ACR Meeting Abstracts

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  • Abstract Number: 0686 • ACR Convergence 2023

    Safety and Efficacy of Avacopan in Patients 65 Years and Older with ANCA-Associated Vasculitis

    David Jayne1, Duvuru Geetha2, Christian Pagnoux3, Sebastian Sattui4 and Peter Merkel5, 1University of Cambridge, Cambridge, United Kingdom, 2Johns Hopkins University, Baltimore, MD, 3Mount Sinai Hospital, Toronto, ON, Canada, 4University of Pittsburgh, Pittsburgh, PA, 5University of Pennsylvania, Philadelphia, PA

    Background/Purpose: Older adults are at increased risk of glucocorticoid (GC)-related toxicity; minimization of GCs is a major focus for treatment of patients with ANCA-associated vasculitis…
  • Abstract Number: 0729 • ACR Convergence 2023

    ROCK1 Orchestrates B-cell Differentiation Under Stress

    Juan Rivera-Correa1, Sanjay Gupta1, Edd Ricker2, Danny Flores-Castro1, Daniel Jenkins1, Stephen Vulcano1, Tania Pannellini1, Matthew Miele3, Zhuoning Li3, Nahuel Zamponi4, Young-Bum Kim5, Evgenia Giannopoulou1, Leandro Cerchietti4 and Alessandra Pernis1, 1Hospital for Special Surgery, New York, NY, 2Columbia University, New York, NY, 3Memorial Sloan Kettering Cancer Center, New York, NY, 4Weill Cornell Medicine, New York, NY, 5Beth Israel Deaconess Medical Center, Boston, MA

    Background/Purpose: The mechanisms utilized by B cells to execute their differentiation programs when exposed to damaging and stressful environments, which often accompany severe infections and…
  • Abstract Number: 0737 • ACR Convergence 2023

    Unraveling Pathophysiology and Hematopoiesis of VEXAS Syndrome by Multi-omics Analysis and Targeted Gene Editing

    Corrado Campochiaro1, Raffaella Molteni2, Martina Fiumara3, Alessandro Tomelleri4, Elisa Diral5, Davide Stefanoni2, Angelica Varesi3, Alessandra Weber3, Roberta Alfieri6, Luisa Albano3, Maddalena Panigada2, Eleonora Cantoni2, Daniele Canarutto3, Luca Basso-Ricci3, Pamela Quaranta3, Angelo D’Alessandro7, marco Matucci Cerinic8, Raffaella Di Micco3, Serena Scala3, Alessandro Aiuti3, Fabio Ciceri5, Ivan Merelli3, Lorenzo Dagna9, Simone Cenci2, Luigi Naldini3, Samuele Ferrari3 and Giulio Cavalli2, 1IRCCS San Raffaele Hospital, Unit of Immunology, Rheumatology, Allergy and Rare Disease. Vita-Salute San Raffaele University, Milan, Italy, 2Division of Genetics and Cell Biology, IRCCS San Raffaele Scientific Institute, Milan, Italy, 3San Raffaele Telethon Institute for Gene Therapy, IRCCS San Raffaele Scientific Institute, Milan, Italy, 4Unit of Immunology, Rheumatology, Allergy and Rare Diseases, San Raffaele Scientific Institute, Milano, Italy, 5Unit of Haematology and Bone Marrow Transplantation, IRCCS San Raffaele Hospital, Milan, Italy, 6Institute for Biomedical Technologies, National Research Council, Segrate, Italy, 7Department of Biochemistry and Molecular Genetics, University of Colorado Denver - Anschutz Medical Campus, Aurora, CO, 8Unit of Immunology, Rheumatology, Allergy and Rare diseases, IRCCS San Raffaele Hospital, Milan, Milan, Italy, 9Department of Internal Medicine, IRCCS San Raffaele Hospital and Vita-Salute San Raffaele University, Milan, Italy

    Background/Purpose: VEXAS syndrome is an adult-onset, X-linked, life-threatening, autoinflammatory and hematological disease caused by somatic mutation in UBA1 gene. To understand its pathophysiology, we aimed…
  • Abstract Number: 0724 • ACR Convergence 2023

    Synovial Macrophage Subsets Defined by ScRNAseq Demonstrate Sexually Dimorphic Gene Expression in RA and a Mouse Inflammatory Arthritis Model

    Richard Bell, Ewurama Cann, Chao Yang, Accelerating Medicine Partnership Rheumatoid Arthritis, Amit Lakhanpal, Laura Donlin and Lionel Ivashkiv, Hospital for Special Surgery, New York, NY

    Background/Purpose: Macrophages (MФ) play a key pathogenic role in Rheumatoid Arthritis (RA), a disease that exhibits female sex bias. Recent work using scRNAseq has defined…
  • Abstract Number: 0719 • ACR Convergence 2023

    Polymyalgia Rheumatica Following SarsCOV-2 Vaccination: A Single Center Cohort Study

    Lindsay Lally1, Aliza Bloostein1, Deanna Jannat-Khan1 and Robert Spiera2, 1Hospital for Special Surgery, New York, NY, 2Hosptial for Special Surgery, New York, NY

    Background/Purpose: Polymyalgia rheumatica (PMR) incidence peaks in individuals between 70 and 80 years of age; this same group is also at increased risk of complications…
  • Abstract Number: 0727 • ACR Convergence 2023

    A Novel Active Pemphigus Vulgaris Mouse Model Induced by Immunization with TLR Ligand Adjuvanted Recombinant Desmoglein 3 Protein Vaccine

    Changxing Gao, Zijun Wang and Qianjin Lu, Hospital for Skin Diseases, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing, China; Key Laboratory of Basic and Translational Research on Immune-Mediated Skin Diseases, Chinese Academy of Medical Sciences, Nanjing, China. 3. Jiangsu Key Laboratory of Molecular Biology for Skin Diseases and STIs, Nanjing, China

    Background/Purpose: Pemphigus vulgaris (PV) is an autoimmune bullous skin disease mediated by desmoglein-3 (Dsg3) specific auto-antibody present in skin and mucosae. The binding of pathogenic…
  • Abstract Number: 0690 • ACR Convergence 2023

    Treating Patients with ANCA-Associated Vasculitis and Very Severe Renal Injury with an Intensified B Cell Depletion Therapy: Comparison with a Control Cohort Receiving a Conventional Therapy

    Dario Roccatello1, Savino Sciascia2, SILVIA GRAZIETTA FODDAI3, gIACOMO QUATTROCCHIO4, MASSIMO RADIN3, IRENE CECCHI3, ALICE BARINOTTI3, ELENA RUBINI3, MICHELA FERRO4, EMANUELE DE SIMONE4, carla naretto4, ANTONELLA BARRECA5, ANDREA SAMMARTINO4, DANIELA ROSSI4 and ROBERTA FENOGLIO4, 1University of Torino, Torino, Italy, 2University of Turin, Torino, Italy, 3University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy, 4University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, San Giovanni Bosco Hub Hospital, Turin, Italy, 5Pathology Unit, Città della Salute e della Scienza, Turin, Italy

    Background/Purpose: Rituximab (RTX), an anti-CD20 monoclonal antibody, has shown to be an effective induction treatment for small-vessel vasculitides associated with antineutrophil cytoplasm antibodies (AAV) in…
  • Abstract Number: 0748 • ACR Convergence 2023

    Assessment of Myositis-related Interstitial Lung Disease by ⁶⁸ Ga-DATA.SA.FAPi PET/CT

    Kastriot Kastrati1, Thomas S Nakuz2, Oana C Kulterer2, Stephan Blüml3, Michael Bonelli3, Irina Gessl4, Hans-Peter Kiener3, Werner Langsteger2, Daniel Mrak3, Florian Prayer5, Helmut Prosch5, Elisabeth Simader3, Tatjana Traub-Weidinger2, Daniel Aletaha6, Helga Radner7, Marcus Hacker2 and Peter Mandl3, 1Dept of Biomedical Imaging and Image-guided Therapy, Medical University of Vienna (Austria), Vienna, Austria, 2Division of Nuclear Medicine, Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Vienna, Austria, 3Division of Rheumatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria, 4Department of Rheumatology, Medical University of Vienna, Vienna, Austria, 5Department of Biomedical Imaging and Image-guided Therapy, Medical University of Vienna, Vienna, Austria, 6Division of Rheumatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria, Wien, Austria, 7Division of Rheumatology, Medical University of Vienna, Vienna, Austria

    Background/Purpose: Interstitial lung disease (ILD) is a common manifestation of idiopathic inflammatory myopathies (IIM) ranging up to 78% in IIM and is a key contributor…
  • Abstract Number: 0754 • ACR Convergence 2023

    Towards a Guide for Evidence-based Remote Monitoring: Sensitivity of Patient Reported Outcomes to Change in Disease Activity Status in Early and Established Rheumatoid Arthritis

    Agnes Loiojen1, Elise van Mulligen2, Annette van der Helm-van Mil3 and Pascal de Jong2, 1Erasmus Medical Center, department of rheumatology, Rotterdam, Netherlands, 2Erasmus Medical Center, Rotterdam, Netherlands, 3Leiden University Medical Center, Leiden, Netherlands

    Background/Purpose: In the treatment and follow-up of rheumatoid arthritis (RA) patients, it has become more desirable to detect changes in disease activity through remote monitoring.…
  • Abstract Number: 0734 • ACR Convergence 2023

    Immune Isoform Atlas: Landscape of Alternative Splicing in Human Immune Cells and Involvement of Dysregulated Alternative Splicing in Autoimmune Diseases

    Jun Inamo1, Akari Suzuki2, Mahoko Takahashi Ueda1, Kensuke Yamaguchi1, Hiroshi Nishida3, Katsuya Suzuki4, Yuko Kaneko5, Tsutomu Takeuchi6, Hiroaki Hatano7, Kazuyoshi Ishigaki8, Yasushi Ishihama3, Kazuhiko Yamamoto9 and Yuta Kochi1, 1Tokyo Medical and Dental University, Tokyo, Japan, 2RIKEN Center for Integrative Medical Science, Kanagawa, Japan, 3Kyoto University, Kyoto, Japan, 4Keio University, Shinjuku, Japan, 5Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan, 6Keio University School of Medicine and Saitama Medical University, Tokyo, Japan, 7Laboratory for Human Immunogenetics, RIKEN Center for Integrative Medical Sciences, Tokyo, Japan, 8RIKEN, Tokyo, Japan, 9Department of Allergy and Rheumatology, Graduate School of Medicine, The University of Tokyo and Laboratory for Autoimmune Diseases, Center for Integrative Medical Sciences, RIKEN, Yokohama, Japan

    Background/Purpose: Alternative splicing events play a critical role in the immune system1,2 and one of major causal mechanisms for complex traits including immune-mediated diseases (IMDs),…
  • Abstract Number: 0753 • ACR Convergence 2023

    Facility Variation in HLA-B*58:01 Allele Testing for Asian and Black Patients Receiving Allopurinol in the Veterans Affairs Healthcare System

    Jeremy Sullivan1, Anna Ware2, Gary Tarasovsky3, Cherish Wilson4, Mary Whooley3, Jasvinder Singh5, Jinoos Yazdany6 and Gabriela Schmajuk4, 1University of California San Francisco, San Francisco, CA, 2Palo Alto VA, Minneapolis, MN, 3San Francisco VA, San Francisco, CA, 4UCSF / SFVA, San Francisco, CA, 5University of Alabama at Birmingham, Birmingham, AL, 6University of California, General Department of Medicine, Division of Rheumatology, San Francisco, CA

    Background/Purpose: New guidelines published in 2020 conditionally recommend HLA-B*58:01 allele testing for South Asian and Black patients receiving allopurinol to reduce the risk of severe…
  • Abstract Number: 0738 • ACR Convergence 2023

    A Genome-wide Association Study Suggests New Susceptibility Loci for Primary Antiphospholipid Syndrome

    Desire Casares1, Manuel Martínez-Bueno2, Maria Orietta Borghi3, Guillermo Pons-Estel4, PRECISESADS Clinical Consortium2, Yu Zuo5, Gerard Espinosa6, Alexandra Zhernakova7, Cisca Wijmenga7, Timothy Radstake8, Lucas van den Hoogen9, Guillermo Reales10, Chris Wallace10, Joel Guthridge11, Judith James11, Ricard Cervera12, Pierluigi Meroni13, Javier Martin14, Jason Knight5, Marta Alarcon-Riquelme15 and Amr Sawalha1, 1University of Pittsburgh, Pittsburgh, PA, 2Centre for Genomics and Oncological Research, Granada, Spain, 3University of Milan, Milan, Italy, 4CREAR, Rosario, Argentina, 5University of Michigan, Ann Arbor, MI, 6Hospital Clinic, Barcelona, Spain, 7University of Groningen, Groningen, Netherlands, 8University Medical Center Utrecht, Carlisle, IL, 9Radboudumc and Sint Maartenskliniek, Nijmegen, Netherlands, 10University of Cambridge, Cambridge, United Kingdom, 11Oklahoma Medical Research Foundation, Oklahoma City, OK, 12Hospital Clínic de Barcelona, Barcelona, Spain, 13IRCCS Istituto Auxologico Italiano, Cusano Milanino, Milan, Italy, 14Instituto de Parasitologia y Biomedicina Lopez-Neyra - CSIC, Granada, Spain, 15Center for Genomics and Oncological Research (GENYO), Granada, Spain

    Background/Purpose: Primary antiphospholipid syndrome (PAPS) is a rare autoimmune disease characterized by the presence of antiphospholipid antibodies and the occurrence of thrombotic events and pregnancy…
  • Abstract Number: 0697 • ACR Convergence 2023

    Alterations in Composition and Function of Gut Microbiota in Patients with Large-Vessel Vasculitis

    Adam Mayer1, Weiming Hu2, Kyle Bittinger2, Rui Liang3, Naomi Amudala3, Shubhasree Banerjee3, Peter Merkel3 and Rennie Rhee3, 1University of Pennsylvania/Children's Hospital of Philadelphia, Philadelphia, PA, 2Children's Hospital of Philadelphia, Philadelphia, PA, 3University of Pennsylvania, Philadelphia, PA

    Background/Purpose: The gut microbiome has a critical role in immune homeostasis and there is growing interest in understanding the relationship between the microbiome and autoimmunity.…
  • Abstract Number: 0656 • ACR Convergence 2023

    Concordance and Prognostic Relevance of Different Definitions of Systemic Sclerosis Interstitial Lung Disease Progression

    enrico De Lorenzis1, Francesco Del Galdo2, gerlando Natalello1, Stefano Di Donato2, lucrezia verardi1, Vishal Kakkar2, pier giacomo Cerasuolo1, Francesco Varone3, Luca Richeldi3, Maria Antonietta D'Agostino1 and Silvia Bosello1, 1Division of Rheumatology - Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli, Rome, Italy, 2University of Leeds - Leeds Institute of Rheumatic and Muskuloskeletal Medicine, Leeds, United Kingdom, 3Division of Pulmonology - Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli, Rome, Italy

    Background/Purpose: Interstitial Lung Disease (ILD) in systemic sclerosis (SSc) is a common complication that has varied progression rate and prognosis. Different progression definitions include minimal…
  • Abstract Number: 0759 • ACR Convergence 2023

    Derivation and Validation of Four Patient Clusters in Still’s Disease, Results from GIRRCS AOSD-study Group and AIDA Network Still Disease Registry

    Piero Ruscitti1, Antonio Vitale2, Ilenia Di Cola1, Roberto giacomelli3 and Luca Cantarini4, 1University of L'Aquila, L'Aquila, Italy, 2University of Siena, Siena, Italy, 3University of Rome Campus Biomedico, Rome, Italy, 4Department of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy

    Background/Purpose: Still's disease is a rare inflammatory disorder, these patients have a highly heterogeneous disease according to age of onset, clinical presentation, presence of life-threatening…
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