ACR Meeting Abstracts

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Abstracts tagged "Autoinflammatory diseases"

  • Abstract Number: 1452 • ACR Convergence 2021

    COVID-19 Infection Among Autoimmune/Auto-inflammatory Rheumatic Disease Patients: Data from an Observational Study

    Clio Mavragani1, Athanasios-Dimitrios Bakasis2, Kyriaki Boki3, Athanasios Tzioufas4, Panayiotis Vlachoyiannopoulos1, Ioanna Stergiou5, Fotini Skopouli6 and Haralampos Moutsopoulos7, 1National and Kapodistrian University of Athens, Athens, Greece, 21 Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece, Athens, Greece, 33 Rheumatology Unit, Sismanoglio General Hospital, Athens, Greece, Athens, Greece, 4Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece, 5Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, 6Department of Nutrition and Clinical Dietetics, Harokopio University of Athens, Athens, Greece, 7Athens Academy of Athens, Chair Medical Sciences/Immunology, Athens, Greece

    Background/Purpose: The impact of COVID-19 infection in patients with autoimmune/auto-inflammatory rheumatic diseases (AARD) under immunomodulatory treatment is not entirely clear and deeper knowledge is of…
  • Abstract Number: 0539 • ACR Convergence 2021

    Vgll3-transgenic Autoimmune Mice Display Features of Cutaneous Fibrosis

    Mehrnaz Gharaee-Kermani1, Allison Billi2, Marisa Hildebrandt2, Jacob Martens3, Rachael Wasikowski2, Joanne (Michelle) Kahlenberg1 and Johann Gudjonsson2, 1Internal Medicine - Division of Rheumatology and Department of Dermatology, University of Michigan, Ann Arbor, MI, 2Department of Dermatology, University of Michigan, Ann Arbor, MI, 3Graduate Program in Immunology, University of Michigan, Ann Arbor, MI, USA, Ann Arbor, MI

    Background/Purpose: Fibrosis is characterized by collagen deposition, fibro/myofibroblast accumulation, and extracellular matrix remodeling and can lead to disfiguring skin changes. In cutaneous lupus, scar formation…
  • Abstract Number: 1454 • ACR Convergence 2021

    RHAPSODY: Rilonacept, an IL-1α and IL-1β Trap, Resolves Pericarditis Episodes and Reduces Risk of Recurrence in a Phase 3 Trial of Patients with Recurrent Pericarditis

    Allan Klein1, Massimo Imazio2, Paul Cremer1, Antonio Brucato3, Antonio Abbate4, Fang Fang5, Antonella Insalaco6, Martin LeWinter7, Basil S. Lewis8, David Lin9, Sushil A. Luis10, Stephen J. Nicholls11, Arian Pano5, Alistair Wheeler12, Liangxing Zou5 and John F Paolini5, 1Department of Cardiovascular Imaging, Center for the Diagnosis and Treatment of Pericardial Diseases, Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, 2University Cardiology, Cardiovascular and Thoracic Department, AOU Città della Salute e della Scienza di Torino, Torino, Italy, 3Department of Biomedical and Clinical Science, University of Milano, Fatebenefratelli Hospital, Milan, Italy, 4VCU Pauley Heart Center, Virginia Commonwealth University, Richmond, VA, 5Kiniksa Pharmaceuticals Corp., Lexington, MA, 6Department of Pediatric Medicine, Division of Rheumatology, Bambino Gesù Children's Hospital, Rome, Italy, 7Cardiology Unit, The University of Vermont Medical Center, The University of Vermont, Burlington, VT, 8Cardiovascular Clinical Research Institute, Lady Davis Carmel Medical Center, Haifa, Israel, 9Minneapolis Heart Institute at Abbott Northwestern Hospital, Minneapolis, MN, 10Division of Cardiovascular Ultrasound, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, NY, 11MonashHeart, Department of Cardiology, Monash University, Clayton, Australia, 12Kiniksa Pharmaceuticals, Ltd., Hamilton, Bermuda

    Background/Purpose: Recurrent pericarditis (RP) is an autoinflammatory disease with no FDA-approved therapies. RHAPSODY, a global Phase 3 study, evaluated rilonacept, a once-weekly IL-1α/IL-1β trap, in…
  • Abstract Number: 0565 • ACR Convergence 2021

    Increased Risk for Inflammatory Arthritis in Veterans with Depression or Anxiety

    Brian Breviu1, Shaobo Pei1, Matthew Kirkpatrick2, Bingjian Feng1 and Jessica Walsh3, 1Salt Lake City Veteran Affairs and University of Utah Medical Centers, Salt Lake City, UT, 2University of Utah, Salt Lake City, UT, 3Salt Lake City Veteran Affairs Medical Center (VAMC)/University of Utah Hospital, Salt Lake City, UT

    Background/Purpose: Depression and anxiety are common in patients with inflammatory arthritis (IA), and have been reported as risk factors for various inflammatory diseases. The study…
  • Abstract Number: 1457 • ACR Convergence 2021

    Clinical Heterogeneity of the VEXAS Syndrome: A Case Series

    Matthew Koster1, Taxiarchis Kourelis1, Kaaren Reichard1, Tanaz Kermani2, David Beck3, Daniela Ospina Cardona4, Matthew Samec1, Abhishek Mangaonkar1, Kebede Begna1, Christopher Hook1, Jennifer Oliveira1, Samih Nasr1, Benedict Tiong5, Mrinal Patnaik1, Michelle Burke1, Clement Michet1 and Kenneth Warrington6, 1Mayo Clinic, Rochester, MN, 2University of California Los Angeles, West Hills, CA, 3National Institutes of Health, Bethesda, MD, 4National Institutes of Health, Germantown, MD, 5University of California Los Angeles Medical Center, Culver City, CA, 6Mayo Clinic, Rochester, Minnesota

    Background/Purpose: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently described myeloid-driven autoinflammatory condition caused by somatic mutations affecting methionine-41 (p.Met41) in the…
  • Abstract Number: 0947 • ACR Convergence 2021

    Cytokine Competent Gut-joint Migratory T Cells Contribute to Inflammation in the Joint

    Adam Lefferts1, David Claypool1, Eric Norman1, Uma Kantheti1 and Kristine Kuhn2, 1University of Colorado, Anschutz Medical Campus, Aurora, CO, 2University of Colorado Anschutz Medical Campus, Aurora, CO

    Background/Purpose: Although studies have identified the presence of gut-associated cells in the enthesis of joints affected by spondyloarthritis, a direct link through cellular transit between…
  • Abstract Number: 1508 • ACR Convergence 2021

    Decrease of Angiogenic T Cells Associated to the Presence of Interstitial Lung Disease in Patients with Connective Tissue Diseases

    Verónica Pulito-Cueto1, Sara Remuzgo-Martinez1, Fernanda Genre1, Belén Atienza-Mateo2, Victor M. Mora-Cuesta3, David Iturbe-Fernández3, Leticia Lera-Gómez1, Raquel Perez-Fernández1, Pilar Alonso-Lecue4, Javier Rodriguez-Carrio5, Diana Prieto-Peña6, Virginia Portilla6, Ricardo BLANCO7, Alfosno Corrales6, José M. Cifrián8, Raquel López-Mejías1 and Miguel Ángel gonzalez-Gay9, 1Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL, Santander, Spain, 2Group "Research in genetic epidemiology and atherosclerosis of systemic diseases and in bone metabolic diseases of the locomotor system", IDIVAL; and Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, 3Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System; Department of Pneumology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, 4Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL; Department of Pneumology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, 5Deparment of Functional Biology, Immunology Area, Faculty of Medicine, Universidad de Oviedo, Oviedo, Asturias, Spain., Oviedo, Spain, 6Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL; Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, 7Hospital University Marqués de Valdecilla, Santander, Spain, 8Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL; Department of Pneumology, Hospital Universitario Marqués de Valdecilla; School of Medicine, Universidad de Cantabria, Santander, Spain, 9Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL, Division of Rheumatology, Hospital Universitario Marqués de Valdecilla; School of Medicine, Universidad de Cantabria, Santander, Spain. Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa

    Background/Purpose: Interstitial lung disease (ILD) is one of the most significant complications of connective tissue diseases (CTD) leading to an increase of the morbidity and…
  • Abstract Number: 0996 • ACR Convergence 2021

    The Stimulator of Interferon Genes (STING) Protects from Bone Loss Through Regulation of Tonic and Induced Type I Interferon Pathways

    Susan MacLauchlan1, Priyanka Kushwaha1, Albert Tai2, Jia (Sijia) Chen3, Catherine Manning1, Katherine Fitzgerald4, Shruti Sharma2 and Ellen Gravallese5, 1Brigham and Women's Hospital, Boston, MA, 2Tufts University School of Medicine, Boston, MA, 3Brigham and Women's Hospital, Cambridge, MA, 4University of Massachusetts Medical School, Worcester, MA, 5Brigham and Women's Hospital, Harvard Medical School, Chestnut Hill, MA

    Background/Purpose: The intracellular DNA sensing Stimulator of Interferon Genes (STING) pathway is critical for detection of viral and bacterial pathogen DNA. Hyperactivating mutations in this…
  • Abstract Number: 1546 • ACR Convergence 2021

    Immune Responses to COVID-19 Vaccines in Patients Using Immunosuppressive Medication for Inflammatory Arthritis – An Observational Study of 1500 Patients

    Ingrid Jyssum1, Anne Therese Tveter1, Fridtjof Lund-Johansen2, Ludvig Munthe2, Sella Provan1, Kristin Jørgensen3, Gunnveig Grødeland2, Grete Kro2, David Warren2, Joseph Sexton1, Tore Kvien1, Siri Mjaaland4, Espen Haavardsholm1, John Torgils Vaage2, Silje Watterdal Syversen1 and Guro Goll1, 1Diakonhjemmet Hospital, OSLO, Norway, 2Oslo University Hospital, Oslo, Norway, 3Akershus University Hospital, Lørenskog, Norway, 4Norwegian Institute of Public Health, Oslo, Norway

    Background/Purpose: To assess the strength and duration of the immunological response to COVID-19 vaccines in patients treated with immunosuppressive medication for inflammatory arthritis.Methods: Adult patients…
  • Abstract Number: 1011 • ACR Convergence 2021

    Validation of Bioinformatics Pipeline to Detect NEMO-Deleted Exon 5 Autoinflammatory Syndrome (NEMO-NDAS) and Preliminary Clinical and Immunologic Characterization

    Adriana Almeida de Jesus1, Bin Lin2, Eric Karlins3, Dana Kahle4, Andre Rastegar2, Jacob Mitchell2, Sofia Torreggiani2, Farzana Bhuyan2, Sara Alehashemi5, Kader Cetin Gedik6, Kat Uss2, Chyi-Chia Lee7, Hyesun Kuehn8, Sergio Rosenzweig8, Katherine Calvo8, Magdalena Walkiewicz9, Justin Lack10, Eric Hanson11, Amer Khojah12, Eveline Wu13, Christiaan Scott14, Timothy Ronan Leahy15, Emma MacDermott15, Orla Kileen15, Thaschawee Arkachaisri16, Zoran Gucev17, Kathryn Cook18, Vafa Mammadova19, Gulnara Nasrullayeva19, Scott Canna20, Douglas Kuhns21, Clifton Dalgard22, Timothy Moran23, Andrew Oler3 and Raphaela Goldbach-Mansky24, 1TADS/NIAID/NIH, Silver Spring, MD, 2TADS/NIAID/NIH, Bethesda, MD, 3BCBB/NIAID/NIH, Bethesda, MD, 4National Institutes of Health, Chevy Chase, MD, 5TADS/NIAID/NIH, Clarksville, MD, 6Translational Autoinflammatory Diseases Section (TADS)/NIAID/NIH, Bethesda, MD, 7NCI/NIH, Bethesda, MD, 8CC/DLM/NIH, Bethesda, MD, 9CSI/NIAID/NIH, Bethesda, MD, 10NCBR/NIAID/NIH, Bethesda, MD, 11Indiana University School of Medicine, Indianapolis, IN, 12Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, 13UNC Chapel Hill, Chapel Hill, NC, 14Paediatric Rheumatology, University of Cape Town, Cape Town, South Africa, 15Children’s Health Ireland (CHI) at Crumlin, Dublin, Ireland, 16KK Women's and Children's Hospital, SingHealth, Singapore, Singapore, 17University Children's Hospital, Medical Faculty Skopje, Skopje, Macedonia, 18Akron Childrens Hospital, Copley, OH, 19Azerbaijan Medical University, Baku, Azerbaijan, 20Children's Hospital of Philadelphia, Philadelphia, PA, 21Frederick National Laboratory for Cancer Research/NIH, Frederick, MD, 22TAGC/USUHS, Bethesda, MD, 23University of North Carolina School of Medicine, Chapel Hill, NC, 24NIH/NIAID, Potomac, MD

    Background/Purpose: Splice site variants in IKBKG that lead to exon 5 deletion cause NEMO-deleted exon 5 autoinflammatory syndrome (NEMO-NDAS). NEMO-NDAS clinically mimics the interferonopathy chronic…
  • Abstract Number: 1548 • ACR Convergence 2021

    Q Fever as a Mimicker of Rheumatologic Conditions: A Case Series from Two Tertiary Care Academic Centers in Southern California

    Manushi Aggarwal and Marven Cabling, Loma Linda University Health, Redlands, CA

    Background/Purpose: Q fever, an endemic disease in Southern California, is a zoonosis caused by Coxiella burnetii. The infection can present with multiple non-specific acute and chronic manifestations including fever, headache,…
  • Abstract Number: 1062 • ACR Convergence 2021

    Telemedicine Enriched Care Model to Optimize Care for Patients with Autoinflammatory Diseases

    Lea Oefelein1, Jens Klotsche2, Susanne Benseler3, Jasmin Kuemmerle-Deschner1 and Tatjana Welzel1, 1Pediatric Rheumatology and Autoinflammatory Reference Center, University Children's Hospital Tuebingen, Tuebingen, Germany, 2German Rheumatism Research Center, Berlin, Germany, 3University of Calgary, Calgary, AB, Canada

    Background/Purpose: Autoinflammatory diseases (AID) are severe potentially life-threatening conditions requiring personalized therapy and monitoring which only few expert centers can provide. Long travel distances impede…
  • Abstract Number: 1618 • ACR Convergence 2021

    Disease Flares in CANDLE/PRAAS with Dose Reductions of Baricitinib

    Kader Cetin Gedik1, Grace Materne2, Ana Ortega-Villa3, Gina Montealegre Sanchez4, Adam Reinhardt5, Paul Brogan6, Yackov Berkun7, Sara Murias8, Maria Robles9, Susanne Schalm10, Adriana Almeida de Jesus11 and Raphaela Goldbach-Mansky12, 1Translational Autoinflammatory Diseases Section (TADS)/NIAID/NIH, Bethesda, MD, 2Translational Autoinflammatory Diseases Section, NIAID/NIH, Bethesda, TN, 3Biostatistics Research Branch, Division of Clinical Research, NIAID/NIH, Bethesda, 4NIAID/NIH, Bethesda, MD, 5Boys Town National Research Hospital, Omaha, NE, 6UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, 7Hadassah Hebrew University Medical Center, Jerusalem, Israel, 8Hospital Infantil La Paz, Madrid, Spain, 9Eskenazi Health Center, IndianaPolis, IN, 10Rheumatologie im Zentrum, Munich, Germany, 11TADS/NIAID/NIH, Silver Spring, MD, 12NIH/NIAID, Potomac, MD

    Background/Purpose: Patients with chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperatures /proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS) respond to treatment with baricitinib but require higher exposure…
  • Abstract Number: 0043 • ACR Convergence 2021

    Takinib Inhibits IL-1β-Induced Activation of Signal Transducer and Activator of Transcription 3 (STAT3) in Human Rheumatoid Arthritis Synovial Fibroblasts

    Anil singh1, Paul Panipinto1, Ruby Siegel1, Farheen Shaikh1, Mukesh Chourasia2 and Salah-uddin Ahmed1, 1Washington State University, Spokane, WA, 2Amity University, Noida, India

    Background/Purpose: Interleukin-1 (IL-1) is a crucial mediator of inflammatory cartilage and bone destruction in rheumatoid arthritis (RA). IL-1β signaling relies on the activation of TGF-beta…
  • Abstract Number: 1083 • ACR Convergence 2021

    Outcomes of COVID-19 Illness in Systemic Autoinflammatory Diseases and Changes in Flares During the COVID-19 Pandemic: An International Survey

    Samira Nazzar Romero1, Lakshmi Moorthy2, Jennifer Tousseau3, Sivia Lapidus4, Mariana Correia Marques5, Leanne Mansfield6, Marinka Twilt7, Grant Schulert8, Maria Gutierrez9, Saskya Angevare10, Fatma Dedeoglu11 and Karen Durrant12, 1University of California Los Angeles, Los Angeles, CA, 2Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, 3Autoinflamamtory Alliance, Lincoln, CA, 4The Joseph M. Sanzari Children's Hospital, Hackensack Meridian Health, Montclair, NJ, 5UPMC Children`s Hospital of Pittsburgh, Pittsburgh, PA, 6Hospital for Special Surgery, New York, NY, 7Alberta Children's Hospital, Calgary, AB, Canada, 8Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 9John's Hopkins, Baltimore, MD, 10Autoinflammatory Alliance / Kaisz / ENCA as patient organizations, Amersfoort, Netherlands, 11Boston Children's Hospital, Boston, MA, 12Autoinflammatory Alliance, San Francisco, CA

    Background/Purpose: The exaggerated inflammatory responses to the SARS-CoV-2 virus and the paucity of data on COVID-19 infection risk in systemic autoinflammatory disease (SAID) patients posed…
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Accepted abstracts are made available to the public online in advance of the meeting and are published in a special online supplement of Arthritis & Rheumatology. Information contained in those abstracts may not be released until the abstracts appear online. Academic institutions, private organizations and companies with products whose value may be influenced by information contained in an abstract may issue a press release to coincide with the availability of an ACR abstract on the ACR website. However, the ACR continues to require that information that goes beyond that contained in the abstract (e.g., discussion of the abstract done as part a scientific presentation or presentation of additional new information that will be available at the time of the meeting) is under embargo until Saturday, November 11, 2023.

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