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: 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: 0812 • ACR Convergence 2022
3-years Safety and Efficacy Outcomes of Canakinumab Treatment in Cryopyrin-associated Periodic Syndromes (CAPS) – Data from the RELIANCE Registry
Background/Purpose: Cryopyrin-associated periodic syndromes (CAPS) are monogenic autoinflammatory diseases with severe systemic inflammation. The IL-1β inhibitor canakinumab (CAN) leads to a rapid remission of CAPS…Abstract Number: 0813 • ACR Convergence 2022
Canakinumab in Patients with Tumor Necrosis Factor Receptor-associated Periodic Syndrome (TRAPS) – Long-term Efficacy and Safety Data from a RELIANCE Registry Interim Analysis
Background/Purpose: TRAPS is a rare hereditary autoinflammatory disease characterized by periodic fever and severe systemic and organ inflammation. Successful treatment was achieved with the interleukin-1β…Abstract Number: 0825 • ACR Convergence 2022
Predicting Outcomes in Macrophage Activation Syndrome (MAS): A Retrospective Study on 43 Consecutive Patients with MAS
Background/Purpose: Macrophage activation syndrome is a diagnosis along the spectrum of hemophagocytic lymphohistiocytosis (HLH) spectrum disorders. It is a rare, yet potentially fatal disease characterized…Abstract Number: 1119 • ACR Convergence 2022
Investigating Macrophage Heterogeneity in the Esophagus and Lungs of SSc Patients
Background/Purpose: Our group has made important contributions to an emerging understanding of monocytes and macrophages as central to SSc pathogenesis. There are numerous studies that…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…Abstract Number: 0138 • ACR Convergence 2022
Patients with Familial Mediterranean Fever (FMF) Under Canakinumab Treatment – Long-term Efficacy and Safety Interim Data of the RELIANCE Registry
Background/Purpose: Familial Mediterranean fever (FMF) is a chronic disease characterized by recurrent episodes of fever and serositis, with a risk of severe complications (e. g.…Abstract Number: 0510 • ACR Convergence 2021
Fibrinogen Modified with Malondialdehyde-Acetaldehyde Adduct (MAA) And/or Citrulline (CIT) Induces Unique Cellular Responses in Human RA Synoviocytes
Background/Purpose: Malondialdehyde (MDA) is produced in response to oxidative stress and is associated with inflammation and disease pathogenesis. MDA can break down and form acetaldehyde…Abstract Number: 0948 • ACR Convergence 2021
Immunometabolism of Neutrophils in Antiphospholipid Syndrome (APS)
Background/Purpose: Neutrophil hyperactivity and neutrophil extracellular trap (NET) release (NETosis) appear to play roles in APS pathophysiology. Until recently, it was thought that neutrophils relied…Abstract Number: 1518 • ACR Convergence 2020
The Role for Neutrophils in the Early Phases of Enthesitis in Spondyloarthritis
Background/Purpose: Neutrophils are present in the early phases of spondyloarthritis (SpA)-associated uveitis, skin and intestinal disease, but their role in enthesitis remains unknown. We investigated…