ACR Meeting Abstracts

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  • Abstract Number: 0669 • ACR Convergence 2023

    Optical Coherence Tomography Angiography in Systemic Sclerosis: Correlations with Morphological and Functional Peripheral Microvascular Status

    Carlo Alberto Cutolo1, Elvis Hysa2, Andrea Cere3, Paola Toma1, Tommaso Cannavacciuolo1, Chiara Toma1, Serena Balito4, Veronica Gerli4, Alberto Sulli3, Vanessa Smith5, Carlo Enrico Traverso1, Massimo Nicolò1 and Maurizio Cutolo6, 1Ophthalmology Clinic DiNOGMI, IRCCS Ophthalmology Unit San Martino Polyclinic, University of Genoa, Genoa, Italy, 2Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino Polyclinic Hospital, Genova, Italy, 3Laboratory of Experimental Rheumatology and Academic Division of Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino Polyclinic Hospital, Genoa, Italy, 4Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Italy - IRCCS San Martino Polyclinic, Genoa, Italy, 5Ghent University Hospital, Gent, Belgium, 6Laboratory of Experimental Rheumatology, Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino Polyclinic, Genova, Italy

    Background/Purpose: Systemic sclerosis (SSc) is a rare and complex autoimmune disease featured by a progressive microvascular damage. The involvement of the ocular microvasculature has been…
  • Abstract Number: 0637 • ACR Convergence 2023

    Comparison of Four Risk Stratification Models for Prediction of Mortality in Systemic Sclerosis-associated Pulmonary Arterial Hypertension in the EUSTAR Cohort

    Hilde Jenssen Bjørkekjær1, Cosimo Bruni2, Cathrine Brunborg3, Patricia Carreira4, Paolo Airò5, Carmen Pilar Simeon-Aznar6, Marie-Elise Truchetet7, Alessandro Giollo8, Alexandra Balbir-Gurman9, Mickael Martin10, Christopher Denton11, Armando Gabrielli12, Havard Fretheim13, Imon Barua13, Helle Bitter14, Oyvind Midtvedt13, Torhild Garen15, Kaspar Broch16, Arne Andreassen17, Yoshiya Tanaka18, Gabriela Riemekasten19, Ulf Müller-Ladner20, marco Matucci Cerinic21, Ivan Castellvi22, Elise Siegert23, Eric Hachulla24, Oliver Distler2 and Anna-Maria Hoffmann-Vold13, 1Department of Rheumatology, Hospital of Southern Norway, Kristiansand & University of Oslo, Institute of Clinical Medicine, Oslo, Norway, 2Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, 3Oslo Centre for Biostatistics and Epidemiology, Research Support Services, Oslo University Hospital - Rikshospitalet, Oslo, Norway, 4Hospital Universitario 12 de Octubre, Madrid, Spain, 5Spedali Civili di Brescia, Scleroderma UNIT, UOC Reumatologia ed Immunologia Clinica, Piazzale Spedali Civili 1, 25123, Brescia, Italy, 6Department of Internal Medicine, Systemic Autoimmune Diseases Unit, Hospital Universitario Vall d'Hebronh, Barcelona, Spain, 7Bordeaux University Hospital, Bordeaux, France, 8University of Verona, Rheumatology Section, Department of Medicine, Verona, Italy, Verona, Italy, 9Rheumatology Institute, Rambam Health Care Campus and Rappaport Faculty of |Medicine, Technion, Haifa, Israel, 10Department of Internal Medicine, INSERM U1313, Poitiers University, Poitiers University Hospital, Poitiers, France, 11University College London, London, United Kingdom, 12Ospedali Riuniti Marche, Ancona, Italy, 13Oslo University Hospital, Oslo, Norway, 14Sorlandet sykehus, Kristiansand, Norway, 15Dept of Rheumatology, University Hospital Oslo, Oslo, Norway, 16Oslo University Hospital, Rikshospitalet, Department of Cardiology, Oslo, Norway, KG Jebsen center, Institute for Experimental Medical Research, University of Oslo, Oslo, Norway, 17Oslo University Hospital, Rikshospitalet, Department of Cardiology, Oslo, Norway, 18University of Occupational and Environmental Health, Kitakyushu, Japan, 19University Clinic Schleswit-Holstein (UKSH), Lübeck, Germany, 20Justus Liebig University Gießen, Campus Kerckhoff, Bad Nauheim, Germany, 21Unit of Immunology, Rheumatology, Allergy and Rare diseases, IRCCS San Raffaele Hospital, Milan, Milan, Italy, 22Rheumatology, Hospital Universitari de la Santa Creu i Sant Pau, Sant Just Desvern, Spain, 23Department of Rheumatology, Charité University Hospital, Charité Platz 1, D-10117, Berlin, Germany, 24University of Lille, Lille, France

    Background/Purpose: The 2022 ESC/ERS Guidelines recommend comprehensive risk stratification at diagnosis of pulmonary arterial hypertension (PAH) to guide optimized management.1Several risk stratification tools have been…
  • Abstract Number: 0667 • ACR Convergence 2023

    The Risk of Stroke and Myocardial Infarction in Systemic Sclerosis: A Population-based Study

    Jiwon Hwang1, Yeonghee Eun2, Kyungdo Han3 and Joong Kyong Ahn2, 1Samsung Changwon Hospital, Sungkyunkwan University School of Medicine, Changwon, South Korea, 2Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, South Korea, 3Department of Statistics and Actuarial Science, Soongsil University, Seoul, South Korea

    Background/Purpose: Previous studies have suggested a link between systemic sclerosis (SSc) and cardiovascular disease, but large-scale data are still lacking due to the nature of…
  • Abstract Number: 0672 • ACR Convergence 2023

    Risk of New-onset Depression in Patients with Systemic Sclerosis : A Nationwide Population-based Study

    Hahee Son and Su-Jin Moon, Yeouido St. Mary's Hospital, Catholic University of Korea, Seoul, South Korea

    Background/Purpose: Systemic sclerosis (SSc) is a rare chronic inflammatory disease characterized by vasculopathy, autoimmunity and progressive fibrosis in various organs. The patients with SSc are…
  • Abstract Number: 0693 • ACR Convergence 2023

    Comparison of the Cardiovascular Risk in Patients with Vasculitis and Diabetes Mellitus

    Ana Belen Arevalo1, Harshvardhan Zala2, Andrea Ramirez Gomez1 and Kichul Ko3, 1University of Chicago, Chicago, IL, 2Indiana University School of Medicine, Indianapolis, IN, 3The University of Chicago, Chicago, IL

    Background/Purpose: Cardiovascular disease (CVD) has been reported as the most common cause of death in ANCA-associated vasculitis (AAV)1,2. Systemic inflammation, in addition to traditional risk…
  • Abstract Number: 0688 • ACR Convergence 2023

    Efficacy and Safety Experience with Avacopan Beyond 52 Weeks in the Early Access Program (EAP)

    Federico Alberici1, Carlo Salvarani2, Christine Chan3, Achim Obergfell4 and Tamara Popov4, 1Universita degli Studi di Brescia, Brescia, Italy, 2Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy, 3CSL Vifor, Redwood City, CA, 4CSL Vifor, Glattbrugg, Switzerland

    Background/Purpose: Avacopan, a selective C5aR1 inhibitor, has demonstrated efficacy and safety over 52 weeks in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. However, efficacy and…
  • Abstract Number: 0677 • ACR Convergence 2023

    Analysis of Clinical Outcomes in Eosinophilic Granulomatosis with Polyangiitis (EGPA) Treated with Mepolizumab over 2 Years for Remission Induction or Maintenance: A Single Center Experience in Japan

    SOKO KAWASHIMA1, Yoshinori Komagata1, Mitsumasa Kishimoto2 and Shinya Kaname1, 1Kyorin University School of Medicine, Tokyo, Japan, 2Kyorin University School of Medicine, Yokohoma, Japan

    Background/Purpose: Contrary to Western countries, MPO-ANCA-associated vasculitis (MPO-AAV) is dominant in Japan or Asian countries. It is possible that therapeutic responses to EGPA with mepolizumab…
  • Abstract Number: 0655 • ACR Convergence 2023

    A Novel Association Between Lipodermatosclerosis and Key Vascular Outcomes in Systemic Sclerosis

    Srijana Davuluri1, Puneet Kapoor2, Swarna Nandyala2, David Fiorentino3, Julia Simard4 and Lorinda Chung5, 1Stanford University- School of Medicine, Sunnyvale, CA, 2Stanford University- School of Medicine, Palo Alto, CA, 3Department of Dermatology, Stanford University, Redwood City, CA, 4Department of Epidemiology & Population Health, Stanford University School of Medicine, Stanford, CA, 5Department of Medicine, Division of Immunology and Rheumatology, Stanford University School of Medicine, Woodside, CA

    Background/Purpose: Lipodermatosclerosis (LDS) is a progressive manifestation of chronic venous insufficiency characterized by distinctive lower extremity changes, including an "inverted champagne bottle" appearance, skin hyperpigmentation,…
  • 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: 0646 • ACR Convergence 2023

    Racial Variability in Immune Responses Only Partially Explains Differential Systemic Sclerosis Disease Severity

    Kamini Kuchinad1, Ji Soo Kim1, Adrianne Woods1, Gwen Leatherman1, Laura Gutierrez2, Maureen Mayes3, Robyn Domsic4, Paula Ramos5, Richard Silver5, John Varga6, Lesley Ann Saketkoo7, Suzanne Kafaja8, Victoria Shanmugam9, Jessica Gordon10, Lorinda Chung11, Elana Bernstein12, Pravitt Gourh13, Francesco Boin14, Daniel Kastner15, Scott Zeger16, Livia Casciola-Rosen1, Fred Wigley1 and Ami Shah17, 1Department of Rheumatology, Johns Hopkins Medicine, Baltimore, MD, 2Johns Hopkins Medicine, Baltimore, MD, 3Division of Rheumatology, University of Texas McGovern Medical School, Houston, TX, 4University of Pittsburgh, Pittsburgh, PA, 5Medical University of South Carolina, Charleston, SC, 6University of Michigan, Ann Arbor, MI, 7University Medical Center - Comprehensive Pulmonary Hypertension Center and ILD Clinic Programs // New Orleans Scleroderma and Sarcoidosis Patient Care & Research Centeris, New Orleans, LA, 8UCLA Department of Medicine, Division of Rheumatology, Los Angeles, CA, 9Victoria Shanmugam, MD, Great Falls, VA, 10Hospital for Special Surgery, New York, NY, 11Department of Medicine, Division of Immunology and Rheumatology, Stanford University School of Medicine, Woodside, CA, 12Columbia University, New York, NY, 13National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, 14Cedars-Sinai Medical Center, Los Angeles, CA, 15National Human Genome Research Institute, Bethesda, MD, 16Johns Hopkins University, Baltimore, MD, 17Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, Ellicott City, MD

    Background/Purpose: Individuals with self-identified Black race have a higher incidence of systemic sclerosis (SSc), develop SSc at a younger age, and have a more severe…
  • Abstract Number: 0699 • ACR Convergence 2023

    T Cell Subset Analysis in Patients with Large-Vessel Vasculitis

    Rebecca Kuan1, Christopher Redmond1, McKella Sylvester2, Mary Maclean1, Francoise Meylan1, Marcela Ferrada1, Kaitlin Quinn1 and Peter Grayson3, 1National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, 2Colgate University, Bethesda, MD, 3National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Chevy Chase, MD

    Background/Purpose: Large-vessel vasculitis (LVV) is characterized by granulomatous inflammation of the aorta and its major branches. The two major forms are giant cell arteritis (GCA)…
  • Abstract Number: 0694 • ACR Convergence 2023

    Off-label Use of Biologics in Urticarial Vasculitis: A European Cohort Study

    Lucas Maisonobe1, Anne-Sophie Korganow2, Alvise Berti3, Alban DEROUX4, Nicolas Dupin5, Selim Aractingi5, Giacomo Emmi6, Frédéric Vandergheynst7, Marc Fabre8, Nicolas Kluger9, Marielle Roux8, Noemie Abisror10, Audrey Benyamine11, Giulia Cassone12, Francois Chasset13, Marie-Sylvie Doutre14, Aurelie Foucher15, Caroline Freguin16, Delphine Gobert17, Yannick Gombeir7, José Hernandez-Rodriguez18, Noémie Legouellec19, Anne-Lise Pinault20, Dimitri Titeca-Beauport21, Marie Jachiet22 and Benjamin Terrier23, 1Assistance Publique - Hopitaux de Paris, Paris, France, 2Strasbourg University Hospital, Strasbourg, France, 3University of Trento, Rochester, MN, 4CHU de Grenoble, Grenoble, France, 5CHU Cochin, Paris, France, 6University of Florence, Florence, Italy, 7Université Libre de Bruxelles, Bruxelles, Belgium, 8Centre Hospitalier Pierre Oudot, Bourgoin Jallieu, France, 9Helsinki University Central Hospital, Helsinki, Finland, 10Internal Medicine Saint Antoine Hospital, Paris, France, 11Hopital Nord Marseille, Marseille, France, 12Universita degli Studi di Modena e Reggio, Modena, Italy, 13Dermatology Department, Tenon Hospital, Paris, France, 14CHU Bordeaux, Bordeaux, France, 15CHU de la Réunion, Saint pierre, France, 16Centre Hospitalier Saint-Brieuc, Saint Brieux, France, 17CHU Sant-Atoine, Paris, France, 18Hospital Clinic de Barcelona, Barcelona, Spain, 19Centre Hospitalier de Valenciennes, Valenciennes, France, 20Centre Hospitalier Epinal, Epinal, France, 21CHU Amiens, Amiens, France, 22CHU Saint-Louis, Paris, France, 23Department of Internal Medicine, Hôpital Cochin, AP-HP, Paris, France

    Background/Purpose: Urticarial vasculitis (UV) is characterized by atypical urticarial lesions and leukocytoclastic vasculitis, sometimes with extra-cutaneous manifestations. First-line treatment is often based on colchicine, hydroxychloroquine,…
  • Abstract Number: 0695 • ACR Convergence 2023

    Impact and Cardiovascular Outcomes of Large Vessel Vasculitis in Atrial Fibrillation Hospitalization: A Nationwide Inpatient Database Study

    Sabahat Usmani1, Yanint Raksadawan1, Amro Taha1, Harigopal Sandhyavenu1, Jalpan Patel1, Thatiana Schulze Goni1, Hafiz Muhammad Waqar Younas1 and Irisha Badu2, 1Weiss Memorial Hospital, Chicago, IL, 2Onslow Memorial Hospital, Jacksonville, FL

    Background/Purpose: Atrial fibrillation is the most commonly treated cardiac arrhythmia, associated with significant morbidity and mortality. Recent research is suggestive that autoimmunity and inflammation might…
  • Abstract Number: 0696 • ACR Convergence 2023

    Association of Large-vessel Vasculitis with Inflammatory Bowel Diseases: A European Case-control Study

    Francois Maillet1, Yann Nguyen2, Olivier Espitia3, Laurent Perard4, Carlo Salvarani5, Etienne Rivière6, Cécile-Audrey Durel7, Philippe GUILPAIN8, Luc Mouthon9, Anna Kernder10, Javier Loricera11, Pascal Cohen12, Isabelle Melki13, Claire De Moreuil14, Nicolas Limal15, Arsène Mekinian16, Nathalie Costedoat-Chalumeau17, Nathalie Morel12, Jonathan Boutemy18, Loic Raffray19, Jean-Sébastien Allain20, Valerie Devauchelle21, Isabelle Kone-Paut22, Marc Fabre23, Marie Durel24, Antoine Dossier25, Sébastien Abad26, Marcella Visentini27, Adrien Bigot28, Halil Yildiz29, Olivier Fain30, Maxime Samson31, Guillaume Gondran32, Vered Abitbol1 and Benjamin Terrier33, 1Cochin Hospital, Paris, France, 2Department of Internal Medicine, Hôpital Beaujon, AP-HP, Clichy, France., Montmorency, France, 3CHU de Nantes, Nantes, France, 4CH Saint Hoseph Saint Luc, Lyon, France, 5Azienda USL -IRCCS di Reggio Emilia and Università di Modena e Reggio Emilia, Reggio Emilia, Italy, 6CHU Bordeaux, Bordeaux, France, 7CHU Lyon, Lyon, France, 8University of Montpellier, Montpellier, France, 9Hopital Cochin - Paris University, Paris, France, 10HHU Düsseldorf, Düsseldorf, Germany, 11Hospital Universitario Marqués de Valdecilla, Santander, Spain, 12CHU Cochin, Paris, France, 13Hopital Robert Debré, Sérurier, France, 14CHU de Brest, Brest, France, 15AP-HP, Créteil, France, 16Department of Internal Medicine, Hôpital Saint-Antoine, AP-HP, Paris, France, 17Inserm DR Paris 5, Paris, France, 18CHU de Caen, Caen, France, 19CHU la Réunion, La Réunion, France, 20CHU Rennes, Rennes, France, 21UBO, Brest, France, 22Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAIA, Bicêtre Hospital, APHP, University of Paris Saclay, Paris, France, 23CH Bourgoin-Jallieu, Bourgoin-Jallieu, France, 24CH Metz, Metz, France, 25AP-HP, Paris, France, 26Avicenne Hospital, Bobigny, France, 27Rome Hospital, Rome, France, 28Tours Hospital, Tours, France, 29Saint Luc Hospital, Bruxelles, Belgium, 30Hopital SAINT ANTOINE APHP, Paris, France, 31Department of Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France, 32Limoges Hospital, Limoges, France, 33Department of Internal Medicine, Hôpital Cochin, AP-HP, Paris, France

    Background/Purpose: The association of large vessel vasculitis (LVV), whether Takayasu arteritis (TA) or giant cell arteritis (GCA), with inflammatory bowel disease (IBD) is a rare…
  • Abstract Number: 0708 • ACR Convergence 2023

    NFKB1 and NFKBIA: Relevant Players in the Pathogenesis of IgA Vasculitis?

    Joao Carlos Batista-Liz1, Verónica Pulito-Cueto1, María Sebastián Mora-Gil1, Belén Sevilla-Pérez2, María Teresa Leonardo3, Norberto Ortego-Centeno4, Ana Peñalba3, Javier Narvaez5, luis martin penagos6, Emilio Rodrigo6, Lara Belmar-Vega6, Cristina Gomez-Fernandez7, Luis Caminal-Montero8, Paz Collado9, Miren Uriarte-Ecenarro10, Esther Vicente Rabaneda11, Esteban Rubio12, Manuel León Luque12, Juan María Blanco-Madrigal13, Eva Galindez-Agirregoikoa14, Santos Castañeda15, Miguel A Gonzalez-Gay16, Ricardo Blanco17 and Raquel López-Mejías1, 1Rheumatology Department, Immunopathology Group, Hospital Universitario Marqués de Valdecilla-IDIVAL, Santander, Spain, 2Division of Pediatrics, Hospital Universitario San Cecilio, Granada, Spain, 3Division of Pediatrics, Hospital Universitario Marqués de Valdecilla, Santander, Spain, 4Medicine Department, Universidad de Granada, Granada, Spain, 5Hospital Universitario de Bellvitge, Barcelona, Spain, 6Division of Nephrology, Immunopathology Group, Hospital Universitario Marqués de Valdecilla-DIVAL, Santander, Spain, 7Division of Dermatology,Immunopathology Group, Hospital Universitario Marqués de Valdecilla-IDIVAL, Santander, Spain, 8Internal Medicine Department, Hospital Universitario Central de Asturias, Instituto de Investigación Sanitaria del Principado de Asturias (ISPA), Oviedo, Spain, 9Division of Rheumatology, Hospital Universitario Severo Ochoa, Madrid, Spain, 10Division of Rheumatology, Hospital Universitario de La Princesa, IIS-Princesa, Madrid, Spain, 11Rheumatology, Hospital Universitario de La Princesa, Madrid, Spain, 12Division of Rheumatology, Hospital Universitario Virgen del Rocío, Sevilla, Spain, 13Division of Rheumatology, Hospital Universitario de Basurto, Bilbao, Spain, 14Basurto University Hospital, Bilbao, Spain, 15Hospital Universitario de la Princesa, Madrid, Spain, 16IDIVAL and School of Medicine, UC, Santander; Department of Rheumatology, IIS-Fundación Jiménez Díaz, Madrid, Santander, Spain, 17Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain

    Background/Purpose: Immunoglobulin A Vasculitis (IgAV) is a B-cell-mediated inflammatory disease. NF-kappa B (NF-kB) plays a key role in autoimmunity and inflammation1. In this regard, the…
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