Abstracts tagged "innate immunity"

Abstract Number: 0059 • ACR Convergence 2021
Deep Immune Cell Profiling of Tissue Microenvironment Using Imaging Mass Cytometry in Psoriatic Disease
Lihi Eder¹, Stephane Caucheteux¹, Somi Afiuni², Adriana Krizova¹, James Limacher¹, Hartland Jackson¹ and Vincent Piguet¹, ¹University of Toronto, Toronto, ON, Canada, ²Lunenfeld Tanenbaum Research Institute, Sinai Health System, Toronto, ON, Canada
Background/Purpose: Cross talk between different cell types, such as T cell subsets and other immune cell populations, is important in psoriatic disease. Imaging Mass Cytometry…
Abstract Number: 0461 • ACR Convergence 2020
High-Throughput Single-Cell Analysis Reveals Unique Lung Cellular Subsets in a Murine Model of Rheumatoid Arthritis-Inflammatory Lung Disease
Rohit Gaurav¹, Ted Mikuls¹, Geoffrey Thiele¹, Amy Nelson¹, Meng Niu¹, Chittibabu Guda¹, James Eudy¹, Austin Barry¹, Debra Romberger¹, Michael Duryee¹, Bryant England¹ and Jill Poole¹, ¹University of Nebraska Medical Center, Omaha, NE
Background/Purpose: Rheumatoid arthritis (RA)-associated inflammatory lung disease is an extra-articular manifestation of RA associated with increased morbidity and mortality, whose precise molecular mechanisms remain undetermined.…
Abstract Number: 0462 • ACR Convergence 2020
Lupus-like Autoimmunity and Increased Interferon Response in Patients with STAT3-deficient Hyper-IgE Syndrome
Brian Dizon¹, Rishi Goel², Shuichiro Nakabo², Amanda Urban², Meryl Waldman², Lilian Howard², Dirk Darnell², Munir Buhaya², Sarfaraz Hasni³, Mariana Kaplan⁴, Alexandra Freeman² and Sarthak Gupta¹, ¹National Institutes of Health, BETHESDA, MD, ²National Institutes of Health, Bethesda, ³Lupus Clinical Trials Unit, NIAMS/NIH, Bethesda, MD, ⁴National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD
Background/Purpose: Autosomal dominant hyper-IgE syndrome (AD-HIES), also known as Job’s syndrome, is a rare primary immunodeficiency caused by dominant-negative loss-of-function (LOF) mutations in signal transducer…
Abstract Number: 0463 • ACR Convergence 2020
Molecular Diagnosis of Childhood Immunodysregulation, Endocrinopathy and Enteropathy X-linked (IPEX)-Like Syndrome and Implications for Clinical Management
Sarah Baxter¹, Tom Walsh¹, Silvia Casadei¹, Suleyman Gulsuner¹, Eric Allenspach², David Hagin³, Gesmar Segundo⁴, Troy Torgerson⁵ and Mary-Claire King¹, ¹University of Washington, Seattle, ²Seattle Children's Research Institute, Seattle, ³Tel Aviv Medical Center, Tel Aviv, Israel, ⁴Universidade Federal de Uberlandia, Uberlandia, Brazil, ⁵Allen Institute, Seattle
Background/Purpose: Patients with early-onset immunodysregulation, endocrinopathy and enteropathy but without identified mutations in FOXP3 are termed “IPEX-like,” and undergo trial-and-error immunosuppressive treatment with highly variable…
Abstract Number: 0469 • ACR Convergence 2020
IFNγ Is Essential for Alveolar Macrophage Driven Lung Inflammation in Macrophage Activation Syndrome
Denny Gao¹, Maggie Henderlight¹, Christopher Woods¹, Alexei Grom¹, Sherry Thornton¹, Michael Jordan¹, Katheryn Wikenheiser-Brokamp¹ and Grant Schulert², ¹Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
Background/Purpose: Macrophage activation syndrome (MAS) is a life-threatening cytokine storm syndrome frequently complicating systemic juvenile idiopathic arthritis (SJIA) and driven by IFNγ. MAS is also…
Abstract Number: 0644 • ACR Convergence 2020
Characterizations of Cytokine Storm Associated with COVID19
Ofer Perzon¹, Avi Abutbul¹, Sigal Sviri¹ and Dror Mevorach¹, ¹Hadassah-University Hospital, Jerusalem, Yerushalayim, Israel
Background/Purpose: COVID-19, the name given to the clinical syndrome associated with the newly recognized virus SARS-CoV-2 has become pandemic with mortality estimated based on reports…
Abstract Number: 0949 • ACR Convergence 2020
SLAMF7 Engagement Drives Monocyte Super-Activation in Acute and Chronic Inflammation
Daimon Simmons¹, Hung Nguyen², Emma Gomez-Rivas³, YunJu Jeong⁴, William Apruzzese⁵, Edy Kim⁶ and Michael Brenner⁷, ¹Department of Pathology, Brigham and Women's Hospital, Boston, MA, ²Division of Rheumatology, Brigham and Women's Hospital,, Boston, MA, ³Division of Rheumatology, Brigham and Women's Hospital, Boston, MA, ⁴Division of Pulmonary and Critical Care, Brigham and Women's Hospital, Boston, MA, ⁵., Boston, ⁶Brigham and Women’s Hospital, Division of Pulmonary and Critical Care Medicine, Boston, MA, ⁷Brigham and Women's Hospital and Harvard Medical School, Boston, MA
Background/Purpose: Monocytes orchestrate immune responses that protect against microbes but can also drive pathological inflammation and autoimmune disease. Monocytes are thought to be activated primarily…
Abstract Number: 1156 • ACR Convergence 2020
Comparison of Immunological Biomarkers and Lung Histology in Patients with Elevated IL18 – Pulmonary Alveolar Proteinosis and Recurrent Macrophage Activation Syndrome (IL-18PAP-MAS) and Other Inflammatory Lung Diseases
Alhanouf Alsaleem¹, Adriana de Jesus², Sofia Torreggiani³, Chyi-Chia Lee⁴, Les Folio⁵, Huy Do⁶, Andrew Oler⁷, Caroline Kim³, Stewart Levine⁸, Anthony Suffredini⁹, Cem Gabay¹⁰, Joseph Fontana¹¹, Scott Canna¹² and Raphaela Goldbach-Mansky¹³, ¹Division of Pediatric Rheumatology, Department of pediatrics, King Faisal specialist hospital and research center, Riyadh, Saudi Arabia, RiYADH, Saudi Arabia, ²Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Silver Spring, MD, ³Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, ⁴Pathology Department/NCI/NIH, Bethesda, MD, ⁵Radiology and Imaging Services/NIH, Bethesda, MD, ⁶Radiology and Imaging Sciences, Bethesda, MD, ⁷Bioinformatics and Computational Biosciences Branch/NIAID/NIH, Bethesda, MD, ⁸Laboratory of Asthma and Lung Inflammation, Division of Intramural Research, NHLBI, NIH,, Bethesda, MD, ⁹Critical Care Medicine Department, Clinical Center, NIH, Bethesda, MD, ¹⁰University Hospitals of Geneva, Geneva, Switzerland, ¹¹NHLBI/NIH, Bethesda, MD, ¹²University of PIttsburgh, Pittsburgh, PA, ¹³Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD
Background/Purpose: Recently, pulmonary alveolar proteinosis (PAP) and recurrent macrophage activation syndrome (MAS) have been reported in rare patients (pts) with systemic juvenile idiopathic arthritis (SJIA)…
Abstract Number: 1217 • ACR Convergence 2020
Effects of JAK Inhibitors Against JAK2-mediated Signaling in Innate Immune Cells
Yuya Fujita¹, Naoki Matsuoka¹, Makiko Furuya-Yashiro², Jumpei Temmoku², Yuki Kuroiwa³, Masaru Tanaka⁴, Tomoyuki Asano², Shuzo Sato⁵, Haruki Matsumoto², Hiroshi Watanabe², Hideko Kuzuru⁶, Hiroshi Yatsuhashi⁷, Atsushi Kawakami⁸ and Kiyoshi Migita⁹, ¹Fukushima Medical University School of Medicine, Department of Rheumatology, Fukushima, Fukushima, Japan, ²Fukushima Medical University School of Medicine, Department of Rheumatology, Fukushima, Japan, ³Eli Lilly Japan K.K., Tokyo, ⁴Eli Lilly Japan K.K., Tokyo, Japan, ⁵Fukushima Medical University School of Medicine, Department of Rheumatology, Fukushima, Japan, ⁶NHO Nagasaki Medical Center, Clinical Research Center, Omura, Japan, ⁷NHO Nagasaki Medical Center, Clinical Research Center, Omura, Nagasaki, Japan, ⁸Nagasaki University Graduate School of Biomedical Sciences, Unit of Advanced Preventive Medical Sciences, Department of Immunology and Rheumatology, Nagasaki, Nagasaki, Japan, ⁹Fukushima Medical University School of Medicine, Department of Rheumatology, Fukushima, Fukushima, Japan
Background/Purpose: Janus kinase (JAK) family is comprised of JAK1, JAK2, JAK3 and tyrosine kinase 2 (TYK2). JAKs form homo- or hetero-complexes, the combination of which…
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
Kathleen Baysac¹, Charles Fisher², Hiroto Nakano², Joshua Milner³ and Michael Ombrello⁴, ¹NIAMS, NIH, Bethesda, MD, ²NIAMS, NIH, Bethesda, ³Division of Allergy, Immunology and Rheumatology Columbia University Medical Center, New York, NY, ⁴Translational Genetics and Genomics Unit, NIAMS, NIH, Bethesda, MD
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
Akash Gupta¹, Gunjan Arora¹, Connor Rosen², Yongguo Cao¹, Jiri Cerny³, Carmen Booth⁴, Noah Palm², Aaron Ring² and Erol Fikrig¹, ¹Section of Infectious Diseases, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT, ²Department of Immunobiology, Yale University School of Medicine, New Haven, CT, ³Faculty of Tropical AgriSciences, Czech University of Life Sciences in Prague, Prague, Czech Republic, ⁴Department of Comparative Medicine, Yale University School of Medicine, New Haven, CT
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
Adam Berlinberg¹, Adam Lefferts², Emilie Regner³, Andrew Stahly⁴ and Kristine Kuhn⁴, ¹University of Colorado, Denver, CO, ²University of Colorado, Anschutz Medical Campus, Denver, CO, ³University of California San Francisco, San Francisco, CA, ⁴University of Colorado Anschutz Medical Campus, Aurora, CO
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
Eloi Franco-Trepat¹, Ana Alonso-Pérez¹, Maria Guillán-Fresco¹, Miriam López-Fagundez¹, Andrés Pazos-Pérez¹, Ana Lois Iglesias², Susana Belén Bravo³, Alberto Jorge-Mora¹, JJ Gómez-Reino⁴ and Rodolfo Gómez¹, ¹IDIS-CHUS - Musculoskeletal Pathology Group, Santiago de Compostela, Spain, ²IDIS-CHUS - Musculoskeletal Pathology Group, A Coruna, Spain, ³IDIS-CHUS - Proteomics Unit, Santiago de Compostela, Galicia, Spain, ⁴IDIS-CHUS - Rheumatology Group, Santiago de Compostela, Spain
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
Farzana Bhuyan¹, Adriana Almeida de Jesus², Kim Johnson³, Jacob Mitchell⁴, Yan Huang⁵ and Raphaela Goldbach-Mansky⁵, ¹NIH, bhetesda, MD, ²Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, ³NIH, NIAID, Bethesda, ⁴Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, ⁵NIH, Bethesda
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
Rebecca Schriefer¹, Michelle Elvington², Priyan Weerappuli³ and Alfred Kim⁴, ¹Washington University School of Medicine, Saint Louis, MO, ²Kypha, Inc., Saint Louis, MO, ³University of Michigan, Ann Arbor, MI, ⁴Washington University School of Medicine, St. Louis, MO
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…

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