Abstracts tagged "Granulomatosis with Polyangiitis (GPA)"

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: 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: 0703 • ACR Convergence 2023
Cardiovascular Disease-Related Mortality in Primary Systemic Vasculitis
Alicia Rodriguez-Pla, Sierra Pacific Arthritis and Rheumatology Centers, Fresno, CA
Background/Purpose: Cardiovascular disease (CVD) is the leading cause of death in patients with the most common subtypes of primary systemic vasculitides. We aimed to estimate…
Abstract Number: 0725 • ACR Convergence 2023
Real-life Use of the PEXIVAS Reduced-dose Glucocorticoid Regimen in Granulomatosis with Polyangiitis and Microscopic Polyangiitis
Sophie Nagle¹, Yann Nguyen², Xavier Puéchal³, Dimitri Titeca-Beauport⁴, Thomas Crépin⁵, Rafik Mesbah⁶, Idris Boudhabhay⁷, Gregory Pugnet⁸, Juliette Woessner⁹, Roderau Outh¹⁰, Céline Lebas¹¹, Antoine Néel¹², alexandre Karras¹³, Eric Hachulla¹¹, Benjamin Subran¹⁴, Philippe Kerschen¹⁵, Mathieu Gerfaud-Valentin¹⁶, Stéphane Vinzio¹⁷, Tiphaine Goulenok¹⁸, Raphael Borie¹⁹, Sébastien Humbert²⁰, Yurdagul Uzunhan²¹, Sarah Melboucy-Belkhir²², Jean-Baptiste Gouin²³, Mary-Jane Guerry²⁴ and Benjamin Terrier²⁵, ¹AP-HP Cochin Hospital, Paris, France, ²Department of Internal Medicine, Hôpital Beaujon, AP-HP, Clichy, France., Montmorency, France, ³National Referral Center for Rare Systemic Autoimmune Diseases, Paris, France, ⁴CHU Amiens, Amiens, France, ⁵Amiens University Hospital, Amiens, France, ⁶Boulogne Hospital (CH), Boulogne, France, ⁷Necker University Hospital, Paris, France, ⁸CHU Toulouse Rangueil Service de Medecine Interne et Immunologie Clinique, Toulouse, France, ⁹Avignon Hospital (CH), Avignon, France, ¹⁰Perpignan Hospital (CH), Perpignan, France, ¹¹Lille University Hospital, Lille, France, ¹²CHU Nantes, Nantes, France, ¹³HEGP - APHP, Paris, France, ¹⁴Croix Saint Simon Hospital, Paris, France, ¹⁵Department of Neurology, Luxembourg Hospital Center, Luxembourg City, Luxembourg, ¹⁶Lyon University Hospital, Lyon, France, ¹⁷Grenoble Hospital (CH), Grenoble, France, ¹⁸Assistance Publique Hopitaux de Paris, Paris, France, ¹⁹Bichat-Claude Bernard, Universite de Paris, Paris, France, ²⁰Besançon University Hospital, Besançon, France, ²¹AP-HP, Bobigny, France, ²²Saint Quentin Hospital, Saint-Quentin, France, ²³Vannes Hospital (CH Bretagne Atlantique), Vannes, France, ²⁴Valenciennes Hospital (CH), Valenciennes, France, ²⁵Department of Internal Medicine, Hôpital Cochin, AP-HP, Paris, France
Background/Purpose: Glucocorticoids (GCs) in combination with rituximab (RTX) or cyclophosphamide are the cornerstone of treatment for patients with severe granulomatosis with polyangiitis (GPA) and microscopic…
Abstract Number: 0857 • ACR Convergence 2023
Change in Albuminuria in Patients with ANCA-Associated Vasculitis Treated with Avacopan
Duvuru Geetha¹, Frank Cortazar², alexandre Karras³, Annette Bruchfeld⁴, Huibin Yue⁵, Peter Merkel⁶ and David Jayne⁷, ¹Johns Hopkins University, Baltimore, MD, ²New York Nephrology, Watervliet, NY, ³HEGP - APHP, Paris, France, ⁴Karolinska Institutet, Stockholm, Sweden, ⁵Amgen, Inc., Thousand Oaks, CA, ⁶University of Pennsylvania, Philadelphia, PA, ⁷University of Cambridge, Cambridge, United Kingdom
Background/Purpose: Urinary albumin:creatinine ratio (UACR) is an important biomarker of active glomerulonephritis, a common complication of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). In most glomerular…
Abstract Number: 1535 • ACR Convergence 2023
Epidemiology and Treatment Outcome of ANCA-associated Vasculitis in South Korea: A Nationwide, Population-based Cohort Study
Ji In Jung¹, Jun Won Park², Song Jihun³, Seulggie Choi², Sun Jae Park³, Sang Min Park² and Eun Young Lee⁴, ¹Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, South Korea, ²Seoul National University Hospital, Seoul, South Korea, ³Seoul National University Graduate School, Seoul, South Korea, ⁴Seoul National University College of Medicine, Seoul, South Korea
Background/Purpose: Previous studies on AAV have shown that the epidemiology and clinical features of GPA and MPA differ among countries. Considering their high mortality and…

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