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Abstracts tagged "Granulomatosis with Polyangiitis (GPA)"

  • Abstract Number: 0526 • ACR Convergence 2022

    Characteristics and Outcomes of Participants with and Without Diffuse Alveolar Hemorrhage in the Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis (PEXIVAS) Trial

    Lynn Fussner1, Luis Felipe Flores-Suarez2, Rodrigo Cartin-Ceba3, Ulrich Specks3, P. Gerard Cox4, David Jayne5, Peter Merkel6 and Michael Walsh4, 1The Ohio State University, Columbus, OH, 2Instituto Nacional de Enfermedades Respiratorias, Ciudad de México, Mexico, 3Mayo Clinic, Rochester, MN, 4McMaster University, Hamilton, ON, Canada, 5University of Cambridge, Cambridge, United Kingdom, 6University of Pennsylvania, Philadelphia, PA

    Background/Purpose: Diffuse alveolar hemorrhage (DAH) is a potentially life-threatening manifestation of ANCA-associated vasculitis (AAV). Studies describing patients with DAH in AAV have typically been small…
  • Abstract Number: 0527 • ACR Convergence 2022

    Rituximab as Maintenance Therapy for ANCA-associated Vasculitides: Pooled Analysis and Long-term Outcome of MAINRITSAN Trials

    Florence Delestre1, Pierre Charles2, Loïc Guillevin3, Raphaël Porcher4 and Benjamin Terrier3, 1cochin Hospital, Paris, France, 2Institut Mutualiste Montsouris, Paris, France, 3National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, 4Université Paris Cité, Hôtel-Dieu, Paris, France

    Background/Purpose: Maintenance therapy after induction of remission has been shown to reduce relapse rate and mortality in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. The three MAINRITSAN…
  • Abstract Number: 0528 • ACR Convergence 2022

    Nasal Epithelial Gene Expression Profiling Preceding Relapse in Patients with Granulomatosis with Polyangiitis

    Fang R. Zhao1, Rebecca Deek2, James Garifallou3, Jalal Jalaly2, Cailu Lin4, Danielle Reed4, Shubhasree Banerjee2, Naomi Amudala2, Antoine Sreih5, Sherry Chou2, Virginia Livolsi2, Ronald Collman2, Hongzhe Lee2, Peter Merkel2, Peter Grayson6, Noam Cohen2, Jonathan Miner2 and Rennie Rhee2, 1Washington University in St. Louis, St. Louis, MO, 2University of Pennsylvania, Philadelphia, PA, 3Children's Hospital of Philadelphia, Philadelphia, PA, 4Monell Center, Philadelphia, PA, 5Bristol Myers Squibb, Philadelphia, PA, 6National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD

    Background/Purpose: Predicting relapse is a major challenge in granulomatosis with polyangiitis (GPA). We previously demonstrated changes in the nasal microbiota, particularly Corynebacterium tuberculostearicum, prior to…
  • Abstract Number: 0416 • ACR Convergence 2021

    Pharmacological Response of Rituximab Based on Dose Intensity in Maintenance Therapy of ANCA-Associated Vasculitis

    Jason Springer1 and Ryan Funk2, 1Vanderbilt University Medical Center, Franklin, TN, 2University of Kansas Medical Center, Kansas City, KS

    Background/Purpose: Rituximab (RTX) has demonstrated efficacy in maintenance therapy in ANCA-associated vasculitis. However, different dosing protocols have been used in clinical trials and there is…
  • Abstract Number: 0421 • ACR Convergence 2021

    Negative vs. Positive Antineutrophil Cytoplasmic Antibody Granulomatosis with Polyangiitis, a Case-control Study

    Marta Casal Moura, Samuel D. Falde, Ulrich Specks and Misbah Baqir, Mayo Clinic, Rochester, MN

    Background/Purpose: Antineutrophil cytoplasmic antibody (ANCA) negative granulomatosis with polyangiitis (GPA) is a challenging diagnosis. There is paucity of literature regarding the clinical course of ANCA-negative…
  • Abstract Number: 0431 • ACR Convergence 2021

    Clinical Characteristics and Outcomes of ANCA-Associated and Non Immune-Mediated Hypertrophic Pachymeningitis: A Comparative Study

    Eduardo Martin-Nares, Lilian Guadalupe Cano Cruz and Andrea Hinojosa-Azaola, Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico

    Background/Purpose: Hypertrophic pachymeningitis (HP) is an infrequent manifestation in autoimmune and non-autoimmune conditions. The differences in clinical characteristics and outcomes of ANCA-associated and non-immune-mediated HP…
  • Abstract Number: 0436 • ACR Convergence 2021

    Ocular and Orbital Manifestations of Granulomatosis with Polyangiitis: A Systematic Review of Published Cases

    Madeleine Yeakle1, Allen Tsiyer1, Daniel Bishev1, Kayvon Moin1, Victoria Garofalo1, Allison Parrill1, Alexander Hatsis2, Tyler Wickas2 and Prachi Anand3, 1American University of the Caribbean School of Medicine, Cupecoy, Netherlands Antilles, 2Department of Ophthalmology, Nassau University Medical Center, East Meadow, NY, 3Department of Rheumatology, Nassau University Medical Center, East Meadow, NY

    Background/Purpose: Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s Granulomatosis, is a systemic autoimmune condition caused by granulomatous inflammation of small and medium arteries, arterioles…
  • Abstract Number: 0525 • ACR Convergence 2021

    STEAP3, FZD2 and EGFLAM Are Novel Genetic Risk Loci for Granulomatosis with Polyangitis: A Genome Wide Association Study from UK Biobank

    Mehmet Hocaoglu1, Jamal Mikdashi2, Yi-Ju Chen2, James Perry2 and Charles Hong2, 1University of Maryland Medical Center, Midtown Campus, Baltimore, MD, 2University of Maryland School of Medicine, Baltimore, MD

    Background/Purpose: Granulomatosis with Polyangitis (GPA) is a systemic ANCA associated small vessel vasculitis. Prior genetic studies demonstrated a strong association of HLA Class II region…
  • Abstract Number: 2050 • ACR Convergence 2020

    Autoantibodies Targeting Complement Receptors C3aR and C5aR1 Are Decreased in ANCA-associated Vasculitis and Correlate with a Higher Relapse Rate

    Sebastian Klapa1, Antje Müller2, Andreas Koch3, Anja Kerstein-Staehle4, Wataru Kaehler3, Harald Heidecke5, Susanne Schinke6, Markus Huber-Lang7, Martin Nitschke8, Silke Pitann9, Christian Karsten10, Gabriela Riemekasten11 and Peter Lamprecht2, 1University of Lübeck and Institute of Experimental Medicine, Christian-Albrechts-University of Kiel c/o German Naval Medical Institute, Department of Rheumatology and Clinical Immunology, Luebeck, Germany, 2University of Lübeck, Department of Rheumatology and Clinical Immunology, Lübeck, Germany, 3Institute of Experimental Immunology, Christian-Albrechts-University Kiel, c/o German Naval Medical Institute, Kronshagen, Germany, 4University of Lübeck, Dept Rheumatology and Clinical Immunology, Luebeck, Germany, 5CellTrend GmbH, Luckenwalde, Germany, 6University of Lübeck, Department of Rheumatology and Clinical Immunology, Luebeck, Germany, 7Institute of Clinical and Experimental Trauma Immunology, University Hospital Ulm, Ulm, Germany, 8Department of Internal Medicine I, Transplant Center, University of Lübeck, Luebeck, Germany, 9University of Lübeck, Department of Rheumatology and Clinical Immunology,, Luebeck, Germany, 10Institute of Systemic Inflammation Research, University of Lübeck, Luebeck, Germany, 11University of Lübeck, Department of Rheumatology and Clinical Immunology,, Lübeck, Germany

    Background/Purpose: Activation of the alternative and common terminal complement pathways has been shown in ANCA-associated vasculitis (AAV). Circulating titers of the anaphylatoxin C5a are increased…
  • Abstract Number: 2051 • ACR Convergence 2020

    Localized versus Systemic Granulomatosis with Polyangiitis: Data from the French Vasculitis Study Group Database

    Michele Iudici1, Christian Pagnoux2, Delphine Courvoisier3, Pascal Cohen4, Mohamed Hamidou5, Achille Aouba6, François Lifermann7, Marc Ruivard8, Olivier Aumaitre9, Bernard Bonnotte10, Julien Campagne11, Olivier Decaux12, Eric Hachulla13, Alexandre Karras14, Chahéra Khouatra15, Noémie Jourde-Chiche16, Jean-François Viallard17, Pascal Godmer18, Claire Blanchard-Delaunay19, Alain Le Quellec20, Thomas Quéméneur21, Claire de Moreuil22, Alexis Regent4, Benjamin Terrier4, Luc Mouthon4, Loïc Guillevin4 and Xavier Puechal4, 1National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris-Descartes University and Geneva University Hospitals, Geneva, Switzerland, 2Division of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, 3Division of rheumatology, Geneva University Hospitals, Geneva, Switzerland, 4National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris-Descartes University, Paris, France, 5Department of Internal Medicine, Hôtel-Dieu, CHU Nantes, Nantes, France, 6Department of Internal Medicine, CHU Côte de Nacre, Caen, France, 7Department of Internal Medicine, CH Côte d’Argent, Dax, France, 8Department of Internal Medicine, CHU Estaing, Clermont Ferrand, France, 9Department of Internal Medicine, CHU Gabriel Montpied, Clermont Ferrand, France, 10CHU Dijon, Dijon, France, 11Department of Internal Medicine, Hôpitaux Privés de Metz, Metz, France, 12CHU Rennes, Rennes, France, 13Department of Internal Medicine, CHRU, Lille Cedex, France, 14Department of Nephrology, Hôpital Européen Georges Pompidou, Paris, France, 15Department of Respiratory Medicine, CHU Louis Pradel, Lyon, France, 16Department of Nephrology, CHU de la Conception, Marseille, France, 17Department of Internal Medicine, CHU Haut-Lévêque, Bordeaux, France, 18Department of Internal Medicine, CH Bretagne Atlantique, Vannes, France, 19Department of Internal Medicine, CH, Niort, France, 20Department of Internal Medicine, CHU Saint Eloi, Montpellier, France, 21Department of Internal Medicine, CH, Valenciennes, France, 22Department of Internal Medicine, CHU Cavale Blanche, Brest, France

    Background/Purpose: The clinical picture at onset and evolution of localized forms of granulomatosis with polyangiitis (L-GPA) have already been investigated but, to our knowledge, have…
  • Abstract Number: 0060 • ACR Convergence 2020

    ANCA-associated Vasculitis in Caucasian and Hispanics of the Inland Empire of Southern California

    Sandy Lee1, Patil Injean1, Paulina Tran2, Deepa Panikkath1 and Christina Downey1, 1Loma Linda University Medical Center, Redlands, CA, 2UC Riverside School of Medicine, Riverside, CA

    Background/Purpose: ANCA-associated vasculitis (AAV) is often studied in the Caucasian population with few studies looking at the disease in other races. Disease presentation and treatment…
  • Abstract Number: 1032 • ACR Convergence 2020

    Epidemiology and Geographic Evaluation of ANCA-associated Vasculitis (AAV) at a Rural Academic Health Center Utilizing an Electronic Health Record (EHR)

    Eva Rottmann1, Jason Bankert2, Andrea Berger2, Lester Kirchner2, Joseph Dewalle2, Marissa Meissner3, Jason George2, Ion Dan Bucaloiu2, Jaya Prakash Sugunaraj2, Sudheer Penupolu2 and David Bulbin1, 1Geisinger Health System, Danville, PA, 2Geisinger Medical Center, Danville, PA, 3Geisinger Medical Center, Danville

    Background/Purpose: ANCA-associated vasculitis has a reported prevalence ranging from 3.2 to 9.1 cases per 100,000 individuals, but little is known of geographic variations. The aim of…
  • Abstract Number: 1417 • ACR Convergence 2020

    Risk of Relapse of ANCA-associated Vasculitis in Patients of 75 Years and Older: A Retrospective Study

    Sara Thietart1, Guillaume Beinse2, Perrine Smets3, Alexandre Karras4, Carole Philipponnet5, Jean-François Augusto6, Khalil El Karoui7, Rafik Mesbah8, Dimitri Titeca Beauport9, Mohamed Hamidou10, Pierre-Louis Carron11, Julien Campagne12, Karim Sacre13, Pascal Cohen1, Eric Liozon14, Claire Blanchard-Delaunay15, Alex Kostianovsky16, Christian Pagnoux17, Luc Mouthon1, Loïc Guillevin18, Benjamin Terrier1 and Xavier Puéchal for the French Vasculitis Study Group1, 1National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris-Descartes University, Paris, France, 2Inserm UMR 1138, Centre de Recherche des Cordeliers, Paris, France, 3Department of Internal Medicine, Centre Hospitalier Universitaire Clermont-Ferrand Hôpital Gabriel-Montpied, Clermont Ferrand, France, 4Department of Nephrology, Hôpital Européen Georges Pompidou, Paris, France, 5Department of Nephrology, CHU Clermont-Ferrand, Clermont-Ferrand, Clermont Ferrand, France, 6Department of Nephrology-Dialysis-Transplantation, CHU Angers, Angers, France, 7Department of Nephrology and Transplantation, Hôpital Universitaire Henri-Mondor, Creteil, France, 8Department of Nephrology, CH Boulogne-sur-Mer, Boulogne-Sur-Mer, France, 9Department of Nephrology, Amiens University Medical Center, Amiens, France, 10Department of Internal Medicine, Hôtel-Dieu, CHU Nantes, Nantes, France, 11Department of Nephrology-Dialysis-Transplantation, CHU Grenoble-Alpes, Grenobles, France, 12Department of Internal Medicine, Hôpitaux Privés de Metz, Metz, France, 13Département de Médecine Interne, Hôpital Bichat, APHP, Paris, France, 14Department of Internal Medicine and Clinical Immunology, Limoges University Hospital, Limoges, France, 15Department of Internal Medicine, CH, Niort, France, 16Centro de Educación Médica e Investigaciones Clínicas "Norberto Quirno", Buenos Aeres, Argentina, 17Vasculitis Clinic, Canadian Network for Research on Vasculitides (CanVasc), Department of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, 18Department of Internal Medecine, National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris-Descartes University, Paris, France

    Background/Purpose: ANCA-associated vasculitis (AAV) frequently occurs among older patients, with different clinical presentations than the younger. Little is known on the outcome of older patients…
  • Abstract Number: 1418 • ACR Convergence 2020

    Clinical Presentations and Follow-up Results of Granulomatosis with Polyangiitis: An Analysis of 8 Years Clinical Experience with 220 Patients from a National Referral Center

    Seyed Behnam Jazayeri1, Farzaneh Kianifar2, Samira Alesaeidi2, Soheil Tavakolpour3, Amir Teimourpour4 and Sasan Dabiri2, 1Tehran university of medical scienses, Tehran, Iran, 2Tehran university of medical sciences, Tehran, Iran, 3Dana-faber cancer institute, Harvard Medical School, Boston, MA, 4Department of epidemiology and bio-statistics, Tehran university of medical sciences, Tehran, Iran

    Background/Purpose: Granulomatosis with polyangiitis (GPA) is a rare and life-threatening autoimmune disease. Due to the extremely low prevalence of GPA, monitoring of clinical characteristics and…
  • Abstract Number: 1420 • ACR Convergence 2020

    Characterization of ANCA-Associated Vasculitis Among African American Patients

    Sehris Khawaja1, Saloni Godbole1, Maryann Kimoto2, Virginia Steen3 and Kaitlin Quinn4, 1Georgetown University, Washington, DC, 2UCLA, Los Angeles, CA, 3Division of Rheumatology, Department of Medicine, MedStar Georgetown University Hospital, Washington, DC, 4National Institutes of Health, Washington, DC

    Background/Purpose: ANCA-associated vasculitis (AAV) comprises a group of disorders characterized by inflammation of small and medium-sized arteries.  Prevalence and phenotype of these diseases appear to…
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