Abstracts tagged "ANCA associated vasculitis"

Abstract Number: 2481 • ACR Convergence 2024
General, Nervous System, Eye, and Skin Involvement in the Phase 3 Trial of Avacopan for the Treatment of ANCA-Associated Vasculitis
Rula A. Hajj-Ali¹, Duvuru Geetha², Raashid Luqmani³, Christian Pagnoux⁴, Darcy Trimpe⁵, David Jayne⁶ and Peter Merkel⁷, and ADVOCATE Study Group, ¹Cleveland Clinic, Cleveland, OH, ²Johns Hopkins University, Baltimore, MD, ³University of Oxford, Abingdon, United Kingdom, ⁴Mount Sinai Hospital, Toronto, ON, Canada, ⁵Amgen Inc., Thousand Oaks, CA, ⁶University of Cambridge, Cambridge, United Kingdom, ⁷University of Pennsylvania, Philadelphia, PA
Background/Purpose: The most common types of ANCA-associated vasculitis, granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), can affect a range of organs, including the kidneys,…
Abstract Number: 2651 • ACR Convergence 2024
Vasculitis Associated with VEXAS Syndrome
Megan Sullivan¹, Kambiz Kalantari², Carolyn Mead-Harvey³, Yael Kusne⁴, Mrinal Patnaik², Abhishek Mangaonkar², Ronald Go², Daniel Montes², Kaaren Reichard², Horatiu Olteanu², Melanie Bois², Alexander Hines², Julio Sartori-Valinotti⁵, Kenneth Warrington² and Matthew Koster², ¹Mayo Clinic Arizona, Scottsdale, AZ, ²Mayo Clinic, Rochester, MN, ³Mayo Clinic, Scottsdale, AZ, ⁴Mayo Clinic, Phoenix, AZ, ⁵Mayo Clinic, Rohester, MN
Background/Purpose: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently defined clinical entity that causes hematologic and autoinflammatory symptoms. Since its initial description…
Abstract Number: 0725 • ACR Convergence 2024
Serum Glial Fibrillary Acidic Protein Could Assess Cross-sectional Vasculitis Activity by Reflecting Renal Involvement in Patients with Antineutrophil Cytoplasmic Antibody-associated Vasculitis
Lucy Eunju Lee¹, Taejun Yoon², Yong-Beom Park² and Sang-Won Lee², ¹Yonsei University, Seoul, South Korea, ²Yonsei University College of Medicine, Seoul, Republic of Korea
Background/Purpose: Glial fibrillary acidic protein (GFAP), an intermediate filament protein produced by astrocytes in the CNS, has been identified as a biomarker for disease activity…
Abstract Number: 1276 • ACR Convergence 2024
Therapeutic Drug Monitoring (TDM) of Rituximab to Predict Early B-Cell Repopulation in Children
Rana Alsulami¹, Bindiya Chugani², Joley Johnstone², Ann Yeh², Floris Loeff³, Linda Hiraki², Andrea Knight⁴, Deborah Levy² and Ruud Verstegen², ¹University of Toronto, Toronto, ON, Canada, ²The Hospital for Sick Children, Toronto, ON, Canada, ³Sanquin Diagnostic Services, Amsterdam, Netherlands, ⁴Division of Rheumatology, The Hospital for Sick Children; Neurosciences and Mental Health, SickKids Research Institute; Department of Paediatrics, University of Toronto, Toronto, ON, Canada
Background/Purpose: Rituximab is increasingly used in pediatric inflammatory diseases, with dosing extrapolated from adult data due to a lack of pediatric-specific pharmacokinetic (PK) information. Children…
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: 2483 • ACR Convergence 2024
Efficacy and Safety of Avacopan over 12 Months in Japanese Patients with MPA/GPA: A Single-center Study
Yusuke Ushio¹, Hiromi Shimada², taichi miyagi³, Koichi Sugihara⁴, Mao Mizusaki⁵, Rina Mino⁵, Hayamasa yamaguchi⁵, Naoto Manabe⁵, Shusaku Nakashima² and Hiroaki Dobashi², ¹Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Miki, Kita District, Kagawa, Japan, ²Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Kagawa, Japan, ³Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, kidagun, Japan, ⁴Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Miki-cho, Kita-gun, Kagawa Prefecture, Japan, ⁵Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Miki-cho, Kita-gun, Japan
Background/Purpose: Avacopan, an oral C5a receptor inhibitor, was demonstrated to be an alternative to glucocorticoids (GC) in the treatment of microscopic polyangiitis (MPA)/granulomatosis with polyangiitis…
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: 0726 • ACR Convergence 2024
Management of ANCA Vasculitis-Associated Orbital Inflammatory Disease: A Systematic Literature Review
Emma Neary¹, Katie Healey², Marie Clements-Baker³, Jean-Paul Makhzoum⁴ and Arielle Mendel⁵, ¹McGill University, Montreal, QC, Canada, ²Memorial University of Newfoundland, St. John's, NL, Canada, ³Queen's University, Kingston, ON, Canada, ⁴Vasculitis Clinic, Canadian Network for Research on Vasculitides, Hopital du Sacre-Coeur de Montreal, Montreal, QC, Canada, ⁵McGill University Health Centre, Montreal, QC, Canada
Background/Purpose: Ocular manifestations are common in ANCA-associated vasculitides (AAV), with orbital inflammatory disease (OID) representing a significant subset. Despite being a vision-threatening complication, OID remains…
Abstract Number: 1294 • ACR Convergence 2024
Using Case-Based Continuing Education to Identify and Address Knowledge and Behavior Gaps in ANCA-associated Vasculitis
Bharati Hegde, Jillian Scavone, Katie Robinson and Robert Esgro, Vindico Medical Education, West Deptford, NJ
Background/Purpose: Given the rarity and non-specific symptoms associated with ANCA-associated vasculitis (AAV), rheumatology clinicians are challenged to use best practices for patient recognition and management,…
Abstract Number: 1609 • ACR Convergence 2024
Characteristics of a Cohort of Patients with Interstitial Lung Disease and ANCA Positivity in a University Hospital
Cristina Valero¹, Claudia Valenzuela², Elisa Martínez-Besteiro³, Patricia Quiroga Colina⁴, Arantzazu Alfranca⁵, Esther Vicente-rabaneda⁶, Santos Castañeda⁶ and Rosario García-Vicuña⁷, ¹Hospital de la Princesa, Madrid, Spain, ²ILD Unit, Pulmonology Department, Hospital Universitario de la Princesa, University Autonomade Madrid, Madrid, Spain, Madrid, Madrid, Spain, ³Pulmonology Service, Hospital Universitario de la Princesa, IIS-Princesa, Madrid, Spain, Madrid, Madrid, Spain, ⁴Division of Rheumatology, Hospital Universitario de La Princesa, Madrid, Spain, ⁵Immunology Service, Hospital Universitario de La Princesa, IIS-Princesa, Madrid, Spain, Madrid, Madrid, Spain, ⁶Hospital Universitario de La Princesa, Madrid, Spain, ⁷Hospital Universitario de la Princesa, Madrid, Madrid, Spain
Background/Purpose: The prevalence of ANCA positivity at diagnosis of interstitial lung disease (ILD) ranges between 4-36% for anti-MPO and 2-4% for anti-PR3. ILD is more…
Abstract Number: 2484 • ACR Convergence 2024
Real-World Use of Avacopan for ANCA-Associated Vasculitis Beyond 52 Weeks
Julia Ford¹, Abdallah Geara², Duvuru Geetha³ and Ora Singer⁴, ¹University of Michigan, Ann Arbor, MI, ²University of Pennsylvania, Philadelphia, PA, ³Johns Hopkins University, Baltimore, MD, ⁴University of Michigan, Huntington Woods, MI
Background/Purpose: The landmark ADVOCATE trial, which led to the approval of avacopan (AVP) as adjunctive treatment of ANCA-associated vasculitis (AAV), treated patients with AVP for…
Abstract Number: 2690 • ACR Convergence 2024
ANCA IgG Promotion of Neutrophil Recruitment, Migration and Vascular Damage in ANCA Associated Vasculitis May Be Enhanced by Hypoxia
Noelle Pisacano, Amrita Dhutia, Stephen Rothery, Charles Pusey, Edwin Chilvers, Andrew Cowburn, Katharine Lodge and Maria Prendecki, Imperial College London, London, United Kingdom
Background/Purpose: Neutrophils are main mediators of disease in ANCA associated vasculitis (AAV). Pathogenic ANCA IgG on neutrophils promotes neutrophil adhesion and migration at the endothelium.…
Abstract Number: 0727 • ACR Convergence 2024
Prospective Cellular Biomarkers in ANCA Associated Vasculitis: A Pilot Study
Jakub Videman¹, Adela Skoumalova¹, Marketa Dudkova¹, Martina Skacelova¹, Anna Petrackova², Zuzana Mikulkova², Eva Kriegova³ and Pavel Horak¹, ¹3rd Department of Internal Medicine - Nephrology, Rheumatology and Endocrinology, University Hospital Olomouc & Palacky University Olomouc, Faculty of Medicine and Dentistry, Olomouc, Olomoucky kraj, Czech Republic, ²Department of Immunology, Faculty of Medicine and Dentistry, Palacky University and University Hospital, Olomouc, Czechia, Olomouc, Olomoucky kraj, Czech Republic, ³Department of Immunology, Faculty of Medicine and Dentistry, Palacky University and University Hospital, Olomouc, Czechia, Olomouc, Czech Republic
Background/Purpose: Significant progress has been achieved over the course of recent time in elucidating the complex underlying pathophysiological mechanisms of ANCA associated vasculitis (AAV). Despite…
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: 1610 • ACR Convergence 2024
Anti-neutrophil Cytoplasmic Antibodies Associated Interstitial Pneumonia: A New Clinical Entity?
Maria Rosa Pellico¹, Claudia Iannone², Antonella Caminati³, Roberto Cassandro³, Matteo Colleoni³, Davide Elia³, Francesca Luisi³, Maria Rosa Mirenda³, Lisa Tescaro³, Maurizio Zompatori³, Sergio Harari³ and Roberto Caporali⁴, ¹Department of Rheumatology, University of Milan, and Rheumatology Department, ASST Pini-CTO, Milan, ²Department of Rheumatology, University of Milan, and Rheumatology Department, ASST Pini-CTO, Milan, Italy, ³Pneumology and Semi-Intensive Respiratory Therapy Unit, MultiMedica IRCCS, Milan, Italy, Milan, Italy, ⁴Department of Clinical Sciences and Community Health, University of Milan, and Department of Rheumatology and Medical Sciences, ASST Gaetano Pini-CTO, Milan, Italy
Background/Purpose: Pneumologists do not routinely include screening for anti-neutrophil cytoplasmic antibodies (ANCA) in the evaluation of interstitial pneumonia (IP). Indeed, antibodies against myeloperoxidase (anti-MPO) and…

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