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  • Abstract Number: 1779 • 2019 ACR/ARP Annual Meeting

    CLEC5A/MDL-1 Is Critical for Inflammatory Arthritis and Skin Inflammation

    Cuong Nguyen1, Dai Hui 1, Ran Gu 1, Trevor Chan 2, Alina Marleev 3, Emanual Maverakis 3, Vijay Kuchroo 4, Shie-Liang Hsieh 5, Ilias Tagkopoulos 2, Christopher Ritchlin 6 and Iannis Adamopoulos 1, 1Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis, Sacramento, CA, 2University of California, Davis, Department of Computer Science, California, USA, Davis, CA, 3Department of Dermatology, School of Medicine, UCD, Sacramento, California, USA., Sacramento, CA, 4Center for Neurologic Diseases, Brigham and Women's Hospital, Harvard Medical School, MA, USA, Boston, MA, 5Genomics Research Center, Academia Sinica, 128 Academia Road, Section 2, Nankang, Taipei, Taiwan, Taipei, Taiwan (Republic of China), 6Division of Allergy, Immunology and Rheumatology, Center for Musculoskeletal Research, University of Rochester School of Medicine and Dentistry, Rochester, NY, USA, Rochester, NY

    Background/Purpose: Interleukin-23 (IL-23) and its cognate receptor interleukin-23R (IL-23R):IL-12Rβ1 plays a critical role in the pathogenesis of autoimmune diseases including psoriasis and psoriatic arthritis. Although…
  • Abstract Number: 1780 • 2019 ACR/ARP Annual Meeting

    Microbiome in Offspring of Ankylosing Spondylitis Patients

    Matthew Stoll1, Kimberly DeQuattro 2, Zhixiu Li 3, Henna Sawhney 4, Maria Castillo 4, Pamela F. Weiss 5, Peter Nigrovic 6, Lynn Punaro 7, Kenneth Schikler 8, Barbara Edelheit 9, John Reveille 10, Matthew Brown 3 and Lianne Gensler 11, 1University of Alabama at Birmingham, Birmingham, AL, 2University of California, San Francisco, San Francisco, CA, 3Queensland University of Technology, Queensland, Australia, 4University of California in San Francisco, San Francisco, CA, 5Children's Hospital of Philadelphia, Philadelphia, PA, 6Boston Children's Hospital, Boston, MA, 7Texas Scottish Rite Hospital, Dallas, TX, 8University of Kentucky, Louisville, KY, 9Connecticut Children's Medical Center, Hartford, CT, 10University of Texas McGovern Medical School, Houston, 11University San Francisco California, San Francisco, CA

    Background/Purpose: Spondyloarthritis (SpA) results from the interplay between genetic and environmental factors. An emerging factor is the human intestinal microbiota, which multiple studies in children…
  • Abstract Number: 1781 • 2019 ACR/ARP Annual Meeting

    Cytokine Dependence of Enthesis-resident Lymphocytes in Murine Spondyloarthritis

    Mederbek Matmusaev 1, Emma Haley1, Imtiyaz Hossain 1 and Joerg Ermann 1, 1Brigham and Women's Hospital, Boston

    Background/Purpose: Enthesis-resident CD4-CD8- double-negative (DN) T cells have been implicated in the pathogenesis of spondyloarthritis. Overexpression of IL-23 in adult mice activates these cells to…
  • Abstract Number: 1782 • 2019 ACR/ARP Annual Meeting

    A Human SLE Variant NCF1-R90H Drives Lupus-like Kidney Disease in a Pristane-induced Murine Lupus Model

    Lin-Yu Geng1, Ivan Molano 2, Ling-Xiao Xu 3, Qing Sun 1, Phillip Ruiz 4, Quan-Zhen Li 5, Gary Gilkeson 6 and Betty Tsao 7, 1Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, Charleston, South Carolina, USA., Charleston, SC, 2Division of Rheumatology & Immunology/Medical University of South Carolina, Charelston, 3Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, Charleston, South Carolina, USA., Charelston, SC, 4Department of Surgery/University of Miami School of Medicine, Charleston, 5Department of Immunology & Internal Medicine/University of Texas Southwestern Medical Center, Charleston, 6Division of Rheumatology & Immunology/Ralph H. Johnson VA Medical Center/Medical University of South Carolina, Charleston, SC, 7Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, Charleston, South Carolina, USA., Charleston

    Background/Purpose: We previously identify a p.Arg90His (p.R90H) substitution encoded in phagocyte neutrophil cytosolic factor (NCF1) as a novel risk variant for SLE, with decreased and…
  • Abstract Number: 1783 • 2019 ACR/ARP Annual Meeting

    Reversible Dysregulation of Renal Circadian Rhythm in Lupus Nephritis

    Rakesh Mishra 1, Ramalingam Bethunaickan 1, Celine Berthier 2, Zhengzi Yi 3, Joshua Strohl 1, Patricio Huerta 1, Weijia Zhang 4 and Anne Davidson5, 1Feinstein Institute for Medical Research, manhasset, NY, 2University of Michigan, Ann Arbor, MI, 3Mount Sinai School of Medicine, New York, 4Mount Sinai School of Medicine, New York, NY, 5Feinstein Institutes for Medical Research, Manhasset

    Background/Purpose: Circadian rhythm is a universal phenomenon that governs homeostasis of overall organism functioning as well as of individual organs. Circadian regulation of homeostatic functions…
  • Abstract Number: 1784 • 2019 ACR/ARP Annual Meeting

    Single Cell Analysis of Renal Myeloid Cells from NZB/WF1 Mice with Lupus Nephritis Reveals Multiple Subsets with Altered Functions

    Rakesh Mishra 1, Celine Berthier 2, Heather Geiger 3, Weijia Zhang 4 and Anne Davidson5, 1Feinstein Institute for Medical Research, manhasset, NY, 2University of Michigan, Ann Arbor, MI, 3New York Genome Center, New York, NY, 4Mount Sinai School of Medicine, New York, NY, 5Feinstein Institutes for Medical Research, Manhasset

    Background/Purpose: Renal infiltration with macrophages and dendritic cells is associated with poor prognosis in humans with lupus nephritis (LN). However, the precise pathogenic and/or protective…
  • Abstract Number: 1785 • 2019 ACR/ARP Annual Meeting

    The Pathogenic Potential of Choroid Plexus T Cells in Neuropsychiatric Lupus

    Erica Moore1, Michelle Huang 1, Cara Reynolds 1 and Chaim Putterman 2, 1Albert Einstein College of Medicine, Bronx, NY, 2Albert Einstein College of Medicine, New York, NY

    Background/Purpose: The pathogenesis of neuropsychiatric Systemic Lupus Erythematosus (NPSLE) is incompletely understood, but is considered to be complex and multifactorial. Additionally, there is a wide…
  • Abstract Number: 1786 • 2019 ACR/ARP Annual Meeting

    Surrogate Pathways of Complement Activation in Novel Polygenic SLE-like Models of Kidney Injury

    Sladjana Skopelja-Gardner1, Xizhang Sun 2, Yufeng Peng 2, Lucrezia Colonna 2, Payton Hermanson 2, Lena Tanaka 2, Joyce Tai 2, David Salant 3 and Keith Elkon 2, 1University of Washington, Seattle, WA, 2University of Washington, Seattle, 3Boston University, Boston

    Background/Purpose: Lupus nephritis (LN) is a major contributor to morbidity and mortality in systemic lupus erythematosus (SLE). Defective clearance of apoptotic cells (AC), autoantibodies, and…
  • Abstract Number: 1787 • 2019 ACR/ARP Annual Meeting

    The Glucocorticoid-Induced Protein GILZ Represent a Checkpoint in the IFN Program in SLE

    Eric Morand1, Champa Nataraja 1, Melissa Northcott 1, Wendy Dankers 1, Jacqueline Flynn 1, Wendy Zhu 1, Taylah Bennett 1, Mehnaz Pervin 1, Brendan Russ 1, James Harris 1 and Sarah Jones 1, 1Monash University, Melbourne, Victoria, Australia

    Background/Purpose: Given the primacy of Type I interferon (IFN) pathways in SLE pathogenesis, and the near-ubiquity of glucocorticoid (GC) use in SLE treatment, clarifying the…
  • Abstract Number: 1788 • 2019 ACR/ARP Annual Meeting

    Anti-neutrophil Extracellular Trap (NET) Autoantibodies in Primary Antiphospholipid Syndrome

    Yu Zuo1, Srilakshmi Yalavarthi 2, Kelsey Gockman 2, David Karp 3, Quan-Zhen Li 4 and Jason Knight 5, 1University of Texas Southwestern, Dallas, TX, 2University of Michigan, Ann Arbor, MI, 3UTSouthwestern Medical Center, Dallas, TX, 4Department of Immunology & Internal Medicine/University of Texas Southwestern Medical Center, Charleston, 5Division of Rheumatology, Department of Internal Medicine, Michigan Medicine, Ann Arbor, MI

    Background/Purpose: Neutrophil extracellular traps (NETs) are prothrombotic tangles of chromatin and microbicidal proteins ejected from neutrophils in response to a variety of stimuli.  In antiphospholipid…
  • Abstract Number: 1789 • 2019 ACR/ARP Annual Meeting

    Defibrotide Inhibits Antiphospholipid Antibody-Mediated NET Release and Endothelial Cell Activation

    Ramadan Ali1, Hui Shi 1, Srilakshmi Yalavarthi 1 and Jason Knight 2, 1University of Michigan, Ann Arbor, MI, 2Division of Rheumatology, Department of Internal Medicine, Michigan Medicine, Ann Arbor, MI

    Background/Purpose: Defibrotide is a mixture of phosphodiester oligonucleotides derived from porcine intestinal mucosa, currently approved for treatment of hepatic sinusoidal obstruction syndrome. Defibrotide has been…
  • Abstract Number: 1790 • 2019 ACR/ARP Annual Meeting

    The Epidemiology of the Antiphospholipid Syndrome in the UK, 1990 – 2016

    Mia Rodziewicz1, David D'Cruz 2, Martin Gulliford 3 and Nisha Hazra 3, 1The Louise Coote Lupus Unit, Guy's and St Thomas' Hospital NHS Foundation Trust, London, United Kingdom, 2The Louise Coote Lupus Unit, Guy's Hospital, London, England, United Kingdom, 3School of Population Health and Environmental Sciences, King’s College London, London, United Kingdom

    Background/Purpose: Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterised by recurrent vascular thrombosis and/or pregnancy morbidity in the presence of persistently positive antiphospholipid autoantibodies.…
  • Abstract Number: 1791 • 2019 ACR/ARP Annual Meeting

    Antiphospholipid Antibody Profile Stability over Time: Prospective Results from AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository (“Registry”)

    Elena Gkrouzman1, Ecem Sevim 2, Jackie Finik 3, Danieli Andrade 4, Vittorio Pengo 5, Savino Sciascia 6, Maria Tektonidou 7, Amaia Ugarte 8, Cecilia Chighizola 9, H Michael Belmont 10, Laura Pérez Sánchez 11, Lanlan Ji 12, Paul Fortin 13, Maria Efthymiou 14, Guilherme De Jesus 15, David Branch 16, Cecilia Nalli 17, Michelle Petri 18, Ricard Cervera 19, Esther Rodriguez 20, Jason Knight 21, Tatsuya Atsumi 22, Rohan Willis 23, Maria Laura Bertolaccini 24, Hannah Cohen 14, Jacob Rand 25, Doruk Erkan 1 and On Behalf Of APS ACTION 26, 1Hospital for Special Surgery, New York City, 2Hospital for Special Surgery, New York, NY, 3Hospital for Special Surgery, New York, NY, New York, NY, 4Hospital das Clinicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, Brazil, 5Azienda Ospedaliera of Padova, University of Padova, Padova, Italy, 6Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d’Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Torino, Italy, 7First Department of Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece, Athens, Greece, 8Hospital Universitario Cruces, University of the Basque Country Autoimmune Diseases Research Unit, Department of Internal M edicine, BioCruces Health, Biscay, Spain, 9Rheumatology, Istituto Auxologico Italiano, University of Milan, Milan, Italy, 10NYU Langone Health, New York, NY, 11IMIBIC/Reina Sofia Hospital/University of Cordoba, Cordoba, Spain, 12Peking University First Hospital, Beijing, China (People's Republic), 13Division de Rhumatologie, Département de Médecine, CHU de Québec – Université Laval, Axe maladies infectieuses et inflammatoires, Centre de recherche du CHU de Québec – Université Laval, Canada, Quebec, QC, Canada, 14University College London, London, United Kingdom, 15Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil, 16University of Utah Health Sciences Center, Salt Lake City, UT, 17Rheumatology and Clinical Immunology, Spedali Civili, Brescia, Italy, Brescia, Italy, 18Johns Hopkins University School of Medicine, Baltimore, MD, 19Hospital Clinic of Barcelona, Barcelona, Spain, 20Hospital Universitario 12 de Octubre, Madrid, Spain, 21Division of Rheumatology, Department of Internal Medicine, Michigan Medicine, Ann Arbor, MI, 22Hokkaido University, Sapporo, Japan, 23Antiphospholipid Standardization Laboratory, University of Texas Medical Branch, Galveston, TX, 24King's College London, London, United Kingdom, 25Weill Cornell Medicine, New York, 26APS ACTION, New York, NY

    Background/Purpose: APS ACTION "Registry" was created to study long-term outcomes in persistently antiphospholipid antibody (aPL)-positive patients with and without other systemic autoimmune diseases. Our primary…
  • Abstract Number: 1792 • 2019 ACR/ARP Annual Meeting

    Cognitive Dysfunction (CD) and Serum Levels of Brain-Derived Neurotrophic Factor (BDNF) in Primary Antiphospholipid Syndrome (PAPS)

    Renata Rosa1, Michelle Remião Ugolini-Lopes 2, Ana Paula Gandara 2, Kenia Campanholo 2, Livia Dutra 3 and Danieli Andrade 4, 1Faculdade de Medicina da Universidade de São Paulo (USP), Brazil., São Paulo, Sao Paulo, Brazil, 2Faculdade de Medicina da Universidade de São Paulo (USP), Brazil., São Paulo, Brazil, 3Escola Paulista de Medicina (EPM), Brazil, São Paulo, Brazil, 4Hospital das Clinicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, Brazil

    Background/Purpose: Cognition dysfunction (CD) is a poorly understood non-stroke central neurologic manifestation of antiphospholipid syndrome, whose diagnosis involves a specific neuropsychological (NP) evaluation. Brain-derived neurotrophic…
  • Abstract Number: 1793 • 2019 ACR/ARP Annual Meeting

    Descriptive Analysis of Biopsy-proven Antiphospholipid Antibody-associated Nephropathy Patients Included in the AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository (“Registry”)

    Maxime Taghavi1, Medha Barbhaiya 2, Maria Tektonidou 3, Paul Fortin 4, Danieli Andrade 5, Jason Knight 6, Bahar Artim-Esen 7, Tatsuya Atsumi 8, Hannah Cohen 9, Lanlan Ji 10, Savino Sciascia 11, Surya Seshan 12, Doruk Erkan 13 and on Behalf of APS ACTION 14, 1Brugmann Hospital, Brugmann, Belgium, 2Hospital for Special Surgery, Weill Cornell Medicine, New York, NY, 3First Department of Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece, Athens, Greece, 4Division de Rhumatologie, Département de Médecine, CHU de Québec – Université Laval, Axe maladies infectieuses et inflammatoires, Centre de recherche du CHU de Québec – Université Laval, Canada, Quebec, QC, Canada, 5Hospital das Clinicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, Brazil, 6Division of Rheumatology, Department of Internal Medicine, Michigan Medicine, Ann Arbor, MI, 7Istanbul University School of Medicine, Istanbul, Turkey, 8Hokkaido University, Sapporo, Japan, 9University College London, London, United Kingdom, 10Peking University First Hospital, Beijing, China (People's Republic), 11Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d’Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Torino, Italy, 12Weill Cornell Medicine, New York, 13Hospital for Special Surgery, New York City, 14Hospital for Special Surgery, New York

    Background/Purpose: Antiphospholipid antibody (aPL) nephropathy is a distinct entity that can be challenging to recognize and treat; it remains unknown if uniform pathologic criteria are…
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