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  • Abstract Number: 0459 • ACR Convergence 2020

    Autologous Hematopoietic Stem Cell Transplantation for Behçet’s Disease: A Retrospective Survey of Patients Treated in Europe, on the Behalf of the Autoimmune Diseases Working Party of the European Society for Blood and Bone Marrow Transplantation

    Mathieu Puyade1, Patel Amit2, Yeong Jer Lim3, Norbert Blank4, Manuela Badoglio5, Francesca Gualandi6, David D Ma7, Raffaella Greco8, Natalia Maximova9, Tobias Alexander10 and John A Snowden11, 1Service de Médecine Interne et Maladies Infectieuses ,CIC-1402 Centre Hospitalier Universitaire de Poitiers, Poitiers, France, 2The Christie NHS Foundation Trust, Manchester, United Kingdom, 3University of Liverpool, Liverpool, United Kingdom, 4Department of Hematology, Oncology and Rheumatology, Internal Medicine V, University Hospital of Heidelberg, Eppelheim, Germany, 5EBMT ADWP Office, Paris, France, 6U.O. EMATOLOGIA Centro Trapianti Midollo - OSPEDALE POLICLINICO SAN MARTINO, GENOVA, Italy, 7Department of Haematology and BM Transplantation, St Vincent’s Hospital Sydney and St Vincent’s Clinical School, Faculty of Medicine, Sidney, New South Wales, Australia, 8Ematologia Trapianto Midollo San Raffaele, Milano, Italy, 9Institute for Maternal and Child Health – IRCCS Burlo Garofolo, Trieste, Italy, 10Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Department of Rheumatology and Clinical Immunology, Berlin, Deutsches Rheuma-Forschungszentrum, Berlin, Germany, 11Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom

    Background/Purpose: Behçet’s Disease (BD) is a rare autoimmune disease mostly presenting with recurrent oral and genital aphtosis, ulcers, and uveitis. Rare patients are refractory to…
  • Abstract Number: 0460 • ACR Convergence 2020

    Lymphocyte Clonal Expansion Distinguishes Different Forms of Uveitis

    Michael Paley1, Lynn Hassman1, Philip Ruzycki1, Ekaterina Esaulova1, Grace Paley1, Jennifer Laurent1, Luke Springer1, Lacey Feigl1, Maxim Artyomov1 and Wayne Yokoyama2, 1Washington University, Saint Louis, 2Washington University, St Louis, MO

    Background/Purpose: Anterior uveitis is a form of ocular inflammation associated with rheumatologic disease and can be categorized as granulomatous or non-granulomatous. Whether different ocular manifestations…
  • 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: 0464 • ACR Convergence 2020

    Assessment of the COVID-19 Pandemic from the Perspective of People with Rheumatic Musculoskeletal Diseases in Europe. Preliminary Results from the REUMAVID Study

    Marco Garrido-Cumbrera1, Helena Marzo-Ortega2, José Correa-Fernández3, Sergio Sanz-Gomez4, Laura Christen5 and Victoria Navarro-Compán6, 1Health & Territory Research (HTR), Universidad de Sevilla / Spanish Federation of Spondyloarthritis Associations, Sevilla, Spain, 2The University of Leeds, Leeds Institute for Rheumatic and Musculoskeletal Medicine, NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, Leeds, UK, Leeds, United Kingdom, 3Health & Territory Research (HTR), Universidad de Sevilla, Sevilla, Spain, 4Health & Territory Research, Seville, Spain, 5Novartis Pharma AG, Basel, Switzerland, 6Hospital Universitario La Paz IdiPaz, Madrid, Pais Vasco, Spain

    Background/Purpose: The COVID-19 pandemic is an unprecedented public health crisis affecting people worldwide, including those with rheumatic and musculoskeletal diseases (RMDS).  REUMAVID aims to assess…
  • Abstract Number: 0465 • ACR Convergence 2020

    The COVID-19 Pandemic and Its Effect on Patients with Chronic Rheumatologic Disease Regarding the Value of Vaccination Recommendations

    Rebecca Overbury1, Gregory Stoddard2 and Tracy Frech3, 1University of Utah and Primary Children's Hospital, Salt Lake City, UT, 2University of Utah, Salt Lake City, UT, 3University of Utah and Salt Lake Veterans Affair Medical Center, Salt Lake City, UT

    Background/Purpose: Vaccinations are an important component of care in rheumatology as many patients are immunocompromised by treatment therapies. Patients are sometimes reticent to receive recommended…
  • Abstract Number: 0466 • ACR Convergence 2020

    Dosage Modification of Immunomodulatory Medications by Rheumatology Patients in New York City During the Peak of the COVID-19 Pandemic

    Marianna Frey1, Gregory Vitone2, Candace Feldman3, Lindsay Lally1, Anne Bass4, Jane Salmon1, Mary Crow1, Michael Lockshin5, Vivian Bykerk1, Lisa Mandl1 and Medha Barbhaiya5, 1Hospital for Special Surgery, New York, NY, 2Hospital for Special Surgery, New York, 3Brigham and Women's Hospital, Boston, 4Hospital for Special Surgery/Weill Cornell Medicine, New York, NY, 5Hospital for Special Surgery, Barbara Volcker Center for Women and Rheumatic Diseases, New York, NY

    Background/Purpose: Due to concerns about underlying immune dysregulation and immunosuppression, patients with systemic rheumatic diseases living in COVID-19 “hot spots” may have modified their immunomodulatory…
  • Abstract Number: 0467 • ACR Convergence 2020

    Incidence of COVID-19 in Patients Treated with Infliximab Compared to Patients Treated with Rituximab

    Cathy Melong Pianta1, Kim Lauper1, Delphine Courvoisier1, Tim Cunningham2, Daniéle Allali3 and Axel Finckh4, 1Division of rheumatology, Geneva University Hospitals, Geneva, Switzerland, 2Private practice, Geneva, Geneva, Switzerland, 3Division of immunology, Geneva University Hospitals, Geneva, 4Division of Rheumatology, University Hospitals of Geneva, Geneva, Switzerland

    Background/Purpose: The prevalence of anti-SARS-CoV-2 IgG infection was estimated at 9.7% in the Geneva population end of April 2020. (1) Immunosuppressed patients may be at…
  • Abstract Number: 0468 • ACR Convergence 2020

    Concerns and Health-Related Behaviors During the COVID-19 Pandemic in Patients with or Without Autoimmune Rheumatic Disease in a Large Physician Network

    Michael George1, Shilpa Venkatachalam2, Shubhasree Banerjee1, Joshua Baker1, Peter Merkel1, David Curtis3, Kelly Gavigan4, Maria (Maio) Danila5, Daniel Kirby6, George Munoz7, William Sunshine8, William Nowell9 and Jeffrey R Curtis10, 1University of Pennsylvania, Philadelphia, PA, 2Global Healthy Living Foundation, Upper Nyack, 3Global Healthy Living Foundation, New York City, 4Global Healthy Living Foundation, Upper Nyack, NY, 5University of Alabama at Birmingham (UAB), Birmingham, AL, 6AARA, Charlotte, NC, 7AARA, Aventura, FL, 8AARA, Boca Raton, FL, 9Global Healthy Living Foundation, New York City, NY, 10Division of Clinical Immunology and Rheumatology, University of Alabama at Birmingham, Birmingham, AL

    Background/Purpose: Patients with autoimmune rheumatic diseases (ARD) may be particularly concerned about COVID-19. We aimed to compare concerns and health-related behaviors of patients with common…
  • 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: 0470 • ACR Convergence 2020

    Th1 Polarization Defines the T Cell Compartment in the Joints of Oligoarticular Juvenile Idiopathic Arthritis Patients

    Amelie Jule1, Kacie Hoyt1, Kevin Wei2, Siobhan Case3, Margaret Chang1, Ezra Cohen1, Fatma Dedeoglu1, Melissa Hazen1, Jonathan Hausmann4, Olha Halyabar5, Erin Janssen5, Pui Lee6, Jeffrey Lo1, Mindy Lo1, Esra Meidan7, Jordan Roberts1, Mary Beth Son1, Robert Sundel5, Talal Chatila1, Peter Nigrovic8 and Lauren Henderson9, 1Boston Children's Hospital, Boston, MA, 2Brigham and Women's Hospital and Harvard Medical School, Boston, MA, 3Brigham and Women's Hospital, Boston, MA, 4Boston Children's Hospital / Beth Israel Deaconess Medical Center, Cambridge, MA, 5Children's Hospital/Boston Medical Center, Boston, MA, 61.Boston Children's Hospital;2.Brigham and Women's Hospital;3.Harvard Medical School, Newton, MA, 7Boston Children's Hospital, Somerville, MA, 8Division of Rheumatology, Inflammation and Immunity, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA; Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA, Boston, 9Boston Children's Hospital, Watertown, MA

    Background/Purpose: Oligoarticular juvenile idiopathic arthritis (oligo JIA) is defined by limited joint involvement at disease onset. Some children achieve long-term remission while others continue to…
  • Abstract Number: 0471 • ACR Convergence 2020

    Splice Site Variants in IKBKG, Encoding NEMO, Detected by a Customized Analysis of Next-Generation Sequencing Data Cause an Early-onset Autoinflammatory Syndrome of Panniculitis and Cytopenias in Male and Female Patients

    Adriana de Jesus1, Sofia Torreggiani2, Bin Lin2, Jacob Mitchell2, Eric Karlins3, Andrew Oler3, Sara Alehashemi4, Dana Kahle5, Katelin R. Honer2, Gema Souto Adeva2, Eric Hanson6, Gina Montealegre Sanchez7, Amer Khojah8, Timothy Moran9, Eveline Wu9, Chris Scott10, Timothy Ronan Leahy11, Emma Jane MacDermott11, Orla Killeen12, Thaschawee Arkachaisri13, Zoran Gucev14, Kathryn Phillippi15, Vafa Mammadova16, Gulnara Nasrullayeva16 and Raphaela Goldbach-Mansky17, 1Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Silver Spring, MD, 2Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, 3Bioinformatics and Computational Biosciences Branch/NIAID/NIH, Bethesda, MD, 4Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Clarksville, MD, 5Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, 6Indiana University School of Medicine, Indianapolis, IN, 7NIAID/NIH, Rockville, MD, 8Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, 9UNC Chapel Hill, Chapel Hill, NC, 10University of Cape Town, Cape Town, South Africa, 11Our Lady's Children's Hospital, Dublin, Ireland, 12National Centre for Paediatric Rheumatology, CHI at Crumlin, Dublin, Ireland, 13Duke-NUS Medical School, Singapore, Singapore, 14University Children's Hospital, Medical Faculty Skopje, Skopje, Macedonia, 15Akron Children’s Hospital, Akron, OH, 16Azerbaijan Medical University, Baku, Azerbaijan, 17Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD

    Background/Purpose: The Inhibitor of Kappa-B Kinase Regulatory Subunit Gamma (IKBKG) is located on the X chromosome and encodes the NF-κB essential modulator (NEMO). Loss-of-function mutations…
  • Abstract Number: 0472 • ACR Convergence 2020

    ApoB:ApoA1 Ratio Could Predict Atherosclerotic Risk in Juvenile-SLE Patients Associated with Altered Interferon Signalling in CD8+ T-cells

    George Robinson1, Kirsty Waddington2, Leda Coelewij2, Junjie Peng2, Meena Naja2, Chris Wincup2, Anna Radziszewska2, Hannah Peckham2, David Isenberg2, Yiannis Ioannou2, Ines Pineda Torra2, Coziana Ciurtin2 and Elizabeth Jury2, 1University College London, Hertford, United Kingdom, 2University College London, London, United Kingdom

    Background/Purpose: Systemic lupus erythematosus (SLE) is an autoimmune rheumatic disease characterised by immune-dysregulation, chronic inflammation, type-I interferon (IFN) signatures and increased cardiovascular disease (CVD) risk…
  • Abstract Number: 0473 • ACR Convergence 2020

    Characterization of DOCK8 as a Novel Gene Associated with Cytokine Storm Syndrome

    Mingce Zhang1, Remy Cron2, Devin Absher3, Prescott Atkinson1, Winn Chatham1 and Randy Cron1, 1University of Alabama at Birmingham, Birmingham, AL, 2University of Alabama at Birmingham, Birmingham, 3HudsonAlpha Institute for Biotechnology, Huntsville, AL

    Background/Purpose: Cytokine storm syndrome (CSS), also known as macrophage activation syndrome (MAS) or secondary hemophagocytic lymphohistiocytosis (HLH), is a life threatening condition that commonly presents…
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All abstracts accepted to ACR Convergence are under media embargo once the ACR has notified presenters of their abstract’s acceptance. They may be presented at other meetings or published as manuscripts after this time but should not be discussed in non-scholarly venues or outlets. The following embargo policies are strictly enforced by the ACR.

Accepted abstracts are made available to the public online in advance of the meeting and are published in a special online supplement of our scientific journal, Arthritis & Rheumatology. Information contained in those abstracts may not be released until the abstracts appear online. In an exception to the media embargo, 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 of editorial news coverage) is under media embargo until 10:00 AM CT on October 25. Journalists with access to embargoed information cannot release articles or editorial news coverage before this time. Editorial news coverage is considered original articles/videos developed by employed journalists to report facts, commentary, and subject matter expert quotes in a narrative form using a variety of sources (e.g., research, announcements, press releases, events, etc.).

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