopulations and Are Polyfuntional
Background/Purpose: A variety of T cells such as Th1, Th2, Th9, Th17, NKT, MAIT, etc have been attributed to multiple autoimmune diseases. In psoriatic arthritis…Abstract Number: 0282 • ACR Convergence 2020
Expression of the cGAMP Transporter SLC19A1 Is Altered in Systemic Lupus Erythematosus
Background/Purpose: Inappropriate sensing of nucleic acids leading to enhanced type I interferon (IFN) induction is a hallmark of SLE, contributing to breakdown of immune tolerance…Abstract Number: 1953 • ACR Convergence 2020
Somatic Mutations in a Single Residue of UBA1 Cause VEXAS, a Severe Adult-Onset Rheumatic Disease Presenting as Relapsing Polychondritis, Polyarteritis Nodosa, or Giant Cell Arteritis
Background/Purpose: Identifying the causes of adult-onset rheumatic diseases remains a challenge, and limits diagnosis, prognosis, and targeted treatment. We hypothesized that mutations in genes regulating…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…
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