ACR Meeting Abstracts

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  • Abstract Number: L03 • ACR Convergence 2023

    Efficacy and Safety of Targeted Therapies in VEXAS Syndrome: Retrospective Study from the French VEXAS Group

    Jerome Hadjadj1, Yann Nguyen1, Dalila Mouloudji1, Rim Bourguiba1, Mael Heiblig2, Aloui Hassina1, Valentin Lacombe3, Samuel Ardois4, Corrado Campochiaro5, Alexandre Maria6, Thibault Comont7, Estibaliz Lazaro8, Francois Lifermann9, Guillaume Le Guenno10, Herve Lobbes10, Roderau Outh11, Julien Campagne12, Cyrille Coustal6, Alice Garnier13, Yvan Jamilloux2, Aurore Meyer14, Noemie Abisror15, Olivier Kosmider1, Vincent Jachiet1, Olivier FAIN16, Benjamin Terrier17, Arsene Mekinian1 and Sophie Georgin-Lavialle18, 1APHP, Paris, France, 2Lyon Hospital, Lyon, France, 3Angers Hospital, Angers, France, 4Rennes Hospital, Rennes, France, 5San Raffaele Scientific Institute, Milan, Italy, 6Montpellier Hospital, Montpellier, France, 7Oncopole Toulouse, Toulouse, France, 8Bordeaux Hospital University, Pessac, France, 9Dax Hospital, Dax, France, 10Clermont Hospital, Clermont Ferrand, France, 11Perpignan Hospital, Perpignan, France, 12Hpital Robert Schuman, Metz, France, 13Nantes Hospital, Nantes, France, 14Strasbourg Hospital, Strasbourg, France, 15Internal Medicine Saint Antoine Hospital, Paris, France, 16Hopital Saint Antoine APHP, Paris, France, 17Cochin Hospital, Paris, France, 18AP-HP, Tenon hospital, Paris, France

    Background/Purpose: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a monogenic disease of adults due to acquired somatic mutations of the UBA1 gene. Patients…
  • Abstract Number: L08 • ACR Convergence 2023

    Early Clinical Development of CIT-013, a First in Class NETosis Inhibitor, in a Randomized Phase I Dose Escalation Study in Healthy Volunteers Demonstrating Potent Inhibition of LPS Induced Neutrophil Extracellular Trap Formation

    Leonie Middelink1, Renato Chirivi2, Helmuth van Es2, Eric Meldrum2, Peter van Zandvoort2, Tirza Bruurmijn2, Matthijs Moerland3 and Patrick Round2, 1Middelinc., Utrecht, Netherlands, 2Citryll BV, Oss, Netherlands, 3CHDR, Leiden, Netherlands

    Background/Purpose: Aberrant Neutrophil Extracellular Traps (NETs) production contributes to the pathophysiology of multiple inflammatory and autoimmune diseases such as Rheumatoid arthritis (RA) Hidradenitis suppurativa (HS),…
  • Abstract Number: L05 • ACR Convergence 2023

    DICKENS: A Multicentre Randomised Controlled Trial of Diacerein for Knee Osteoarthritis with Effusion-Synovitis

    Guoqi Cai1, Graeme Jones2, Flavia Cicuttini3, Anita Wluka3, Yuanyuan Wang3, Catherine Hill4, Helen Keen5, Benny Antony2, barbara de Graaff2, Michael Thompson2, Tania Winzenberg2, Kathy Buttigieg2, Petr Otahal2 and Dawn Aitken2, 1School of Public Health, Anhui Medical University, Hefei, China, 2Menzies Institute for Medical Research, University of Tasmania, Hobart, Australia, 3Monash University, Melbourne, Australia, 4The Queen Elizabeth Hospital, University of Adelaide, Adelaide, Australia, 5Fiona Stanley Hospital, Murdoch, Australia

    Background/Purpose: There is an unmet need for treatments of knee OA. Diacerein is recommended for alleviating pain in OA patients for its anti-inflammatory effect by…
  • Abstract Number: L20 • ACR Convergence 2023

    Telitacicept, a Human Recombinant Fusion Protein Targeting and Neutralizing B Lymphocyte Stimulator (BlyS) and a Proliferation-Inducing Ligand (APRIL), in Rheumatoid Arthritis (RA) Patients with an Inadequate Response to Methotrexate (MTX): A Randomized, Double-Blind, Phase 3 Study

    Li Wang1, Dong Xu2, Jianmin Fang3, Qing Zuraw4 and Fengchun Zhang1, 1Peking Union Medical College Hospital, Beijing, China, 2Peking Union Medical College Hospital, Dong Cheng District, China, 3RemeGen Co, Ltd., Yantai, China, 4RemeGen Biosciences, Inc., San Francisco, CA

    Background/Purpose: Telitacicept is a recombinant fusion protein targeting and neutralizing BLyS and APRIL. This phase 3, randomized, double-blind study evaluated the efficacy and safety of…
  • Abstract Number: 0281 • ACR Convergence 2023

    Is There a Decreased Risk of Developing CF Arthropathy in CF Patients Undergoing Treatment That Targets the F508del Mutation?

    Kristin Schmiedeberg1, Anna-Lena Walter1, Lukas Joos1, Martin Brutsche1, Johannes von Kempis2 and Andrea Rubbert-Roth1, 1Cantonal Hospital St. Gallen, St. Gallen, Switzerland, 2Kantonsspital St. Gallen, Division of rheumatology and immunology, St. Gallen, Switzerland

    Background/Purpose: Cystic fibrosis (CF) is characterized by mutations within the CFTR (cystic fibrosis transmembrane conductance regulator) gene that result in a defect of the chloride…
  • Abstract Number: 0286 • ACR Convergence 2023

    Relationship Between High Resolution Computed Tomography(HRCT) Quantitative Scores and Physiological and Clinical Features in Antisynthetase Syndrome Related Interstitial Lung Disease

    Sangmee Bae1, Fereidoun Abtin2, Grace Kim3, Daniela Markovic4, Siamak Moghadam-Kia5, Chester V. Oddis6, Lila Pourzand2, Didem Saygin6, Daniel Sullivan7, Koichi Yamaguchi5, Donald Tashkin3, Christina Charles-Schoeman8, Jonathan Goldin3 and Rohit Aggarwal6, 1UCLA Rheumatology, Los Angeles, CA, 2UCLA Radiological Sciences, Los Angeles, CA, 3University of California Los Angeles, Los Angeles, CA, 4UCLA Department of Medicine Statistics Core, Los Angeles, CA, 5University of Pittsburgh Medical Center Rheumatology, Pittsburgh, PA, 6University of Pittsburgh, Pittsburgh, PA, 7UCLA Pulmonology, Pittsburgh, PA, 8UCLA Medical Center, Santa Monica, CA

    Background/Purpose: High resolution chest computed tomography (HRCT) plays an important role in the clinical evaluation of interstitial lung disease (ILD) by demonstrating the extent of…
  • Abstract Number: 0283 • ACR Convergence 2023

    Transcriptomic Profiles in Muscle Biopsies from Systemic Sclerosis Patients with Different Autoantibodies

    Maria Casal-Dominguez1, Jose Cesar Milisenda2, Iago Pinal-Fernandez1, Katherine Pak3, Sandra Muñoz-Braceras1, Jose Jiram Torres-Ruiz1, Stefania Dell´Orso4, Faiza Naz4, Gustavo Gutierrez-Cruz4, Shamima Islam1, Yaiza Duque-Jaimez2, Ana Matas-Garcia2, Francesc J Garcia-Garcia2, Mariona Guitart-Manpel2, Gloria Garrabou2, Ernesto Trallero-Araguas5, Brian Wallit6, Lisa Christopher-Stine7, Tom Lloyd8, Alfredo Guillen-Del-Castillo9, Carmen Pilar Simeon-Aznar10, Josep Maria Grau2, Albert Selva-O’Callaghan5 and Andrew Mammen11, 1Muscle Disease Unit, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, 2Muscle Research Unit, Internal Medicine Service, Hospital Clinic, Barcelona, Spain, 3National Institutes of Health, Bethesda, MD, 4National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, 5Systemic Autoimmune Disease Unit, Vall d’Hebron Institute of Research, Barcelona, Spain, 6National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, MD, 7Johns Hopkins University, Baltimore, MD, 8Johns Hopkins University School of Medicine, Baltimore, MD, 9Unit of Autoimmune Diseases, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain, 10Department of Internal Medicine, Systemic Autoimmune Diseases Unit, Hospital Universitario Vall d'Hebronh, Barcelona, Spain, 11NIH, Bethesda, MD

    Background/Purpose: The inflammatory myopathies (IM) include dermatomyositis (DM), antisynthetase syndrome (AS), immune-mediated necrotizing myopathy (IMNM), inclusion body myositis (IBM), and overlap myositis (OM), in which…
  • Abstract Number: 0284 • ACR Convergence 2023

    Phenotyping Calcinosis: A Rare Manifestation of Rare Diseases

    Michele Diomedi1, Federico Fattorini1, Simone Barsotti2, Elenia Laurino1, Chiara Cardelli1, Linda Carli1 and Marta Mosca1, 1Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy, 2Internal Medicine, Ospedale di Livorno, Pisa, Italy

    Background/Purpose: Idiopathic inflammatory myopathies (IIMs) are rare systemic autoimmune disorders, with a pleiotropic clinical picture, specifically characterized from the inflammatory involvement of striate muscles. Calcinosis…
  • Abstract Number: 0276 • ACR Convergence 2023

    Disease Control in Patients with Monogenetic Autoinflammatory Diseases Under Canakinumab Treatment – Comparison of 30 Months Interim Data from the RELIANCE Registry

    Ivan Foeldvari1, Tilmann Kallinich2, Norbert Blank3, Joerg Henes4, Birgit Kortus-Goetze5, Prasad T. Oommen6, Anne Pankow7, Tobias Krickau8, Catharina Schuetz9, Gerd Horneff10, Juergen Rech11, Frank Weller-Heinemann12, Ales Janda13, Markus Hufnagel14, Florian M. Meier15, Frank Dressler16, Michael Borte17, Ioana Andreica18, Peter Wasiliew19, Michael Fiene20, Daniel Windschall21, Martin Krusche22, Tania Kuempfel23, Julia Weber-Arden24 and Jasmin B. Kuemmerle-Deschner25, 1Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, 2Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité Universitätsmedizin Berlin, Nuremberg, Germany, 3University Hospital Heidelberg, Heidelberg, Germany, 4University Hospital Tuebingen, Tuebingen, Germany, 5Department of Internal Medicine, Division of Nephrology,University Hospital of Giessen and Marburg, Marburg, Germany, 6Department of Pediatric Oncology, Hematology and Clinical Immunology, Center for Child and Adolescent Health,Medical Faculty Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, 7Department of Rheumatology and Clinical Immunology,Charité-Universitätsmedizin Berlin, Berlin, Germany, 8Pediatrics, Friedrich-Alexander University Erlangen-Nuernberg (FAU), Erlangen, Germany, 9Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus,Technische Universität Dresden, Dresden, Germany, 10Asklepios Klinik Sankt Augustin GmbH, Bonn, Germany, 11University Clinic Erlangen, Erlangen, Germany, 12Division of Pediatric Rheumatology, Prof. Hess Children's Hospital, Bremen, Bremen, Germany, 13Department of Pediatrics and Adolescent Medicine, University Medical Center Ulm, Ulm, Germany, 14Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Medical Center, Medical Faculty, University of Freiburg, Freiburg, Germany, 15Department of General Pharmacology and Toxicology, Goethe University Hospital and Goethe University Frankfurt, Frankfurt am Main, Germany, 16Department of Paediatric Pneumology, Allergology and Neonatology, Children's Hospital, Hannover Medical School, Hannover, Germany, 17Hospital for Children & Adolescents, St. Georg Hospital, Leipzig, Germany, 18Rheumazentrum Ruhrgebiet Herne, Herne, Germany, 19Division of Pediatric Rheumatology and autoinflammation reference center Tuebingen, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany, 20Rheumatology Center Greifswald, Greifswald, Germany, 21Clinic of Paediatric and Adolescent Rheumatology, St. Josef-Stift Sendenhorst, Northwest German Center for Rheumatology, Sendenhorst, Germany, 22UKE, Hamburg, Germany, 23Institute of Clinical Neuroimmunology, Biomedical Center and University Hospital, Ludwig-Maximilians Universität München, Muenchen, Germany, 24Novartis Innovative Medicines, Nuernberg, Germany, 25med.uni-tuebingen, Tübingen, Germany

    Background/Purpose: Treatment of autoinflammatory periodic diseases (AID) with the interleukin-1β inhibitor canakinumab (CAN) has been shown to be safe and effective in controlled trials and…
  • Abstract Number: 0280 • ACR Convergence 2023

    Immunosuppression in the Treatment of Susac Syndrome: A Retrospective Evaluation of the Largest Cohort of Susac Syndrome

    Adam Brown1, Robert Rennebohm2, Ghulam Abbas Kharal2, Devon Conway2, Sunil Srivastava3, Sumit Sharma2, Careen Lowder2, Leonard Calabrese4 and Rula Hajj-Ali2, 1Cleveland Clinic Department of Rheumatic and Immunologic Diseases, Cleveland, OH, 2Cleveland Clinic, Cleveland, OH, 3Cincinnati Eye Institute, Cincinnati, OH, 4Cleveland Clinic / Department of Rheumatic and Immunologic Diseases, Cleveland, OH

    Background/Purpose: Susac Syndrome is a rare autoimmune condition causing microvascular occlusions in the brain, retina and inner ear leading to the characteristic triad of encephalopathy,…
  • Abstract Number: 0277 • ACR Convergence 2023

    Anti-carbamylated Antibodies in Autoimmunity – Focus on Their Diagnostic and Prognostic Value: A Systematic Review and Meta-analysis

    Julie Sarrand, Dorian Parisis, Muhammad Soyfoo and Paschalis Sidiras, Erasmus Hospital, Brussels, Belgium

    Background/Purpose: This is the first systematic review and meta-analysis to examine the prevalence, the diagnostic and the prognostic value of anti-carbamylated protein (CarP) antibodies in…
  • Abstract Number: 0274 • ACR Convergence 2023

    A Mix-and-match COVID-19 Vaccination Strategy in Patients with Rheumatic Diseases on Rituximab

    Nathalie Amiable1, Mehdi Benlarbi2, Mathieu Dube3, Sonia Theriault4, Alexandra Godbout1, Anne-Sophie Julien5, Gloria-Gabrielle Ortega-Delgado6, Melina Duchesne7, Rose Cloutier8, Josee Perreault9, Annie Gravel10, Lison Fournier11, Giuliana Alfonso12, Josiane Bourre-Tessier13, Marie Hudson14, Nicolas Richard15, Jean-Paul Makhzoum16, Arielle Mendel17, Sasha Bernatsky12, Marc Dionne18, Michael Libman19, Gaston De Serres20, Mélanie Dieudé21, louis Flamand22, Daniel Kaufmann23, Andrés Finzi24, Renée Bazin25, Ines Colmegna26 and Paul R. Fortin27, 1Centre ARThrite - CHU de Quebec - Universite Laval, Quebec City, QC, Canada, 2CRCHUM, Université de Montréal, Montreal, QC, Canada, 3Centre de recherche du Centre Hospitalier de l'Université de Montréal, Montreal, QC, Canada, 4The Research Institute of the McGill University Health Centre, Montreal, QC, Canada, 5Département de mathématiques et statistique, Université Laval, Québec, QC, Canada, 6Centre de Recherche du CHUM, Montreal, QC, Canada, 7Centre de recherche du Centre Hospitalier de l'Universitaire de Montréal, Montreal, QC, Canada, 8Centre de recherche hospitalier de l'Université de Montréal, Montreal, QC, Canada, 9Hema-Quebec, Quebec City, QC, Canada, 10CHU de Québec - CHUL Axe des Maladies Infectieuses et Immunitaires, Quebec City, QC, Canada, 11Centre de recherche du CHU de Quebec - Université Laval, Quebec City, QC, Canada, 12Research Institute of the McGill University Health Centre, Montreal, QC, Canada, 13Centre hospitalier de l'Université de Montréal (CHUM), Université de Montréal, Montréal, QC, Canada, 14McGill University, Montréal, QC, Canada, 15Hopital Maisonneuve Rosemont, Montreal, QC, Canada, 16Vasculitis Clinic, Canadian Network for Research on Vasculitides, Hopital du Sacre-Coeur de Montreal, Montreal, QC, Canada, 17McGill University Health Centre, Montréal, QC, Canada, 18Centre de recherche du CHU de Québec - Université Laval, Quebec City, QC, Canada, 19McGill Centre for Viral Diseases, Montreal, QC, Canada, 20Centre de recherche du CHU de Québec – Université Laval, Quebec City, QC, Canada, 21Microbiology, Infectiology and Immunology Department, Faculty of Medicine, Université de Montréal, CHUM Research Center, Héma-Québec, Montreal, QC, Canada, 22Centre ARThrite - CHU de Quebec - Université Laval, Quebec City, QC, Canada, 23University of Montreal, University of Lausanne, Lausanne University Hospital, Montreal, QC, Canada, 24Université de Montréal, Montreal, QC, Canada, 25Héma-Québec, Affaires Médicales et Innovation, Quebec City, QC, Canada, 26The Research Institute of the McGill University Health Centre, Montréal, QC, Canada, 27Centre ARThrite - CHU de Québec - Université Laval, Quebec City, QC, Canada

    Background/Purpose: Patients with rheumatic diseases (RD) on rituximab (RTX) have increased mortality following COVID-19 and reduced antibody response post-vaccine. We tested whether a mix-and-match strategy…
  • Abstract Number: 0278 • ACR Convergence 2023

    NOD2 Genotyping Landscape in Yao Syndrome

    Hafsa Nomani1, Ashmia Saif2, Frank Hwang1 and Qingping Yao1, 1Stony Brook University, Stony Brook, NY, 2Stony Brook University Hospital, Syosset, NY

    Background/Purpose: Yao syndrome (YAOS, OMIM 617321) is formerly designated NOD2-associated autoinflammatory disease. A spectrum of NOD2 mutations have been associated with this disease. This study…
  • Abstract Number: 0273 • ACR Convergence 2023

    Disease Characteristics and Pancreatic Damage in IgG4-Related Disease with Pancreatic Involvement

    Guy Katz1, Liam Harvey2, Yasmin Hernandez-Barco1, Ana Fernandes1, Grace McMahon1, Isha Jha1, Zachary Wallace3, Cory Perugino1 and John Stone4, 1Massachusetts General Hospital, Boston, MA, 2Frank H. Netter School of Medicine at Quinnipiac University, New Haven, CT, 3Massachusetts General Hospital, Newton, MA, 4Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Concord, MA

    Background/Purpose: IgG4-related disease (IgG4-RD) is a systemic immune-mediated fibroinflammatory disease that can affect nearly every organ system. Autoimmune pancreatitis (AIP) is among the most common…
  • Abstract Number: 0282 • ACR Convergence 2023

    DNA Methylation Markers in Peripheral Blood as Effective Diagnostic Biomarkers for Adult-onset Still’s Disease

    Shijia Rao1, Bo Zhang2, Yuwei Li3, Tian Zhou1, Xiaoli Shi1, Wei Shi4, Yongfang Jiang1, Wenlong Wang1, Yun Xu1, Yi Tian1, Min Zhang1, Jing Ma1, Hongyu Luo1, Hui Luo4, Hongjun Zhao4, Fen Li1, Guanghui Ling1, Jing Tian1, Xiaojing Kang5, Junqin Liang5, Aijun Chen6, Shuang Chen6, Jianjun Qiao7, Gang Wang8, Lei wang8, Bing Li8, Youkun Lin9, Sijian Wen9, Zhiqiang Song10, Min Zhang10, Jun Liu4, Yixin Tan1, Haijing Wu1, Hai Long1, Ming Zhao11 and Qianjin Lu12, 1The Second Xiangya Hospital, Central South University, Changsha, China, 2Chinese Academy of Medical Sciences & Peking Union Medical College, Nanjing, China, 3University of Science and Technology of China, Hefei, China, 4Xiangya Hospital, Central South University, Changsha, China, 5People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, China, 6The First Affiliated Hospital of Chongqing Medical University, Chongqing, China, 7The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China, 8Xijing Hospital, Fourth Military Medical University, Xi'an, China, 9The First Affiliated Hospital of Guangxi Medical University, Nanning, China, 10Southwest Hospital, Third Military Medical University (Army Medical University), Chongqing, China, 11Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing, China, 12Hospital for Skin Diseases, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Changsha, China

    Background/Purpose: Adult-onset still's disease (AOSD) is an auto-inflammatory disease affecting multiple systems. The diagnostic procedures of AOSD are still a challenge. We aimed to find…
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