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: 1553 • ACR Convergence 2023
Central Nervous System Involvement and Mimickers in ANCA Associated Vasculitis
Yeliz Yagiz Ozogul¹, Sinem Nihal Esatoglu², Murat Ozogul³, Osman Kizilkilic⁴, Yesim Ozguler⁵, Ugur Uygunoglu⁶, Vedat Hamuryudan² and Gulen Hatemi², ¹Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Department of Internal Medicine, Istanbul, Turkey, ²Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, ³Haydarpasa Numune Training and Research Hospital, Department of Radiology, Istanbul, Turkey, ⁴Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Department of Radiology, Istanbul, Turkey, ⁵Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Department of Internal Medicine, Division of Rheumatology, İstanbul, Turkey, ⁶Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Department of Neurology, Istanbul, Turkey
Background/Purpose: Central nervous system (CNS) involvement is rare in ANCA associated vasculitis (AAV). On the other hand, AAV patients may develop complications or other conditions…
Abstract Number: 1584 • ACR Convergence 2023
Effect of Trimethoprim Sulfamethoxazole Prophylaxis on Infections During Treatment of Granulomatosis with Polyangiitis with Rituximab: A Population-Based Study
Arielle Mendel¹, Hassan Behlouli², Evelyne Vinet¹, Jeffrey Curtis³ and Sasha Bernatsky², ¹McGill University Health Centre, Montréal, QC, Canada, ²Research Institute of the McGill University Health Centre, Montreal, QC, Canada, ³University of Alabama at Birmingham, Birmingham, AL
Background/Purpose: Infections during treatment of ANCA-associated vasculitis (AAV) lead to excess mortality. Trimethoprim sulfamethoxazole (TMP-SMX), recommended for pneumocystis jirovecii pneumonia (PJP) prophylaxis, has broad antimicrobial…
Abstract Number: 2375 • ACR Convergence 2023
Clinical Characteristics of ANCA-associated Vasculitis with High Levels of Serum Interleukin 7
Shoichi Fukui¹, Haruna Matsuo², Kanako Kojima³, Shota Kurushima¹, Tohru Michitsuji⁴, Yoshika Tsuji⁵, Toshimasa Shimizu³, Masataka Umeda³, Remi Sumiyoshi⁵, Takashi Igawa¹, Tomohiro Koga⁵, Shin-ya Kawashiri¹, Naoki Iwamoto¹, Tomoki Origuchi¹ and Atsushi Kawakami¹, ¹Nagasaki University, Nagasaki, Japan, ²Nagasaki University, Nagasaki City, Japan, ³Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan, ⁴Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Medical Sciences, Nagasaki, Japan, ⁵Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University, Nagasaki, Japan
Background/Purpose: Interleukin 7 (IL-7) is a hematopoietic growth factor produced by stromal cells in the bone marrow and thymus, which is indispensable in maintaining immune…
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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