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…Abstract Number: 0072 • ACR Convergence 2024
The Association Between Activation of Inflammasome and HLA-B27 in Dendritic Cells of Patients with Axial Spondyloarthritis
Background/Purpose: Axial spondyloarthritis (axSpA) exhibits a predominant involvement of the innate immune system, marked by abnormal functions of innate immune cell such as dendritic cells…Abstract Number: 0917 • ACR Convergence 2024
Role of Mutual Information Profile Shifts in Assessing the Pathogenicity of Mutations on Protein Functions: The Case of Pyrin Mutations in Familial Mediterranean Fever
Background/Purpose: Predicting the pathogenicity of amino acid substitutions is crucial for understanding the functional consequences of genetic variations. Several computational methods frequently used so far…Abstract Number: 0920 • ACR Convergence 2024
TGF-β Activated Kinase 1 Inhibition by Pentagalloyl Glucose Inhibits NLRP3 Inflammasome Formation and Ameliorates MSU-Induced Inflammation
Background/Purpose: Monosodium urate (MSU)-induced inflammation is caused by the deposition of MSU crystals in the joints and periarticular tissues under conditions of hyperuricemia. These deposits…Abstract Number: 0058 • ACR Convergence 2023
Macrophage Extracellular Traps Induced by Monosodium Urate or Calcium Pyrophosphate Crystals Form Independently of NLRP3 Inflammasome Activation
Background/Purpose: Monosodium urate (MSU) and calcium pyrophosphate (CPP) crystals are potent inducers of inflammation by activation of the inflammasome in neutrophils phagocytosing the crystals and…Abstract Number: 1106 • ACR Convergence 2023
Monosodium Urate and Calcium Pyrophosphate Crystal-induced Inflammation Relies on Cell Volume Regulation and LRRC8/VRAC Channel Activation
Background/Purpose: Monosodium urate (MSU) and calcium pyrophosphate (CPP) crystals are responsible for interleukin (IL)-1β dependent acute arthritis. The release of mature IL-1β is dependent on…Abstract Number: 2058 • ACR Convergence 2023
IL-1 Blocking Treatment Slows the Progression of Sensorineural Hearing Loss in Patients with NOMID
Background/Purpose: NOMID is a severe form of cryopyrin-associated periodic syndrome characterized by systemic inflammation and CNS manifestations, including sensorineural hearing loss. IL-1 blocking agents, Anakinra…Abstract Number: PP11 • ACR Convergence 2023
We Suffered For Decades, But Then She Was Born
Background/Purpose: I was born in 1981; my mother in 1949; and my grandmother in 1926. Our story runs at least four generations deep, that we…Abstract Number: 032 • 2023 Pediatric Rheumatology Symposium
From Bedside to Bench and Back: Discovery of a Novel Missense Variant in NLRP3 Causing Atypical Cryopyrin-Associated Periodic Syndromes with Hearing Loss as the Primary Presentation, Responsive to Anti-IL1 Therapy
Background/Purpose: Cryopyrin-associated periodic syndromes (CAPS) are a spectrum of rare autoinflammatory diseases caused by gain-of-function mutations inthe NLRP3 gene. These mutations cause inflammasome hyperactivity and…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: 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: 0557 • ACR Convergence 2022
VEXAS Syndrome Is Characterized by Blood and Tissues Inflammasome Pathway Activation and Monocyte Dysregulation
Background/Purpose: Acquired mutations in the UBA1 gene, occurring in myeloid cells and resulting in expression of a catalytically impaired isoform of the enzyme E1, were…Abstract Number: 0818 • ACR Convergence 2022
NLRP12-associated Autoinflammatory Disease in Chinese Adult Patients: A Single-center Study
Background/Purpose: NLRP12-associated autoinflammatory disease (NLRP12-AID) is an autosomal dominant autoinflammatory disorder caused by variants of the NLRP12 gene, which lead to a reduction of the…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: 1010 • ACR Convergence 2021
Proton Pump Inhibitors Suppress IL-1 Mediated Carditis in a Murine Model of Kawasaki Disease
Background/Purpose: Kawasaki disease (KD), is the leading cause of acquired heart disease in childhood. Up to 20% of patients may develop coronary artery lesions (CAL)…
ACR Meeting Abstracts