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Abstracts tagged "Granulomatosis with Polyangiitis (GPA)"

  • Abstract Number: 2486 • ACR Convergence 2024

    Glucocorticoids May Mitigate the Risk of Avacopan-Induced Liver Dysfunction in ANCA-Associated Vasculitis: Data from a Multicenter Observational Study in Japan

    Tomohisa Uchida1, Shoichi Fukui1, Naoki Iwamoto1, Ayaka Umetsu2, Momoko Okamoto2, Keita Fujikawa2, Akinari Mizokami2, Takuya Tomokawa3, Kazusato Hara3, Yoshiro Horai3 and Atsushi Kawakami4, 1Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan, 2Department of Rheumatology, Japan Community Healthcare Organization (JCHO) Isahaya General Hospital, Isahaya, Japan, 3Department of Rheumatology, Sasebo City General Hospital, Sasebo, Japan, 4Nagasaki University, Nagasaki, Japan

    Background/Purpose: Avacopan, a C5a receptor inhibitor, has emerged as a novel treatment for ANCA-associated vasculitis (AAV), providing an alternative to glucocorticoids1. Despite its promising potential,…
  • Abstract Number: 2488 • ACR Convergence 2024

    Patient Characteristics and Treatment Patterns Before and After Initiation of Avacopan in the United States: An Early View Based on a Claims Database Analysis

    Sushmitha Inguva1, Pallavi Rane1, Darcy Trimpe1, Sam Oh1, Jasjit Multani2, Hsiu-Ching Chang2, Marie Yasuda2, Chi-Chang Chen2, Duvuru Geetha3, Peter Merkel4 and Zachary Wallace5, 1Amgen Inc., Thousand Oaks, CA, 2IQVIA, Wayne, PA, 3Johns Hopkins University, Baltimore, MD, 4University of Pennsylvania, Philadelphia, PA, 5Massachusetts General Hospital, Newton, MA

    Background/Purpose: The United States (US) Food and Drug Administration approved avacopan to treat adults with severe active granulomatosis with polyangiitis or microscopic polyangiitis in October…
  • Abstract Number: 2689 • ACR Convergence 2024

    Characterization of Alpha-1 Antitrypsin Function in ANCA-Associated Vasculitis

    Lynn Fussner1, Ivan Bilic2, Carol McAlear3, David Cuthbertson4, Jie Cheng5, Elise Chen5, Markus Weiller2, Ulrich Specks6 and Peter Merkel3, and for the Vasculitis Clinical Research Consortium, 1The Ohio State University, Columbus, OH, 2Takeda Pharmaceuticals, Vienna, Austria, 3University of Pennsylvania, Philadelphia, PA, 4University of South Florida, Tampa, FL, 5Takeda Pharmaceuticals, Cambridge, MA, 6Mayo Clinic, Rochester, MN

    Background/Purpose: Two separate genome-wide association studies demonstrated that polymorphisms in SERPINA1, encoding serine protease inhibitor alpha-1 antitrypsin (A1AT), are associated with increased risk of developing…
  • Abstract Number: 0697 • ACR Convergence 2024

    Association of ANCA-Associated Vasculitis and Development of SSc and SSc-Associated Antibodies: Impact of Pulmonary Arterial Hypertension

    Brett Dinner1, Ahmed Abdelmaksoud2, Ann Igoe3, Taylor Viggiano4 and Vivek Nagaraja5, 1Creighton University, Paradise Valley, AZ, 2University of California, Riverside, Riverside, CA, 3Flow, Tempe, AZ, 4Mayo Clinic, Phoenix, 5Mayo Clinic Arizona, Scottsdale, AZ

    Background/Purpose: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a spectrum of autoimmune disorders characterized by necrotizing small-to-medium vessel inflammation and circulating ANCAs. Represented by granulomatosis…
  • Abstract Number: 0719 • ACR Convergence 2024

    Risk of Vertebral Fracture in Individuals with Granulomatosis with Polyangiitis and End-Stage Renal Disease

    Joanna El Hajj1, Jennifer Waller1, Yagni Patel1, Wendy Bollag1, Stephanie Baer2 and Rachel Elam3, 1Augusta University, Augusta, GA, 2VA Augusta Healthcare System, Augusta, GA, 3Augusta University, Evans, GA

    Background/Purpose: End-stage renal disease (ESRD) secondary to vasculitis is linked to a higher risk of vertebral fractures compared to other causes of ESRD. It remains…
  • Abstract Number: 0721 • ACR Convergence 2024

    Does Serial ANCA Testing Help Predict the Risk of Relapse in Granulomatosis with Polyangiitis? A Single-center Cohort Study

    Xavier Puéchal1, Michele Iudici1, Elodie Perrodeau2, Claire Goulvestre3, Pascal cohen4, Alexis Régent1, Luc Mouthon5, Loïc Guillevin4, Raphael Porcher6 and Benjamin Terrier7, 1National Referral Center For Rare Systemic Autoimmune Diseases, Paris, France, 2Hôtel-Dieu, Paris, France, 3Laboratoire d'immunologie, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France, 4National Referral Center For Rare Systemic Autoimmune Diseases, Paris, Ile-de-France, France, 5Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes et Autoinflammatoires Rares d'Ile de France de l’Est et de l’Ouest, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, 6Department of Rheumatology, Bicêtre AP-HP Hôpital, Université Paris-Saclay, Paris, Ile-de-France, France, 7Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France

    Background/Purpose: ANCA-associated vasculitis (AAV) is a frequently relapsing disease for which a meta-analysis concluded that an increase or persistence of ANCA during remission is only…
  • Abstract Number: 0774 • ACR Convergence 2024

    A Multicenter, Randomized, Controlled Trial to Evaluate the Effects of Low-dose Glucocorticoids Compared to Stopping Glucocorticoids to Maintain Remission of Granulomatosis with Polyangiitis: The TAPIR Trial

    Peter Merkel1, Christian Pagnoux2, Nader Khalidi3, Ulrich Specks4, Curry Koening5, Carol Langford6, Larry Moreland7, Paul Monach8, Jason Springer9, Shubhasree Banerjee1, Simon Carette10, Rennie Rhee1, Medha Soowamber11, Kenneth Warrington4, Renee Borchin12, Cristina Burroughs13, Carol McAlear1, David Cuthbertson13 and Jeffrey Krischer13, 1University of Pennsylvania, Philadelphia, PA, 2Mount Sinai Hospital, Toronto, ON, Canada, 3McMaster University, Hamilton, ON, Canada, 4Mayo Clinic, Rochester, MN, 5University of Texas Dell Medical School, Austin, TX, 6Cleveland Clinic, Moreland Hills, OH, 7University of Colorado, Denver, CO, 8VA Boston Healthcare System, Boston, MA, 9Vanderbilt University Medical Center, Franklin, TN, 10Mount Sinai Hosptial, Toronto, ON, Canada, 11University of Toronto, Toronto, ON, Canada, 12University of South Florida, Tampa, 13University of South Florida, Tampa, FL

    Background/Purpose: Use of low-dose glucocorticoids to maintain remission in patients with granulomatosis with polyangiitis (GPA) remains controversial.  Additionally, there is no consensus on how to…
  • Abstract Number: 0823 • ACR Convergence 2024

    A Randomized, Double-Blind, Placebo-Controlled Trial of Abatacept for the Treatment of Relapsing, Non-Severe, Granulomatosis with Polyangiitis

    Carol Langford1, Nader Khalidi2, Jason Springer3, Marcia Friedman4, Bernhard Hellmich5, Christian Pagnoux6, Natasha Dehghan7, Ora Singer8, Curry Koening9, Yih Chang Lin10, Paul Monach11, Larry Moreland12, Aurore Fifi-Mah13, Oliver Flossmann14, Lindsy Forbess15, Peter Lanyon16, Eamonn Molloy17, Ulrich Specks18, Robert Spiera19, Elaine Yacyshyn20, Carol McAlear21, Cristina Burroughs10, Rachel Jones22, Rennie Rhee21, Rula A. Hajj-Ali23, Kenneth Warrington18, David Cuthbertson10, Jeffrey Krischer10, David Jayne22 and Peter Merkel21, and the Vasculitis Clinical Research Consortium and the European Vasculitis Society, 1Cleveland Clinic, Moreland Hills, OH, 2McMaster University, Hamilton, ON, Canada, 3Vanderbilt University Medical Center, Franklin, TN, 4Oregon Health and Science University, Portland, OR, 5Medius Kliniken, Kirchheim unter Teck, Germany, 6Mount Sinai Hospital, Toronto, ON, Canada, 7University of British Columbia - Vancouver, Vancouver, BC, Canada, 8University of Michigan, Huntington Woods, MI, 9University of Texas Dell Medical School, Austin, TX, 10University of South Florida, Tampa, FL, 11VA Boston Healthcare System, Boston, MA, 12University of Colorado, Denver, CO, 13University of Calgary, Calgary, AB, Canada, 14Royal Berkshire Hospital, Reading, United Kingdom, 15Cedars-Sinai Medical Center, Los Angeles, CA, 16University of Nottingham, Nottingham, United Kingdom, 17St Vincent's University Hospital, Dublin, Ireland, 18Mayo Clinic, Rochester, MN, 19Scleroderma, Vasculitis, and Myositis Center, Hospital for Special Surgery, Weill Cornell Medical College, New York, NY, 20University of Alberta, Edmonton, AB, Canada, 21University of Pennsylvania, Philadelphia, PA, 22University of Cambridge, Cambridge, United Kingdom, 23Cleveland Clinic, Cleveland, OH

    Background/Purpose: Granulomatosis with polyangiitis (GPA) is a small-vessel vasculitis associated with frequent relapses. Following encouraging results from an open-label study, a randomized, double-blind, placebo-controlled trial…
  • Abstract Number: 1592 • ACR Convergence 2024

    Overlapping Forms of Eosinophilic Granulomatosis with Polyangiitis and Granulomatosis with Polyangiitis: Presentation, Management and Outcomes

    Federica Pallotti1, Camille Mettler2, Roberto Padoan3, Francesca Regola4, Franco Franceschini5, Sergey Moiseev6, Pavel Novikov7, Mario Andrea Piga8, Gianluca Moroncini9, Silke Brix10, Abdul Hadi Kafagi11, Samuel Deshayes12, Achille Aouba12, Julien Campagne13, Paolo Delvino14, Jan Willem Cohen Tervaert15, Luisa Brussino16, Martin Michaud17, Nils Venhoff18, Federico Alberici19, Claudia Iannone20, Sophie Rosenstingl21, Marin Moutel22, Jean-Marc Galempoix23, Vincent Cottin24, Clara Jaccard25, Diane Riehl26, Paul Legendre27, Anne-Claire Billet28, Paola Parronchi29, Luca Quartuccio30, Vítor Silvestre Teixeira31, Allyson Egan32, David Jayne32, Enrico Tombetti33, Marco Caminati34, Christian Pagnoux35, Alexis Régent36, Marc Ruivard37, Loïc Guillevin38, Xavier Puéchal36 and Benjamin Terrier39, and the French Vasculities Study Group and European EGPA Study Group, 1Internal Medicine, Centre Hospitalier Universitaire de Caen, Caen, France, 2Département de Médecine Interne, Centre de Référence National pour les maladies auto-immunes systémiques rares, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France, 3Department of Medicine DIMED, Division of Rheumatology, University of Padua, Padua, Italy, 4Unit of Rheumatology and Clinical Immunology, ASST Spedali Civili Brescia and University of Brescia, Brescia, Italy, 5Scleroderma Unit, Rheumatology and Clinical Immunology Unit, ERN ReCONNET, ASST Spedali Civili, Brescia, Italy, Brescia, Italy, 6Tareev Clinic of Internal Diseases, Sechenov First Moscow State Medical University, Moscow, Russia, 7Sechenov First Moscow State Medical University, Moscow, Russia, 8Postgraduate School of Allergy and Clinical Immunology, Università Politecnica delle Marche, Ancona, Italy, 9Department of Clinical and Molecular Sciences, Marche Polytechnic University & Department of Internal Medicine, Marche University Hospital, Ancona, Italy, 10Division of Cell Matrix Biology and Regenerative Medicine, School of Biological Sciences, The University of Manchester, Manchester, United Kingdom, 11Division of Cardiovascular Sciences, School of Medical Sciences, University of Manchester, Manchester, United Kingdom, 12Service d'immunologie clinique-médecine interne, CHU de Caen Normandie, Caen, France, 13Hôpital Robert Schuman - Competence center for autoimmune diseases, Internal Medicine, Metz, France, 14University of Milano-Bicocca, Milan, Milan, Italy, 15University of Alberta, Edmonton, Canada, 16SSDDU Immunologia Clinica ed Allergologia, AO Mauriziano, Turin, Italy, 17Department of Internal Medicine, Clinique Saint-Exupery, Toulouse, France., Toulouse, France, 18Medical Center - University of Freiburg, Internal Medicine, Department of Rheumatology and Clinical Immunology, Freiburg, Germany, 19Nephrology Unit, ASST Spedali Civili di Brescia, Brescia, Italy, 20Department of Rheumatology, University of Milan, and Rheumatology Department, ASST Pini-CTO, Milan, Italy, 21Service Médecine Interne, Centre hospitalier intercommunal Compiègne Noyon, Compiègne Noyon, France, 22Department of Internal Medicine, Infectious Diseases, and Clinical Immunology, Reims University Hospital, Reims, France, 23J.M. Galempoix, MD, Department of Internal Medicine, Nord Ardennes Hospital, Charleville-Mézières, France, 24Hôpital Louis Pradel, Centre de Référence des Maladies Pulmonaires Rares, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Lyon, France, Lyon, France, 25Gaston Bourret Hospital, Nouméa, New Caledonia, 26Centre hospitalier intercommunal Toulon- La Seyne Sur Mer, Toulon, France, 27Service Médecine Interne et Polyvalente, Centre Hospitaliers Le Mans, Le Mans, France, 28Department of Internal Medicine, Édouard Herriot Hospital, Hospices Civils de Lyon, Lyon, France, 29University of Florence, Florence, Florence, Italy, 30Division of Rheumatology, Department of Medicine, University of Udine, Udine, Italy, 31Department of Rheumatology, Faro Hospital, Algarve, Portugal, 32University of Cambridge, Cambridge, United Kingdom, 33Internal Medicine and Rheumatology, Department of Biomedical and Clinical Sciences, Sacco and Fatebenefratelli Hospitals, Milan, Italy, Milan, Italy, 34Department of Medicine, Asthma, Allergy and Clinical Immunology Section, University of Verona, Verona, Italy, 35Mount Sinai Hospital, Toronto, ON, Canada, 36National Referral Center For Rare Systemic Autoimmune Diseases, Paris, France, 37Internal Medicine Department, Estaing University Hospital, CHU Clermont-Ferrand, Clermonnt-Ferrand, Italy, 38National Referral Center For Rare Systemic Autoimmune Diseases, Paris, Ile-de-France, France, 39Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France

    Background/Purpose: ANCA-associated vasculitis (AAV) include granulomatosis with polyangiitis (GPA), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (EGPA). Although these entities are often easily distinguished in…
  • Abstract Number: 1608 • ACR Convergence 2024

    Interstitial Lung Disease in ANCA-associated Vasculitis: A Retrospective Study of Clinical Characteristics, Radiographic Features, and Outcomes

    Janelle Castellino1, Matas Orentas2, Joshua Moran3, Joshlean Fair1, Yanyu Zhang4 and Aleksandra Bukiej5, 1Rush University Medical Center, Chicago, IL, 2RUMC, Chicago, IL, 3Rush University Medical Center - IM Residency, Chicago, IL, 4Rush University Medical Center, Chicago, 5Rush University Medical Center, River Forest, IL

    Background/Purpose: Interstitial lung disease (ILD) is a significant manifestation of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and…
  • Abstract Number: 2181 • ACR Convergence 2024

    Large Pediatric Cohort with ANCA-associated Vasculitis at a Single Institution: Patient Characteristics, Clinical Course, and Outcomes

    Jessica Bloom1, Anna Monley2, Sarah Reingold3, Robert Fuhlbrigge1 and Peter Merkel4, 1University of Colorado, Denver, CO, 2University of Colorado, Aurora, CO, 3Denver Health, Denver, CO, 4University of Pennsylvania, Philadelphia, PA

    Background/Purpose: Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is an understudied, chronic inflammatory disease in children with significant morbidity and mortality. There are few large pediatric cohorts…
  • Abstract Number: 2478 • ACR Convergence 2024

    Avacopan versus a Prednisone Taper in Patients with ANCA-Associated Vasculitis Without Kidney Involvement in a Phase 3 Trial

    Christian Pagnoux1, Antoine Neel2, Sarah Bray3, Rachel E. Gurlin4, Darcy Trimpe5, David Jayne6 and Peter Merkel7, and ADVOCATE Study Group, 1Mount Sinai Hospital, Toronto, ON, Canada, 2Department of Internal Medicine, CHU Nantes, Nantes, France, 3Amgen Ltd, Cambridge, United Kingdom, 4Amgen Inc., Mountain View, CA, 5Amgen Inc., Thousand Oaks, CA, 6University of Cambridge, Cambridge, United Kingdom, 7University of Pennsylvania, Philadelphia, PA

    Background/Purpose: Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are subtypes of ANCA-associated vasculitis that frequently involve the kidneys. However, a subset of patients with…
  • Abstract Number: 2480 • ACR Convergence 2024

    Avacopan versus a Prednisone Taper in Patients with ANCA-Associated Vasculitis and Ear, Nose, or Throat Involvement in a Phase 3 Trial

    Robert Spiera1, Robert Lebovics2, Sarah Bray3, Rachel E. Gurlin4, David Jayne5 and Peter Merkel6, and ADVOCATE Study Group, 1Scleroderma, Vasculitis, and Myositis Center, Hospital for Special Surgery, Weill Cornell Medical College, New York, NY, 2Hackensack Meridian University Health System, Hackensack, NJ, 3Amgen Ltd, Cambridge, United Kingdom, 4Amgen Inc., Mountain View, CA, 5University of Cambridge, Cambridge, United Kingdom, 6University of Pennsylvania, Philadelphia, PA

    Background/Purpose: In the phase 3 ADVOCATE trial, 45.2% and 42.1% of patients in the avacopan and prednisone taper groups, respectively, had active ear, nose, or…
  • Abstract Number: 2485 • ACR Convergence 2024

    Orbital Pseudotumor in Granulomatosis with Polyangiitis. Interim Analysis of Clinical Outcomes from a European Collaborative Observational Study

    Luca Moroni1, Gabriele Gallina2, Marco Lanzillotta2, Sara Maggioni2, Giovanni Benanti2, Adriana Cariddi2, Paolo Delvino3, Sara Monti4, Alessandra Milanesi5, Filippo Fagni6, Marco Fornaro7, Camille Taille8, Elena Treppo9, Grégory Pugnet10, Emanuele Chiara11, Roberto Padoan12, Luca Iorio13, Federica Davanzo12, Eleonora Fiorin14, Alvise Berti15, Roberto Bortolotti16, Sergey Moiseev17, Pavel Novikov17, Askin Ates18, Ahmet Omma19, Tahir Saygin Ogut20, Gozde Sevgi Kart-Bayram21, Tuba Demirci22, Riza Can Kardas23, Ömer Karadağ21, Marco Matucci-Cerinic24 and Lorenzo Dagna25, 1IRCCS San Raffaele Scientific Institute, Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), Milano, Italy, Milano, Italy, 2IRCCS Ospedale San Raffaele, Milano, Italy, 3University of Milano-Bicocca, Milan, Milan, Italy, 4IRCCS Istituto Auxologico Italiano, Milan, Italy, 5Unit of Rheumatology, Fondazione IRCCS Policlinico San Matteo and University of Pavia, Pavia, Italy, 6Department of Internal Medicine 3 - Rheumatology and Immunology, Friedrich-Alexander University (FAU) Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, Deutsches Zentrum für Immuntherapie (DZI), Friedrich Alexander University Erlangen-Nuremberg and Universitätsklinikum Erlangen, Erlangen, Germany, Erlangen, Germany, 7Università di Bari, Bari, Italy, 8Bichat Hospital, Paris, France, 9Division of Rheumatology, Department of Medicine (DMED), University of Udine, Udine, Italy, Udine, Italy, 10Toulouse Rangueil University Hospital, Toulouse, France, 11Ospedale Careggi, Firenze, Italy, 12Azienda Ospedaliera-Universitaria di Padova, Padova, Italy, 13Rheumatology Unit, Department of Medicine DIMED, University of Padua, Padova, Italy, Padova, Italy, 14Azienda Ospedaliera-Universitaria di Padova, Padova, 15Rheumatology Unit Santa Chiara Hospital of Trento, Trento, Italy, 16Rheumatology Unit Santa Chiara Hospital of Trento, Trento, 17Sechenov First Moscow State Medical University, Moscow, Russia, 18Ankara University, Ankara, Turkey, 19Ankara Bilkent City Hospital, Ankara, Turkey, 20Akdeniz University, Antalya, Turkey, 21Hacettepe University School of Medicine, Ankara, Turkey, 22Dokuz Eylül University, Izmir, Turkey, 23Gazi University, Ankara, Turkey, 24University San Raffaele Milano, Milano, Milan, Italy, 25Ospedale San Raffaele, Milano, Italy

    Background/Purpose: Orbital Pseudotumor (OPT) is a rare organ-threatening manifestation of Granulomatosis with Polyangiitis (GPA), with severe prognosis and historically refractory to most treatments. This is…
  • Abstract Number: 2376 • ACR Convergence 2023

    Eosinophil Activation as a Biomarker for Discriminating Active and Remission Phase in ANCA-Associated Vasculitis

    Teppei Hashimoto1, Kazuteru Noguchi2, Yuko Minagawa2, Takeo Abe2, Masao Tamura2, Tetsuya Furukawa1, Naoto Azuma2 and Kiyoshi Matsui1, 1Hyogo Medical University, Nishinomiya, Japan, 2Hyogo Medical University, School of Medicine, Nishinomiya, Japan

    Background/Purpose: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a life-threatening disease requiring complex management due to a lack of suitable biomarkers. Elevated or persistently positive…
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