Abstracts tagged "Granulomatosis with Polyangiitis (GPA)"

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: 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: 0685 • ACR Convergence 2023
Efficacy and Safety of Avacopan in Patients with ANCA-Associated Vasculitis Receiving Rituximab in a Phase 3 Trial
Duvuru Geetha¹, Anisha Dua², Huibin Yue³, Carlo Salvarani⁴, David Jayne⁵ and Peter Merkel⁶, ¹Johns Hopkins University, Baltimore, MD, ²Northwestern University, Chicago, IL, ³Amgen, Inc., Thousand Oaks, CA, ⁴Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy, ⁵University of Cambridge, Cambridge, United Kingdom, ⁶University of Pennsylvania, Philadelphia, PA
Background/Purpose: The randomized, double-blind, double-dummy, controlled Phase 3 ADVOCATE trial tested whether avacopan, an oral selective C5a receptor inhibitor approved for the treatment of ANCA-associated…
Abstract Number: 2492 • ACR Convergence 2023
The Impact of Neurologic Involvement in ANCA-Associated Vasculitis on Self-Reported Health-Related Quality of Life
Rula Hajj-Ali¹, Chao Zhang², Renee Borchin³, Jenn Gordon⁴, David Cuthbertson³, Carol McAlear⁵, Christine Yeung⁵, David Badenoch⁶, Cristina Burroughs³ and Peter Merkel⁵, ¹Cleveland Clinic, Cleveland, OH, ²Cleveland Clinic Foundation, Cleveland, OH, ³University of South Florida, Tampa, FL, ⁴Vasculitis Patient Powered Research Network (VPPRN), Philadelphia, PA, ⁵University of Pennsylvania, Philadelphia, PA, ⁶Vasculitis Patient Powered Research Network (VPPRN), Los Angeles, CA
Background/Purpose: Neurologic involvement (NI) is a common manifestation in patients with ANCA-associated vasculitis (AAV) and can lead to chronic pain and disability. This project assessed…
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: 0689 • ACR Convergence 2023
Use of Avacopan in Patients with ANCA-associated Vasculitis and Estimated Glomerular Filtration Rate < 15 mL/min/1.73m2
Bryce Barr, Kim Cheema, Aurore Fifi-Mah, Stephanie Garner, Louis Girard and Jeffrey Ma, University of Calgary, Calgary, AB, Canada
Background/Purpose: Glomerulonephritis represents a severe manifestation of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) and is associated with substantial morbidity and mortality. Therefore, maximal recovery…

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