Abstracts tagged "innate immunity"

Abstract Number: 127 • 2023 Pediatric Rheumatology Symposium
What’s in a Name? A20 Protein Expression in an in Vitro Model of A20 Haploinsufficiency
Patricia Pontes Aires¹, DANIELA GERENT PETRY PIOTTO¹, Andre Cunha¹, Sandro Perazzio² and Maria Teresa Terreri³, ¹Universidade Federal de São Paulo, São Paulo, Brazil, ²Universidade de So Paulo; Fleury Laboratories, São Paulo, Brazil, ³UNIFESP, São Paulo, Brazil
Background/Purpose: Tumor necrosis factor alpha (TNF-alpha) induced protein 3 gene, or TNFAIP3, encodes the A20 protein, an important regulator of the NF-κB pathway. Since its…
Abstract Number: 0148 • ACR Convergence 2022
Dysferlin Associated Autoinflammatory Disease Causing Systemic Inflammation and Sterile Lung Abcesses Through Impaired Efferocytosis
Farzana Bhuyan¹, Adriana Almeida de Jesus², Clinton Bradfield³, Mohammad Arif Rahman⁴, Andre Rastegar³, sundar ganesan³, Sachin Gaurav³, Sara Alehashemi³, Ronit Herzog⁵, Steven Holland³, Deborah Consilini⁶, Iain Fraser³ and Raphaela Goldbach-Mansky⁷, ¹National Institutes of Health, Bethesda, MD, ²NIAID, NIH, Silver Spring, MD, ³NIAID, Bethesda, MD, ⁴NCI, Bethesda, MD, ⁵Langone Medical Center, New York, NY, ⁶Nemours Children's Hospital-Delaware, Wilmington, DE, ⁷NIAID, NIH, Rockville
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
Zoe Chafouleas¹, Angelique Cortez¹, James Whitley¹, Dorothea Barton¹, Lin Brown¹, Michael Whitfield², Patricia pioli³ and Sladjana Skopelja-Gardner¹, ¹Dartmouth Hitchcock Medical Center, Lebanon, NH, ²Dartmouth Geisel School of Medicine, Department of Biomedical Data Science, Lebanon, NH, ³Geisel School of Medicine at Dartmouth, Lebanon, NH
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
Hafsa Nomani, Brianne Navetta-Modrov, Mark Yun and QingPing Yao, Stony Brook University, Stony Brook, NY
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
Anne Troldborg¹, Mads Lamm Larsen¹, Erik J.M. Toonen², Lisa Hurler³, Zoltan Prohaszka³, László Cervenak³, Annette Gudmann Hansen⁴ and Steffen Thiel⁴, ¹Department of Rheumatology, Aarhus University Hospital, Aarhus, Denmark, ²Hycult Biotech, Arnhem-Nijmegen, Netherlands, ³Semmelweis University, Budapest, Hungary, ⁴Aarhus University, Aarhus, Denmark
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…
Abstract Number: 0554 • ACR Convergence 2022
Loss of Synovial Tissue Macrophage Homeostasis Precedes Rheumatoid Arthritis Clinical Onset
Megan Hanlon¹, Mary Canavan², conor Smith³, Achilleas Floudas⁴, Nuno Neto⁵, Qingxuan Song⁶, Phil Gallagher⁷, Ronan Mullan⁸, Conor Hurson⁹, Barry Moran⁵, Michael Monaghan⁵, Sunil Nagpal¹⁰, Douglas Veale¹¹ and Ursula Fearon⁵, ¹Molecular Rheumatology, Dublin, Ireland, ²Trinity College, Dublin, Ireland, ³bTrinity College Dublin, Dublin, Ireland, ⁴Molecular Rheumatology Trinity Biomedical Sciences Institute, Dublin, Ireland, ⁵Trinity College Dublin, Dublin, Ireland, ⁶Janssen Research and Development, LLC, Spring House, PA, ⁷St Vincent's University Hospital, Dublin, Ireland, ⁸Tallaght University Hospital, Dublin, Ireland, ⁹St Vincents University Hospital, Dublin, Ireland, ¹⁰Janssen Research, Collegeville, PA, ¹¹St. Vincent's University Hospital, Blackrock, Dublin, Ireland
Background/Purpose: Synovial-tissue macrophages significantly contribute to Rheumatoid Arthritis, yet the precise nature/function of macrophage subsets within the inflamed joint remains unexplored. Here we explore the…
Abstract Number: 1700 • ACR Convergence 2022
IL-21/STAT3 Pathway Mediated Th2-like Vδ2 T Cells Promote Plasmablasts Differentiation in IgG4-Related Disease
Jieqiong Li¹ and Wen Zhang², ¹Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China, ²Peking Union Medical College Hospital, Beijing, China
Background/Purpose: To explore the phenotype and role of gamma delta (γδ) T cells in the pathogenesis of IgG4-related disease (IgG4-RD), focusing on the effect on…
Abstract Number: 0556 • ACR Convergence 2022
Immune Checkpoint VISTA Regulates Type I Interferon (IFN-I) Production and Controls UV Light Triggered Skin IFN-I Response
Zachary Peters¹, Lindsay Mendyka¹, Sicong Shan¹, Angelique Cortez¹, William Rigby², Christopher Burns¹, Randolph Noelle³ and Sladjana Skopelja-Gardner¹, ¹Dartmouth Hitchcock Medical Center, Lebanon, NH, ²Dartmouth Hitchcock Medical Center, Lebanon, PA, ³Geisel School of Medicine at Dartmouth, Lebanon, NH
Background/Purpose: Most lupus patients chronically exhibit higher IFN-I scores in the skin, peripheral blood, and kidneys that is exacerbated by ultraviolet (UV) light. In normal…
Abstract Number: 1702 • ACR Convergence 2022
Targeting CD6-CD318 Axis with UMCD6 (anti-CD6) Enhances in Vivo Killing of Cancer Cells Through Direct Activation of NK Cells
Mikel Gurrea-Rubio¹, Qi Wu¹, Eliza Pei-Suen Tsou¹, M. Asif Amin¹, Phillip Campbell¹, Peggy Randon¹, Matthew Lind¹, Sarah Ory¹, Camila Amarista¹, Sirapa Vichaikul², Jeffrey Ruth¹, Feng Ling³ and David Fox¹, ¹University of Michigan, Ann Arbor, MI, ²Michigan Medicine, Howell, MI, ³Cleveland Clinic, Cleveland, OH
Background/Purpose: Immune checkpoint inhibitors represent a major advance in cancer treatment, but disease prognosis continues to be poor for most patients. Resistance of cancer cells…
Abstract Number: 0562 • ACR Convergence 2022
Large-Scale Targeted Sequencing Study Links Systemic Juvenile Idiopathic Arthritis with Rare Variants of MEFV, LYST, STXBP2, UNC13D
Mariana Correia Marques¹, Danielle Rubin², Emily Shuldiner², Elizabeth Schmitz², Elizabeth Baskin², Andrew Patt³, Alexei Grom⁴, Dirk Foell⁵, Marco Gattorno⁶, John Bohnsack⁷, Rae Yeung⁸, Sampath Prahalad⁹, Elizabeth Mellins¹⁰, Jordi Antón¹¹, Claudio Len¹², Sheila Oliveira¹³, Patricia Woo¹⁴, Seza Ozen¹⁵, INCHARGE Consortium¹⁶ and Michael Ombrello¹⁷, ¹National Institute of Arthritis and Musculoskeletal and Skin Diseases / Children`s National Hospital, Bethesda, MD, ²National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ³National Center for Advancing Translational Sciences, Bethesda, MD, ⁴Divisions of Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ⁵University Hospital Münster, Münster, Germany, ⁶Pediatric Clinic and Rheumatology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy, ⁷University of Utah, Salt Lake City, UT, ⁸The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada, ⁹Emory + Children's Pediatric Institute, Atlanta, GA, ¹⁰Stanford University, Stanford, CA, ¹¹Pediatric Rheumatology Department. Hospital Sant Joan de Déu. Universitat de Barcelona, Esplugues de Llobregat, Spain, ¹²Universidade Federal de São Paulo, São Paulo, Brazil, ¹³Universidade Federal do Rio de Janeiro, Rio De Janeiro, Brazil, ¹⁴University College London, London, United Kingdom, ¹⁵Hacettepe University Faculty of Medicine, Ankara, Turkey, ¹⁶International Childhood Arthritis Genetics Consortium, Bethesda, MD, ¹⁷National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a genetically complex inflammatory condition. It can be marked by severe systemic inflammation that resembles the hereditary periodic…
Abstract Number: 1704 • ACR Convergence 2022
Interaction of Macrophages and Natural Killer Cells in Pathogenesis of HIV-1-Associated Inflammatory Arthritis
Can Sungur¹, Gao Hongbo¹, Li-ping Yang², Liang Shan¹ and Wayne Yokoyama³, ¹Washington University, St. Louis, MO, ²Washington University in St. Louis, St. Louis, MO, ³Washington University School of Medicine, St. Louis, MO
Background/Purpose: Human immunodeficiency virus (HIV) remains a significant life-threatening agent and burden on public health. Lesser studied and understood aspects of HIV infections include HIV-associated…
Abstract Number: 0621 • ACR Convergence 2022
Altered Immunological Circadian Rhythms and the Effect of Treatment with Glucocorticoids on Circadian Rhythms of Immune Cells in Patients with Rheumatoid Arthritis: Bring Back the Rhythm
Siska Wilantri¹, Cindy Strehl¹, Dimas Abdirama¹, Timo Gaber¹, Robert Biesen² and Frank Buttgereit¹, ¹Charité Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin / DRFZ Berlin, Berlin, Germany, ²Charité Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
Background/Purpose: Clinical symptoms of rheumatoid arthritis (RA), including pain, joint stiffness, and swelling, exhibit a distinct circadian rhythm that exacerbates in the early morning in…
Abstract Number: 1711 • ACR Convergence 2022
The Cellular Basis for Type I Interferon Production Following Ultraviolet Light Stimulated Cyclic-GMP-AMP Synthase Activation in the Skin
Jie An, Xizhang Sun, Lena Tanaka and Keith Elkon, University of Washington, Seattle, WA
Background/Purpose: SLE patients characteristically have a type I interferon (IFN-I) signature in peripheral blood cells and this same signature is prominent in lesional and non-lesional…
Abstract Number: 0661 • ACR Convergence 2022
Cutaneous Type I IFN Responses in Systemic Lupus Erythematosus Are Associated with Inflammatory Phenotype and Altered Wound Healing Function of Lupus Fibroblasts from Non-Lesional Skin
Lisa Abernathy-Close, Suzanne Shoffner-Beck, Annie Lu, Amanda Victory, Amy Hurst, Craig Dobry, Rachael Wasikowski, Johann Gudjonsson, Alex Tsoi and J. Michelle Kahlenberg, University of Michigan, Ann Arbor, MI
Background/Purpose: Cutaneous lupus erythematosus (CLE) is a heterogenous, disfiguring, and difficult-to-treat manifestation of systemic lupus erythematosus (SLE) with scar formation in CLE subtypes such as…
Abstract Number: 1730 • ACR Convergence 2022
T Cell-Macrophage Interactions Play a Critical Role in a Mouse Model of Histidyl-tRNA Synthetase-Induced Myositis
Daniel Reay¹, Ying Wang², Wael Jarjour³, Paula Clemens¹ and Dana Ascherman⁴, ¹University of Pittsburgh School of Medicine, Pittsburgh, PA, ²University of Miami Miller School of Medicine, Miami, FL, ³The Ohio State University, Columbus, OH, ⁴University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Histidyl-tRNA synthetase (HRS) is a key target of antigen-specific B and T cell responses in the anti-synthetase syndrome. Despite a clear role for aberrant…

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