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  • Abstract Number: 1063 • ACR Convergence 2022

    Assessment of Calcinosis in Portuguese Patients with Systemic Sclerosis – a Multicenter Study

    Beatriz Samões1, Diogo Fonseca1, Tiago Beirão1, Flavio Costa1, Romana Vieira1, Georgina Terroso2, Raquel Ferreira2, Rafaela teixeira3, André Saraiva4, Maria João Henriques5, Ana Catarina Duarte6, Ana Cristina Cordeiro6, Paulo Vilas-Boas7, Inês Genrinho3, Ana Bento da Silva8, Laura Gago8, Catarina Resende9, Patricia Martins9, Nathalie Madeira10, Sara Paiva Dinis10, Maura Couto3, Inês Santos3, Filipe Araújo11, Ana Filipa Mourão11, Miguel Guerra12, Margarida Oliveira12, Alexandra Daniel13, Marília Rodrigues13, Catarina Soares14, Hugo Parente15, Carolina Furtado16, Tomás Fontes16 and Joana Abelha-Aleixo1, 1Rheumatology Department, Centro Hospitalar de Vila Nova de Gaia / Espinho, Vila Nova de Gaia, Portugal, 2Rheumatology department, Centro Hospitalar Universitário de São João, Porto, Portugal, 3Rheumatology department, Centro Hospitalar Tondela-Viseu, Viseu, Portugal, 4Rheumatology department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal, 5Rheumatology department, Centro Hospitalar e Universitário de Coimbra, Lisboa, Portugal, 6Rheumatology department, Hospital Garcia de Orta, Almada, Portugal, 7Rheumatology Department, Centro Hospitalar Baixo Vouga, Aveiro, Portugal, 8Rheumatology department, Hospital de Egas Moniz, Centro Hospitalar de Lisboa Ocidental, Lisboa, Portugal, 9Rheumatology department, Centro Hospitalar e Universitário de Lisboa Norte, Centro Académico de Medicina de Lisboa, Lisboa, Portugal, 10Rheumatology department, Unidade Local de Saúde da Guarda - Hospital Sousa Martins, Guarda, Portugal, 11Rheumatology and Osteoporosis Unit, Hospital de Sant´Ana, SCML, Lisboa, Portugal, 12Rheumatology Department, Centro Hospitalar Universitário da Cova da Beira, Covilhã, Portugal, 13Rheumatology department, Hospital Distrital de Leiria, Leiria, Portugal, 14Rheumatology department, Unidade Local de Saúde do Alto Minho, Ponte de Lima, Viana do Castelo, Portugal, 15Rheumatology department, Unidade Local de Saúde do Alto Minho, Ponte de Lima, Portugal, 16Rheumatology department, Hospital do Divino Espírito Santo, Ponta Delgada, Portugal

    Background/Purpose: Calcinosis is a challenging problem among Systemic Sclerosis (SSc) patients with a reported prevalence of 18-49%. We aim to define the prevalence of clinical…
  • Abstract Number: 1075 • ACR Convergence 2022

    Efficacy of Mepolizumab in Patients with Eosinophilic Granulomatosis with Polyangiitis and a Vasculitic Phenotype

    Benjamin Terrier1, David Jayne2, Bernhard Hellmich3, Jane Bentley4, Jonathan Steinfeld5, Steven Yancey6, Namhee Kwon7, Praveen Akuthota8, Paneez Khoury9, Lee Baylis10 and Michael Wechsler11, 1National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, 2University of Cambridge, Cambridge, United Kingdom, 3Klinik für Innere Medizin, Rheumatologie & Immunologie, Medius Kliniken, Universität Tübingen, Plochingen, Germany, 4Clinical Statistics, GlaxoSmithKline, Brentford, United Kingdom, 5Clinical Sciences, Respiratory, GSK (affiliation at time of research), Philadelphia, PA, 6Respiratory Therapeutic Area, GSK (affiliation at the time of research), Research Triangle Park, NC, 7Clinical Sciences, Respiratory, GSK, Brentford, United Kingdom, 8Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, University of California, San Diego, CA, 9Human Eosinophil Section, Laboratory of Parasitic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, 10Global Medical Affairs, GlaxoSmithKline, Durham, NC, 11Department of Medicine, National Jewish Health, Denver, CO

    Background/Purpose: Patients with eosinophilic granulomatosis with polyangiitis (EGPA) can have vasculitic or eosinophilic phenotypes. The MIRRA study demonstrated that patients with EGPA spent more time…
  • Abstract Number: 1074 • ACR Convergence 2022

    Safety and Efficacy of Dupilumab in Patients with Relapsing and/or Refractory Eosinophilic Granulomatosis with Polyangiitis

    Berengere Molina1, Maria Letizia Urban2, Roberto Padoan3, Pavel Novikov4, Camille Taillé5, Marco Caminati6, Christine Christides7, nicolas schleinitz8, Laura Moi9, Vincent Cottin10, bertrand godeau11, Laurence Bouillet12, Paolo Fraticelli13, Ann Knight14, Jan Walter Schroeder15, Sylvain Marchand-Adam16, Helder Gil17, Elena Gelain18, Giacomo Emmi2 and Benjamin Terrier19, 1Department of Internal Medicine, Cochin Hospital, Paris, France, 2Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, 3University of Padova, Padova, Italy, 4Tareev’s Clinic, Sechenov First Moscow State Medical University, Moscow, Russia, 5Pulmonology department, Bichat Hospital, Paris, Ile-de-France, France, 6Verona University Hospital, University of Verona, Verona, Italy, 7Avignon hospital, Avignon, France, 8Aix Marseille university, AP-HM, Marseille, France, 9Centre hospitalier universitaire vaudois, Lausanne, Lausanne, Switzerland, 10Coordinating Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, University of Lyon, INRAE, Lyon, France, 11Department of Internal Medicine, National Referral Center for Adult's Immune Cytopenias, Mondor Hospital, Créteil, France, 12Grenoble University Hospital, Grenoble, France, 13Department of Internal Medicine, Ospedali Riuniti di Ancona, Ancona, Italy, 14Akademiska sjukhuset, Uppsala, Sweden, 15ASST GOM Niguarda, Milano, Italy, 16Tours University Hospital, Tours, France, 17Besancon University Hospital, Besançon, France, 18Meyer Children’s University Hospital of Firenze, Florence, Italy, 19National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France

    Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is an ANCA-associated vasculitides characterized by asthma, blood and tissue eosinophilia and systemic manifestations. Glucocorticosteroids (GCs)-dependent asthma and/or disabling…
  • Abstract Number: 1068 • ACR Convergence 2022

    Anti-vinculin Antibodies in Systemic Sclerosis: Associations with Slow Gastric Transit and Extra-Intestinal Clinical Phenotype

    Maria Herran1, Brit Adler2, Jamie Perin3, Walter Morales4, Mark Pimentel5 and Zsuzsanna McMahan6, 1School of Medicine and Health Sciences, Universidad del Rosario, Bogotá, Colombia, 2Johns Hopkins University, Division of Rheumatology, Baltimore, MD, 3John Hopkins University, Baltimore, MD, 4Medically Associated Science and Technology (MAST) Program, Cedars-Sinai, Los Angeles, CA, 5Karsh Division of Gastroenterology and Hepatology, Cedars-Sinai, Los Angeles, CA, 6Johns Hopkins Rheumatology, Baltimore, MD

    Background/Purpose: The gastrointestinal tract is commonly affected in systemic sclerosis (SSc). Prior studies have reported an association between higher levels of anti-vinculin antibodies and gastrointestinal…
  • Abstract Number: 1067 • ACR Convergence 2022

    Analysis of the Transcriptomic Profiles of Rheumatic Skin Diseases Reveals Disease-specific Endotypes

    Sneha Shrotri1, Kathryn Kingsmore Allison1, Prathyusha Bachali2, Amrie Grammer3 and Peter Lipsky1, 1AMPEL BioSolutions, Charlottesville, VA, 2AMPEL BioSolutions, Redmond, WA, 3AMPEL LLC, Charlottesville, VA

    Background/Purpose: Patients with rheumatic skin diseases such as cutaneous lupus erythematosus (CLE) and systemic sclerosis (SSc) can be classified from individuals with healthy skin using…
  • Abstract Number: 1079 • ACR Convergence 2022

    Intravenous Immunoglobulin in Antineutrophil Cytoplasmic Antibody-associated Vasculitis: Study of 28 Cases from a Single Univeristary Hospital

    Fabricio Benavides Villanueva1, Cristina Corrales1, Vanesa Calvo Río2, Javier Loricera3, Nuria Aviles4, Juan M Blanco Madrigal5, Santos Castañeda6, Miguel Angel Gonzalez Gay7 and Ricardo Blanco3, 1Hospital Universitario Marqués de Valdecilla, Santander, Spain, 2Valdecilla Hospital, Santander, Spain, 3Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, 4Hospital Universitario Perpetuo Socorro, Albacete, Spain, 5Basurto University Hospital, Bilbao, Spain, 6Division of Rheumatology, Hospital Universitario de La Princesa, IIS-Princesa, Madrid, Spain, 7Hospital Universitario Marques de Valdecilla, Lugo, Spain

    Background/Purpose: Anti neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) includes granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) or microscopic polyarteritis (MPA). Standard treatment is…
  • Abstract Number: 1080 • ACR Convergence 2022

    Performance of Risk Prediction Models for Relapse and Severe Infection at the End of 18-month Rituximab Maintenance Therapy in ANCA-associated Vasculitides: Data from the MAINRITSAN Trials

    Florence Delestre1, Pierre Charles2, Loïc Guillevin3, Raphaël Porcher4 and Benjamin Terrier3, 1cochin Hospital, Paris, France, 2Institut Mutualiste Montsouris, Paris, France, 3National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, 4Université Paris Cité, Hôtel-Dieu, Paris, France

    Background/Purpose: Long-term follow-up analysis of the three MAINRITSAN trials confirmed the efficacy of rituximab (RTX) in preventing relapses in ANCA-associated vasculitis (AAV), especially RTX administration…
  • Abstract Number: 1070 • ACR Convergence 2022

    Discordance of Patient, Physician and Nurse Practitioner Assessment of Disease Burden in Systemic Sclerosis

    Maylen Nordgård Carstens1, Håvard Fretheim1, Imon Barua1, Henriette Didriksen2, Torhild Garen1, Øyvind Midtvedt1, Øyvind Molberg3 and Anna-Maria Hoffmann-Vold1, 1Oslo University Hospital, Oslo, Norway, 2Oslo University Hospital, Moss, Norway, 3Department of Rheumatology, Oslo University Hospital, Oslo, Norway, Oslo, Norway

    Background/Purpose: Assessment of disease burden is crucial in the management of systemic sclerosis (SSc). The patient global assessment (PatGA) is a patient self-reported measure widely…
  • Abstract Number: 1078 • ACR Convergence 2022

    Glucocorticoid Use and Related Adverse Events in ADVOCATE Trial of Avacopan in ANCA-Associated Vasculitis

    Emil De Gomma1, Peter Merkel2, Michael George3, Rennie Rhee3, Andreas Kronbichler4, David Jayne5, Huibin Yue1 and Pirow Bekker1, 1ChemoCentryx, San Juan Capistrano, CA, 2U of Pennsylvania, Philadelphia, PA, 3University of Pennsylvania, Philadelphia, PA, 4Cambridge University, Sale Creek, TN, 5University of Cambridge, Cambridge, United Kingdom

    Background/Purpose: Although the 2021 ACR/VF guidelines for ANCA-associated vasculitis (AAV) recommend reduced-dose glucocorticoids (GCs) over standard-dose GCs (1), this approach still leads to substantial toxicity,…
  • Abstract Number: 1091 • ACR Convergence 2022

    IgG Antibody Subclass Glycan Modifications in Patients with ANCA-Associated Vasculitis in Active vs Remission States – a Proof-of-Concept Study

    Zachary Wallace1, Tina Motazedi1, Michelle Conroy1, Claire Cook1, John Stone2, Hyon Choi3 and Robert Anthony1, 1Massachusetts General Hospital, Boston, MA, 2Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA, 3MASSACHUSETTS GENERAL HOSPITAL, Lexington, MA

    Background/Purpose: The Fc portion of IgG tends to be hypogalactosylated among patients with ANCA-associated vasculitis (AAV) compared with healthy controls1,2,3. Glycan modifications may affect antibody…
  • Abstract Number: 1085 • ACR Convergence 2022

    Safety of Plasmapheresis as an Adjuvant Therapy in Severe Pediatric Anti-neutrophil Cytoplasmic Antibody Associated Vasculitis: A Single Center Cohort

    Sharanya Joginpalli1, Alvaro Orjuela2, Marietta De Guzman3 and Emily Frierson2, 1Baylor College of Medicine- Texas Children's Hospital, Houston, TX, 2Baylor College of Medicine, Texas Children's Hospital, Houston, TX, 3Baylor College of Medicine/ Texas Children's Hospital, Houston, TX

    Background/Purpose: Therapeutic plasma exchange (TPE) is used in anti-neutrophil cytoplasmic antibody associated vasculitis (AAV) as adjunct treatment for severe disease. There is paucity of data…
  • Abstract Number: 1087 • ACR Convergence 2022

    Identification of Novel Proteomic Biomarkers of Disease Activity in ANCA-Associated Vasculitis Using a High Throughput Approach

    Zachary Wallace1, Amit Joshi1, Xiaoqing Fu1, Claire Cook1, yuqing zhang2 and Hyon Choi3, 1Massachusetts General Hospital, Boston, MA, 2Massachusetts General Hospital, Quincy, MA, 3MASSACHUSETTS GENERAL HOSPITAL, Lexington, MA

    Background/Purpose: ANCA-associated vasculitis (AAV) is associated with excess morbidity and mortality. Identifying novel biomarkers may reveal new therapeutic targets and clinically useful biomarkers of disease…
  • Abstract Number: 1083 • ACR Convergence 2022

    Treatment of ANCA-associated Vasculitis with Extreme Impairment of Renal Function: A Case-Control Study Comparing an Intensive B Cell Depletion Regimen with the Standard of Care Using Oral Cyclophosphamide

    Dario Roccatello1, Savino Sciascia2, Stefano Murgia1, Giacomo Quattrocchio1, Michela Ferro1, Emanuele De Simone1, Carla Naretto1, Antonella Barreca1, Daniela Rossi1, Vittorio Modena1 and Roberta Fenoglio1, 1University of Turin, Turin, Italy, 2University of Turin, Torino, Italy

    Background/Purpose: Rituximab (RTX), an anti-CD20 monoclonal antibody, has shown to be an effective induction treatment for small-vessel vasculitis associated with antineutrophil cytoplasm antibodies (AAV) in…
  • Abstract Number: 1093 • ACR Convergence 2022

    ANCA Associated Vasculitis: Online CME Improves Rheumatologists’ Knowledge and Confidence

    Natalie Guevara1, James Martorano1 and Karen Badal2, 1Medscape Education, New York, NY, 2Medscape, New York, NY

    Background/Purpose: Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) is a rare condition that affects several organ systems, has a high rate of mortality, and involves clinicians…
  • Abstract Number: 1037 • ACR Convergence 2022

    Performance of BASDAI vs. ASDAS in Evaluating Axial Involvement in Patients with PsA Treated with Guselkumab: Pooled Analysis of Two Phase 3 Studies

    Xenofon Baraliakos1, Dafna Gladman2, Soumya Chakravarty3, Cinty Gong4, May Shawi5, Emmanouil Rampakakis6, Kishimoto Mitsumasa7, Enrique R Soriano8 and Philip J Mease9, 1Rheumazentrum Ruhrgebiet Herne, Herne, Germany, 2Toronto Western Hospital, Schroeder Arthritis Institute, Toronto, ON, Canada, 3Janssen Scientific Affairs, LLC; Drexel University College of Medicine, Villanova, PA, 4Janssen Scientific Affairs, LLC, Horsham, PA, 5Immunology Global Medical Affairs, Janssen Pharmaceutical Companies of Johnson & Johnson, Horsham, PA, 6McGill University, Department of Pediatrics and JSS Medical Research, Montréal, QC, Canada, 7Department of Nephrology and Rheumatology, Kyorin University School of Medicine, Tokyo, Japan, 8Rheumatology Unit and University Institute, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, 9Swedish Medical Center/Providence St. Joseph Health, Seattle, WA

    Background/Purpose: Although the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) is used to assess the activity of axial disease in patients (pts) with PsA, only…
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