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Abstracts tagged "ANCA associated vasculitis"

  • Abstract Number: LB16 • ACR Convergence 2025

    Mepolizumab Reduces End-Organ Manifestations Compared with Standard of Care in Patients with EGPA and HES: A US Real-world Analysis

    David Silverman1, Timothy Barnes2, Nnaemeka Odo2, Jared Silver3, Lisa Le2, Amy Edgecomb4 and Hitesh Patel5, 1Rheumatology Department, Kaiser Permanente Colorado Medical Group, Denver, Colorado, 2Value and Evidence Solutions, Optum Life Sciences, Eden Prairie, Minnesota, 3Clinical Development, Amgen, Thousand Oaks, California, 4Anti-infectives and Respiratory, GSK, Philadelphia, Pennsylvania, 5US Medical Affairs, GSK, Durham, North Carolina

    Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) and hypereosinophilic syndrome (HES) are rare systemic diseases characterized by persistent eosinophilia and tissue infiltration, resulting in end-organ dysfunction…
  • Abstract Number: 2520 • ACR Convergence 2025

    Evolution in Risk Stratification for Renal Outcomes in ANCA-Associated Vasculitides Using Established Scores and Histopathological Criteria in a Large European Cohort

    Stefan Krämer1, Kristian Vogt2, Martin Busch3, Tobias Schmitt4, Raoul Bergner5, Sebastian Mosberger5, Thomas Neumann6, Teresa Schreibing1, Fabian Schumbrink1 and Thomas Rauen2, 1Department of Internal Medicine II, RWTH University Hospital Aachen, Aachen, Germany, 2Department of Internal Medicine II, RWTH University Hospital AachenMedicine II, Aachen, Germany, 3Department of Internal Medicine III, University Hospital, Friedrich-Schiller University, Jena, Jena, Germany, 4University Hospital Jena, Friedrich-Schiller University, Department of Internal Medicine III, Jena, Germany, 5Department of Internal Medicine A, Nephrology and Rheumatology, Municipal Hospital Ludwigshafen, Ludwigshafen, Germany, 6Department of Rheumatology, Cantonal Hospital St. Gallen, St. Gallen, Switzerland

    Background/Purpose: Glomerulonephritis is a common and threatening manifestation in ANCA-associated vasculitis (AAV). To assess the risk of end-stage kidney disease (ESKD), various scores have been…
  • Abstract Number: 1769 • ACR Convergence 2025

    Classification of Relapses of Eosinophilic Granulomatosis with Polyangiitis After Two-Years of Treatment with Anti-Interleukin-5/Receptor Therapy

    Christian Pagnoux1, Arnaud Bourdin2, Bernhard Hellmich3, Nader Khalidi4, David Jackson5, David Jayne6, Parameswaran Nair7, Ulrich Specks8, Benjamin Terrier9, Lena Börjesson Sjö10, Priya Jain11, Aadarsh Lal12, Sofia Necander13, Claire Walton14, Michael Wechsler15 and Peter Merkel16, 1Mount Sinai Hospital, University Health Network, Toronto, and Canadian Vasculitis Research Network (CanVasc), Toronto, ON, Canada, 2Department of Respiratory Diseases, Montpellier University Hospitals, Arnaud de Villeneuve Hospital, Montpellier, France, 3Klinik für Innere Medizin, Rheumatologie, Pneumologie, Nephrologie und Diabetologie, Medius Kliniken, Akademisches Lehrkrankenhaus der Universität Tübingen, Kirchheim unter Teck, Germany, 4Department of Medicine, McMaster University and St. Joseph’s Healthcare, Hamilton, Canada, 5Guy’s Severe Asthma Centre, Guy's and St Thomas’ NHS Trust, London, and School of Immunology and Microbial Sciences, King's College London, London, United Kingdom, 6University of Cambridge, Cambridge, United Kingdom, 7Department of Medicine, McMaster University and St. Joseph’s Healthcare, Hamilton, ON, Canada, 8Mayo Clinic, Rochester, MN, 9Cochin Hospital, Paris, France, 10Late-stage Respiratory & Immunology, BioPharmaceuticals R&D, AstraZeneca,, Gothenburg, Sweden, 11BioPharmaceuticals Medical, AstraZeneca, Cambridge, United Kingdom, 12Respiratory & Immunology, AstraZeneca, Bengaluru, Karnataka, India, 13Late-stage Respiratory & Immunology, BioPharmaceuticals R&D, AstraZeneca, Gothenburg, Sweden, 14Late-Stage Respiratory and Immunology, BioPharmaceuticals R&D, AstraZeneca, Cambridge, United Kingdom, 15Department of Medicine, National Jewish Health, Denver, 16University of Pennsylvania, Philadelphia, PA

    Background/Purpose: Results from the ongoing open-label extension (OLE) of the MANDARA trial (NCT04157348) have demonstrated the efficacy of two years of anti-interleukin-5/receptor (anti-IL-5/R) therapies in…
  • Abstract Number: 1601 • ACR Convergence 2025

    Obinutuzumab Induces and Maintains Remission in Refractory or Relapsing ANCA-Associated Vasculitis – A Case Series

    Helen Au1, Christian Löffler2, Hartmut Mahrhofer1, Bastian Walz1 and Bernhard Hellmich1, 1Klinik für Innere Medizin, Rheumatologie und Immunologie, medius Klinik Kirchheim, Kirchheim unter Teck, Germany, 2Medizinische Klinik V, Nephrology, Endocrinology, Hypertensiology and Rheumatology, Universitaetsmedizin Mannheim, University of Heidelberg, Mannheim, Germany

    Background/Purpose: B-cell depletion with anti-CD20 therapy is the current cornerstone of management in organ- or life-threatening ANCA-associated vasculitis (AAV). Guidelines recommend the use of rituximab…
  • Abstract Number: 0717 • ACR Convergence 2025

    Circulating Bacterial sRNAs are Altered in Patients with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis

    Christopher Xavier1, Kevin Byram2, Carol Langford3 and Michelle Ormseth4, 1Meharry Medical College, Nashville, 2Vanderbilt University Medical Center, Nashville, TN, 3Cleveland Clinic, Moreland Hills, OH, 4Vanderbilt University Medical Center, Nashville

    Background/Purpose: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis (AAV) is an autoimmune disease causing small vessel inflammation. Underlying mechanisms of disease are unclear, but the human microbiota may…
  • Abstract Number: 0421 • ACR Convergence 2025

    Inflammatory Orbital Diseases: Pediatric Case Series From a Tertiary Care Center

    Rasha Elrefai1, Katherine Williams2 and Hannah Bradfield3, 1Baylor College of Medicine, Houston, TX, 2Texas Children's Hospital, Houston, TX, 3Baylor College of Medicine/Texas Children's Hospital, Houston, TX

    Background/Purpose: Orbital inflammatory diseases in the pediatric population present diagnostic and therapeutic challenges due to variable clinical manifestations, limited prospective data, and lack of standardized…
  • Abstract Number: 2518 • ACR Convergence 2025

    Impact of psychiatric comorbidity on disease outcomes in ANCA-associated vasculitis: A multi-center retrospective cohort study

    Saloni Patel1, Brendan Denvir2, Maria Kaltchenko1, Aaron Bao3, Varsha Simha1, Michael Cammarata4 and Jun Kang5, 1Johns Hopkins University School of Medicine, Baltimore, 2Johns Hopkins, Baltimore, MD, 3Johns Hopkins School of Medicine, Baltimore, MD, 4Johns Hopkins, Phoenix, MD, 5Johns Hopkins University School of Medicine, Baltimore, MD

    Background/Purpose: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic small vessel vasculitis associated with significant morbidity and complex care needs across a broad demographic…
  • Abstract Number: 1767 • ACR Convergence 2025

    Identification of C3 complement fragments in lesional tissues in ANCA-associated vasculitis

    Claire Galand1, Fei Liu2, Katherine Vernon2, Ravi Vats2, Christine Lian3, V. Michael Holers4, H. Terence Cook5, David Cuthbertson6, Nader Khalidi7, Curry Koening8, Carol Langford9, Carol McAlear10, Paul Monach11, Christian Pagnoux12, Jason Campagna2, Shelia Violette2 and Peter Merkel10, 1Q32 Bio Inc,, Waltham, MA, 2Q32 Bio Inc, Waltham, MA, 3Department of Pathology, Brigham & Women’s Hospital/Harvard Medical School, Boston, MA, 4University of Colorado Anschutz Medical Campus, Aurora, 5Department of Immunology and Inflammation, Imperial College London, London, United Kingdom, 6University of South Florida, Tampa, 7McMaster University, Hamilton, ON, Canada, 8University of Texas Dell Medical School, Austin, TX, 9Cleveland Clinic, Moreland Hills, OH, 10University of Pennsylvania, Philadelphia, PA, 11VA Boston Healthcare System, Boston, MA, 12Mount Sinai Hospital, Toronto, ON, Canada

    Background/Purpose: One proposed mechanism of tissue damage in ANCA-associated vasculitis (AAV) is the formation of neutrophil extracellular traps (NETs) during extravasation of neutrophils into and…
  • Abstract Number: 1602 • ACR Convergence 2025

    Biomarkers of Innate Immune Activation to Identify Non-Response to Rituximab in Granulomatosis with Polyangiitis

    Irene Carrión-Barberà1, Jorge A. Gonzalez-Chapa2, David Cuthbertson3, Nader Khalidi4, Curry Koening5, Carol Langford6, Carol McAlear7, Paul Monach8, Larry Moreland9, Christian Pagnoux10, Philip Seo11, Ulrich Specks12, Kenneth Warrington12, Peter Merkel7 and Christian Lood2, 1Department of Rheumatology. Hospital del Mar, Barcelona, Spain, 2University of Washington, Division of Rheumatology, Seattle, 3University of South Florida, Tampa, 4Department of Medicine, McMaster University and St. Joseph’s Healthcare, Hamilton, Canada, 5University of Texas Dell Medical School, Austin, TX, 6Cleveland Clinic, Moreland Hills, OH, 7University of Pennsylvania, Philadelphia, PA, 8VA Boston Healthcare System, Boston, MA, 9University of Colorado, Denver, CO, 10Mount Sinai Hospital, University Health Network, Toronto, and Canadian Vasculitis Research Network (CanVasc), Toronto, ON, Canada, 11Johns Hopkins University, Baltimore, MD, 12Mayo Clinic, Rochester, MN

    Background/Purpose: Innate immune activation plays a key role in granulomatosis with polyangiitis (GPA), contributing to vascular inflammation via neutrophil extracellular traps, immune complexation (IC), and…
  • Abstract Number: 0718 • ACR Convergence 2025

    Incidence of Venous Thromboembolism in Patients with ANCA-Associated Vasculitides – a Nationwide Registry-Based Study from Sweden

    Hanna Lindberg1, Klytaimnistra Voudouri1, Lars Lindhagen1, Maria K Svensson2, Ann Knight1 and Johanna Dahlqvist3, 1Uppsala University, Uppsala, Sweden, 2Uppsala University, Uppsala, 3Uppsala University Hospital, Uppsala, Sweden

    Background/Purpose: Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) are rare yet severe autoimmune diseases. Previous studies have indicated that patients with AAV have an increased risk…
  • Abstract Number: 0150 • ACR Convergence 2025

    Mortality in Anca-associated Vasculitis

    Fabricio Benavides Villanueva1, Vanesa Calvo-Río2, Diana Prieto-Peña3, Monica Renuncio-García4, Adrian Martin-Gutierrez5, Amparo Sanchez-Lopez6, Claudia Poo-fernandez7, Clara Escagedo-Cagigas8, maria Rodríguez-Vidriales8 and Ricardo Blanco2, 1Division of Rheumatology, Hospital Universitario Marques de Valdecilla, IDIVAL, Immunopathology Group,Santander, Spain, Santander, Spain, 2Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDIVAL, Immunopathology Group, Santander, Spain, Santander, Cantabria, Spain, 3Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Immunopathology Group, Santander, Spain., Santander, Spain, 4Division of Immunology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Immunopathology Group, Santander, Spain., Santander, Cantabria, Spain, 5Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Immunopathology Group, Santander, Spain., Renedo de Piélagos, Spain, 6Division of Dermatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Immunopathology Group, Santander, Spain., Santander, Cantabria, Spain, 7Division of Pneumology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Immunopathology Group, Santander, Spain., Santander, Cantabria, Spain, 8Division of Nephrology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Immunopathology Group, Santander, Spain., Santander, Cantabria, Spain

    Background/Purpose: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), include Granulomatosis with Polyangiitis (GPA), Microscopic Polyangiitis (MPA), and Eosinophilic Granulomatosis with Polyangiitis (EGPA). Mortality rates are scare,…
  • Abstract Number: 2517 • ACR Convergence 2025

    Predictors for Mortality in Patients Who Received Plasmapheresis for Diffuse Alveolar Hemorrhage in ANCA-Associated Vasculitis

    Sai Shanmukha Sreeram Pannala1, Medha Rajamanuri2, Srikar Sama3, Fares Saliba4, Omar Mourad5, Koushik Varma Sangaraju6, Veena Katikineni7 and Anastasia Slobodnick8, 1Zucker School of Medicine at Hofstra/Northwell at Staten Island University Hospital, staten island, new york city, NY, 2Southern Illinois University School of Medicine, Springfield, IL, 3University of California San Francisco Fresno, Fresno, CA, 4Staten Island University Hospital, Staten Island, NY, 5Zucker School of Medicine at Hofstra/Northwell at Staten Island University Hospital, Staten Island, NY, 6St. Luke's University Health Network, Bethlehem, PA, 7Desert Orthopaedic and Rheumatologic Institute, Apple Valley, CA, 8Northwell, Staten Island, NY

    Background/Purpose: Plasmapheresis (PLEX) has a compelling rationale in its ability to remove pathogenic ANCAs, in the management of ANCA-associated vasculitis (AAV), particularly in patients with…
  • Abstract Number: 1768 • ACR Convergence 2025

    TREM-1 and TREM-2 Define Inflammatory and Protective Axes in ANCA-Associated Glomerulonephritis

    Lea Dionet1, Alexandre Lanau2, Paul Breillat3, Quentin Delcros4, Federica Pallotti5, Margot Poux6, Pierre Isnard1, Arthur Lavigne4, Xavier Puéchal7, alexandre karras8, Jean-Paul Duong9, Olivia Lenoir10, Pierre-Louis Tharaux11 and Benjamin Terrier12, 1INSERM, Paris, France, 2Institut Curie, Paris, France, 3INSERM, PARIS 17, Ile-de-France, France, 4AP-HP, Paris, France, 5Internal Medicine and Clinical Immunology, Rennes, France, 6Université de Paris, PARIS 04EME, France, 7Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hospital Cochin, and Université Paris Cité, Paris ( 75014 ), Ile-de-France, France, 8HEGP - assistance publique hopitaux de paris, paris, France, 9INSERM, Paris, 10Paris Cardiovascular Research Center, Université Paris Cité, INSERM U970, Paris, France, PARIS, Ile-de-France, France, 11Paris Cardiovascular Research Center, Université Paris Cité, INSERM U970, Paris, France, PARIS, France, 12Cochin Hospital, Paris, France

    Background/Purpose: ANCA-associated vasculitis (AAV) is a small-vessel vasculitis that can lead to life-threatening renal injury. Neutrophils, monocytes and macrophages play a pivotal role not only…
  • Abstract Number: 1600 • ACR Convergence 2025

    Real-world clinical effectiveness of Benralizumab for Eosinophilic Granulomatosis with Polyangiitis: a National multicenter study

    Fabricio Benavides Villanueva1, Andrea Tristan-Alonso2, Carlos Martinez-Rivera3, Alicia Padilla-Galo4, Marina Blanco-Aparicio5 and Ricardo Blanco6, 1Division of Rheumatology, Hospital Universitario Marques de Valdecilla, IDIVAL, Immunopathology Group,Santander, Spain, Santander, Spain, 2Division of Pneumology, Hospital Universitario Puerta del Hierro, Madrid, Spain, Majadahonda, Madrid, Spain, 3Division of Pneumology, Hospital Universitari Germans Trias i Pujol, Barcelona, Spain, Barcelona, Catalonia, Spain, 4Division of Pneumology, Hospital Universitario Virgen de la Victoria, Málaga, Spain, Malaga, Andalucia, Spain, 5Division of Pneumology, Hospital Universitario A Coruña, A Coruña, Spain, A coruña, Galicia, Spain, 6Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDIVAL, Immunopathology Group, Santander, Spain, Santander, Cantabria, Spain

    Background/Purpose: Benralizumab is approved for eosinophil-driven conditions, like eosinophilic asthma. Recently, the European Medicines Agency (EMA) and U.S. Food and Drug Administration (FDA) authorized its…
  • Abstract Number: 0716 • ACR Convergence 2025

    Utility of Follow-Up Cardiac Magnetic Resonance Imaging In Patients With Eosinophilic Granulomatosis With Polyangiitis

    Chelsea Cheng1, Justine Lafleur-Careau2, Navneet Kang1, Medha Soowamber3 and Christian Pagnoux3, 1Mount Sinai Hospital, Toronto, Canada, 2Mount Sinai Hospital, Saint-Augustin-de-Desmaures, Canada, 3Mount Sinai Hospital, Toronto, ON, Canada

    Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem disorder characterized by asthma, rhinitis, peripheral blood eosinophilia, and necrotizing vasculitis of small vessels. Cardiac…
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