Abstract Number: 0061 • ACR Convergence 2023
Discovery of a RIPK2 Scaffolding Inhibitor for the Treatment of Joint Autoimmune Diseases
Background/Purpose: Receptor interacting protein kinase 2 (RIPK2) is a key signaling node for inflammation caused by peptidoglycan (PGN). In the intestine, RIPK2 integrates signaling originating…Abstract Number: 0275 • ACR Convergence 2023
No Cumulative Effect of Infection Rates in Children Receiving Long-term Canakinumab Treatment in Autoinflammatory Periodic Fever Syndromes − Data from the RELIANCE Registry
Background/Purpose: Autoinflammatory diseases (AID) have been treated safely and effectively with the interleukin-1β inhibitor canakinumab (CAN) in controlled trials and routine clinical practice. The most…Abstract Number: 0276 • ACR Convergence 2023
Disease Control in Patients with Monogenetic Autoinflammatory Diseases Under Canakinumab Treatment – Comparison of 30 Months Interim Data from the RELIANCE Registry
Background/Purpose: Treatment of autoinflammatory periodic diseases (AID) with the interleukin-1β inhibitor canakinumab (CAN) has been shown to be safe and effective in controlled trials and…Abstract Number: 0724 • ACR Convergence 2023
Synovial Macrophage Subsets Defined by ScRNAseq Demonstrate Sexually Dimorphic Gene Expression in RA and a Mouse Inflammatory Arthritis Model
Background/Purpose: Macrophages (MФ) play a key pathogenic role in Rheumatoid Arthritis (RA), a disease that exhibits female sex bias. Recent work using scRNAseq has defined…Abstract Number: 0757 • ACR Convergence 2023
Identification of 23 Novel COPA Rare Exonic Non-Synonymous Variants and Their Associated Autoimmune and Inflammatory Clinical Phenotypes Among 53,364 Individuals
Background/Purpose: COPA syndrome is a rare, severe monogenic autoinflammatory/autoimmune disorder with variable penetrance, most commonly affecting the lungs and joints. It is caused by dominant…Abstract Number: 0797 • ACR Convergence 2023
Innate Lymphoid Cells Enhance Development of CD4+ T-cell Driven Autoimmune Arthritis
Background/Purpose: Innate Lymphoid cells (ILCs) are innate counterparts to T-cells that, based on their functional phenotype, can be divided into three subpopulations called Group 1…Abstract Number: 0838 • ACR Convergence 2023
Infusion of Etanercept into the Peripheral Lymphatics Significantly Reduces Disease Activity in Rheumatoid Arthritis Patients with Inadequate Response to Subcutaneous Injections
Background/Purpose: The lymphatic system serves as a conduit for transporting immune cells and inflammatory molecules present in arthritic joints to lymph nodes (LN), playing a…Abstract Number: 1712 • ACR Convergence 2023
Identification and Functional Characterization of Eight CANDLE/PRAAS Causing Proteasome Variants in Five Unrelated Patients
Background/Purpose: Mutations in genes coding for 20S proteasome subunits or proteasome assembly helpers cause chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperatures (CANDLE) or…Abstract Number: 1932 • ACR Convergence 2023
The Association Between Initial Ferritin and Complications from Adults Onset Stills Disease and Intensive Care Unit Admission
Background/Purpose: Adult Onset Still's Disease (AOSD) is an autoinflammatory disease characterized by quotidian fever, arthralgia, and a salmon colored rash. Due to its rarity, there…Abstract Number: 1936 • ACR Convergence 2023
Higher Rates of Disease Control During the Coronavirus Pandemic in Pediatric Patients with Autoinflammatory Periodic Diseases on Canakinumab Treatment – Interim Data from the RELIANCE Registry
Background/Purpose: Pediatric patients with autoinflammatory diseases (AID) on Canakinumab (CAN) therapy have been affected by the coronavirus pandemic including SARS-CoV-2 infection, SARS-CoV-2 vaccination, and AID…Abstract Number: 2358 • ACR Convergence 2023
Neutrophil Extracellular Traps Induce Endothelial-to-Mesenchymal Transition and Associate with Vascular Complications in Scleroderma
Background/Purpose: Systemic sclerosis (SSc, also known as scleroderma) is a disease characterized by fibrosis, autoimmunity, and vasculopathy. Many devastating complications such as digital ulcerations, scleroderma…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: 1744 • ACR Convergence 2022
Immune Responses to mRNA Vaccines Against SARS-CoV-2 in Patients with Immune-mediated Inflammatory Rheumatic Diseases
Background/Purpose: Patients with immune-mediated rheumatic diseases (IMRDs) are commonly treated with immunosuppressors and prone to infections. Recently introduced mRNA SARS-CoV-2 vaccines have demonstrated extraordinary efficacy…Abstract Number: 1842 • ACR Convergence 2022
Vaccination in Patients with Autoinflammatory Periodic Syndromes Under Canakinumab – Safety Data Interim Analysis of the RELIANCE Registry
Background/Purpose: Treatment of autoinflammatory periodic syndromes with the interleukin-1β inhibitor canakinumab (CAN) has been shown to be safe and effective in clinical trials and in…Abstract Number: 2213 • ACR Convergence 2022
Multi-omic Analysis of Macrophage Activation Syndrome Associated with sJIA Reveals a Potential Role of Type I Interferons in the Expansion of Cycling T Cells
Background/Purpose: Macrophage activation syndrome (MAS) is a complication of systemic juvenile idiopathic arthritis (sJIA) characterized by cytokine storm and overt immune cell activation. We aim…
ACR Meeting Abstracts