Abstract Number: 1931 • ACR Convergence 2022
NOD2 Mutations Are Associated with Upregulated Type 1 Interferon Gene Expression and Development of Granulomatous Hepatitis in Children with Autoimmune Hepatitis
Background/Purpose: Mutations in the gene NOD2 encoding the nucleotide-binding oligomerization domain-containing protein 2 (NOD2) which controls innate responses to LPS have been linked to Blau…Abstract Number: 1177 • ACR Convergence 2022
Identification of a New Type of Pro-phagocytic Macrophages in Patients with Systemic Sclerosis
Background/Purpose: Phagocytosis is a crucial cellular process, which under certain immuno-pathological conditions can be activated in macrophages in an uncontrolled manner. Biomarker studies have implicated…Abstract Number: 1936 • ACR Convergence 2022
A De Novo Dominant-negative PSMB8 mutation is Linked to a More Severe CANDLE-like Phenotype
Background/Purpose: Homozygote (HM)/compound heterozygote (CH) and digenic (DG) mutations in proteasome subunits cause the autoinflammatory syndrome CANDLE, or proteasome-associated autoinflammatory syndrome (PRAAS). Inflammation is mediated…Abstract Number: 1327 • ACR Convergence 2022
Immune Cell Subsets and Risk of Incident Hip Fracture in Older Adults: The Cardiovascular Health Study
Background/Purpose: Cell-mediated immunity may play a role in osteoporosis development. Fracture is the major consequence of osteoporosis. Thus, cell-mediated immunity may impact fracture risk. We…Abstract Number: 2051 • ACR Convergence 2022
Targeting Janus Kinase Pathway in Sjogren’s Disease Corrects IFN-Driven Inflammation and Epithelial Dysfunction
Background/Purpose: Many of the inflammatory cytokines implicated in Sjogren's Disease (SjD) pathogenesis, in particular Type I and II interferons (IFNs), signal through Janus kinases (JAK)…Abstract Number: 1565 • ACR Convergence 2022
Dissection of Ocular and Peripheral Blood Immune Phenotypes Suggests Preferential Transmigration of CD16+ Monocytes to Effector Sites in Behçet’s Disease
Background/Purpose: Cellular immunity of Behçet's Disease (BD) remains poorly understood. Previous work has provided clues pointing to most innate and adaptive immune cell types in…Abstract Number: 1663 • ACR Convergence 2022
Age-Dependent Expansion and Activation of Disease-Associated Microglia in Neuropsychiatric Symptoms of Systemic Lupus Erythematosus
Background/Purpose: Systemic lupus erythematosus (SLE) is a complex autoimmune disease that affects multipe end organs, including the brain. Despite a prevalence of over 50% in…Abstract Number: 0025 • ACR Convergence 2022
Wnt Pathway/Innate Immunity Equilibrium in OA Articular Cells
Background/Purpose: The progressive degradation of osteoarthritis (OA) cartilage has been associated with articular innate immune responses (IIR) mediated by the Toll-like receptor 4 (TLR4). Additionally,…Abstract Number: 1690 • ACR Convergence 2022
Butyric Acid Suppresses Migration of Human Monocyte Derived Dendritc Cell by Inhibiting Actin Polymerization via mDia1 Inhibition
Background/Purpose: Butyric acid is known to improve chronic inflammation such as inflammatory bowel disease and arthritis. Dendritic cells activate in inflammatory condition, migrate to regional…Abstract Number: 0049 • ACR Convergence 2022
IL-18 Receptor-α Signaling Pathway Contributes to Autoantibody-induced Arthritis via Neutrophil Recruitment
Background/Purpose: We have previously reported that IL-18 levels in the lungs, serum and bronchoalveolar lavage fluid (BALF) were increased in patients with idiopathic pulmonary lung…Abstract Number: 1693 • ACR Convergence 2022
Skin Pigmentation Association with Regulation of Cyclic-GMP-AMP Following Skin Exposure to UV Light
Background/Purpose: Amongst the most important cytosolic DNA sensors is Cyclic GMP-AMP synthase (cGAS). Binding of DNA to cGAS results in the synthesis of the cyclic…Abstract Number: 0148 • ACR Convergence 2022
Dysferlin Associated Autoinflammatory Disease Causing Systemic Inflammation and Sterile Lung Abcesses Through Impaired Efferocytosis
Background/Purpose: Using whole exome sequencing (WES), we identified a de novo mutation in DYSF encoding dysferlin in 2 unrelated patients with systemic inflammation and sterile…Abstract Number: 1695 • ACR Convergence 2022
Dermatomyositis (DM) Macrophages Upregulate Genes Involved in the Remodeling of the Extracellular Matrix and Activate Keratinocytes in a Novel 3D Tissue Model of DM Skin Disease
Background/Purpose: With an incidence of 10 cases per million and lack of animal or ex vivo disease models, dermatomyositis (DM) remains a debilitating disease without…Abstract Number: 0149 • ACR Convergence 2022
Autoinflammatory Diseases Associated with NOD2 and Other Concurrent Genetic Mutations
Background/Purpose: Systemic autoinflammatoy diseases (SAIDs) are primarily caused by abnormal innate immune response. NOD-like receptors (NLRs) are intracellular sensors to the immune process, including NOD2,…Abstract Number: 1697 • ACR Convergence 2022
Measurements of Specific Activation Through the Lectin -or Classical Pathway of Complement in Patients with SLE
Background/Purpose: In systemic lupus erythematosus (SLE), the complement system is activated and commonly thought to occur through the classical pathway (CP) [1]. However, our previous…
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