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Abstracts tagged "Granulomatosis with Polyangiitis (GPA)"

  • 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: 1084 • ACR Convergence 2022

    Rituximab versus Cyclophosphamide for Induction Therapy in Granulomatosis with Polyangiitis: A Target Trial Emulation Study

    Xavier Puéchal1, Michele Iudici2, Elodie Perrodeau3, bernard bonnotte4, Francois Lifermann5, Thomas Le Gallou6, Alexandre Karras7, Claire Blanchard-Delaunay8, Thomas Quéméneur9, Achille Aouba10, Olivier Aumaître11, Vincent Cottin12, Mohamed Hamidou13, Marc Ruivard11, Pascal Cohen1, Luc Mouthon1, Loïc Guillevin1, Philippe Ravaud3, Raphaël Porcher3 and Benjamin Terrier1, 1National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, 2Division of Rheumatology, Geneva University Hospitals, Geneva, Switzerland, 3Université Paris Cité, Hôtel-Dieu, Paris, France, 4Centre Hospitalo-Universitaire de Dijon, Dijon, France, 5Dax Hospital, Dax, France, 6Rennes Sud University Hospital, Rennes, France, 7HEGP, Paris, France, 8Niort Hospital, Niort, France, 9Valenciennes Hospital, Valenciennes, France, 10Department of Internal Medicine, UR4650 PSIR, Normandie Univ, UNICAEN, CHU de Caen Normandie, Caen, France, 11Clermont Ferrand University Hospital, Clermont-Ferrand, France, 12Coordinating Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, University of Lyon, INRAE, Lyon, France, 13Nantes University Hospital, Nantes, France

    Background/Purpose: Randomized controlled trials showed rituximab (RTX)'s non-inferiority to cyclophosphamide (CYC) for induction therapy of ANCA-associated vasculitis and neither treatment was favoured in granulomatosis with…
  • Abstract Number: 1948 • ACR Convergence 2022

    Clinical Characteristics and Outcomes of Pediatric ANCA-Associated Vasculitis Patients: Single-Center Inception Cohort

    Hyun Ho Lee1, Lauren Blazek2 and Eveline Wu3, 1The University of North Carolina Chapel Hill, Chapel Hill, NC, 2UNC Kidney Center, The University of North Carolina Chapel Hill, Chapel Hill, NC, 3University of North Carolina, Chapel Hill, NC

    Background/Purpose: ANCA associated vasculitis (AAV) is a chronic autoimmune disorder characterized by ANCA production and small vessel inflammation and necrosis. ANCA autoantigen targets include myeloperoxidase…
  • Abstract Number: 0439 • ACR Convergence 2022

    Clinical Characteristics of Patients with ANCA-Associated Vasculitis with and Without Alpha-1 Antitrypsin Deficiency Alleles

    Lynn Fussner1, David Cuthbertson2, Peter Grayson3, David Jayne4, Carol McAlear5, Michael Walsh6, Katherine Siminovitch7, Ulrich Specks8 and Peter Merkel9, 1The Ohio State University, Columbus, OH, 2University of South Florida, Tampa, FL, 3National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, 4University of Cambridge, Cambridge, United Kingdom, 5Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, USA, Philadelphia, PA, 6McMaster University, Hamilton, ON, Canada, 7University of Toronto, Toronto, ON, Canada, 8Mayo Clinic, Rochester, MN, 9University of Pennsylvania, Philadelphia, PA

    Background/Purpose: There are multiple reasons to suspect that alpha-1 antitrypsin (A1AT) genotype impacts disease characteristics in ANCA-associated vasculitis (AAV). A1AT serves as the predominant endogenous…
  • Abstract Number: 0442 • ACR Convergence 2022

    Induction Therapy Patterns for Severe ANCA-associated Vasculitis Differ Based on Physician Specialty, Expertise, and Practice Setting: An International Survey

    Lilian Xu1, Faten Aqeel1, Ojaswi Tomar2, Tingting Li2 and Duvuru Geetha1, 1Johns Hopkins University School of Medicine, Baltimore, MD, 2Washington University in St. Louis School of Medicine, St. Louis, MO

    Background/Purpose: Therapies for ANCA-associated vasculitis (AAV) have evolved over the last 3 decades. In light of new data on plasma exchange (PLEX) and glucocorticoid (GC)…
  • Abstract Number: 0453 • ACR Convergence 2022

    Validation of the 2022 – American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria in Patients with ANCA Associated Vasculitis

    Aman Sharma1, Prateek Deo1, Shankar Naidu2, Joydeep Samanta3, Sakshi Mittal3, Kusum Sharma3, Ritambhra Nada3, Varun Dhir4, sanjay jain2 and Ranjana Minz2, 1PGIMER, Chandigarh, India, Chandigarh, India, 2Postgraduate Institute of Medical Education and Research, Chandigarh, India, 3PGIMER, Chandigarh, India, 4PGIMER, CHD, INDIA, Chandigarh, India

    Background/Purpose: The purpose of this study was to validate the recently published 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for…
  • Abstract Number: 0454 • ACR Convergence 2022

    Advantages and Disadvantages in Applying 2022 ACR/EULAR Classification Criteria to Conventionally Diagnosed Japanese GPA Patients

    Ryo Kuwata1, Yuko Shirota2, Tsuyoshi Shirai3, Hiroyuki Yamashita1, Hiroko Sato3, Tomoki Takeda2, Yumiko Oka2, Takao Kodera4, Hiroshi Kaneko1, Junichi Kameoka5, Hiroshi Fujii3 and Tomonori Ishii6, 1Division of Rheumatic diseases, National Center for Global Health and Medicine, Tokyo, Japan, 2Division of Hematology and Rheumatology, Tohoku Medical and Pharmaceutical University, Sendai, Japan, 3Department of Rheumatology, Tohoku University Hospital, Sendai, Japan, 4Department of Hematology and Rheumatology, Tohoku Medical and Pharmaceutical University, Sendai, Japan, 5Divison of Hematology and Rheumatology, Tohoku Medical and Pharmaceutical University, Sendai, Japan, 6Clinical Research, Innovation and Education Center, Tohoku University Hospital, Sendai, Japan

    Background/Purpose: The Chapel Hill Consensus Conference and the European Medicines Agency algorithm were commonly used to diagnose patients with granulomatosis with polyangiitis (GPA) 1). Recently…
  • Abstract Number: 0455 • ACR Convergence 2022

    Performance of 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria in Turkish Vasculitis Patients

    Ummugulsum Gazel1, Sevinc Can Sandikci2, Ahmet Omma3, Sinem Burcu Kocaer4, Fatoş Onen5, Onay Gercik6, Servet Akar7, Kerem Abacar8, Ebru Asicioglu9, Serhan Tuglular9, Fatma Alibaz-Oner8 and Haner Direskeneli10, 1University of Ottawa, Rheumatology, Ottawa, Canada, Ottawa, ON, Canada, 2Ankara Numune Education and Research Hospital, Rheumatology, Ankara, Turkey, 3Ankara Numune Education and Research Hospital, Rheumatology, Ankara, Turkey, Istanbul, Turkey, 4Dokuz Eylul University, Faculty of Medicine, Rheumatology, Izmir, Turkey, 5Dokuz Eylul University, Faculty of Medicine, Rheumatology, İzmir, Turkey, 6Izmir Tepecik Research Hospital, Izmir, Turkey, 7Izmir Katip Celebi University School of Medicine, Izmir, Turkey, 8Marmara University, Rheumatology, Istanbul, Turkey, 9Marmara University, Faculty of Medicine, Nephrology, Istanbul, Turkey, 10Marmara University, Rheumatology, Istanbul, Istanbul, Turkey

    Background/Purpose: The American College of Rheumatology (ACR)-1990 criteria is the most used method for the classification of ANCA Associated Vasculitis (AAV). However, with ACR-1990 criteria,…
  • Abstract Number: 0457 • ACR Convergence 2022

    A Systematic Literature Review Informing the 2022 Update of the EULAR Recommendations for the Management of ANCA-associated Vasculitis

    Jan Schirmer1, Beatriz Sanchez-Alamo2, Sara Monti3, Bernhard Hellmich4, David Jayne5 and Gunnar Tomasson6, 1University Medical Center Schleswig-Holstein, Kiel, Germany, 2Lund University, Skåne University Hospital, Lund, Sweden, 3Rheumatology, Fondazione IRCCS Policlinico S. Matteo, University of Pavia, Pavia, Italy, 4Medius Kliniken, University of Tübingen, Kirchheim Teck, Germany, 5University of Cambridge, Cambridge, United Kingdom, 6Centre for Rheumatology Research, University Hospital Reykjavik, Reykjavik, Iceland

    Background/Purpose: Since the publication of the 2016 European Alliance of Associations for Rheumatology (EULAR) recommendations for the management of ANCA-associated vasculitis (AAV), the publication of…
  • 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: 1071 • ACR Convergence 2022

    Trimethoprim Sulfamethoxazole Use in Patients with Granulomatosis with Polyangiitis Treated with Rituximab

    Arielle Mendel1, Hassan Behlouli2, Cristiano Moura2, Evelyne Vinet1, Jeffrey Curtis3 and Sasha Bernatsky2, 1McGill University Health Centre, Montréal, QC, Canada, 2Research Institute of the McGill University Health Centre, Montréal, QC, Canada, 3Division of Clinical Immunology & Rheumatology, University of Alabama at Birmingham, Birmingham, AL

    Background/Purpose: Trimethoprim sulfamethoxazole (TMP-SMX) prophylaxis is recommended during induction of ANCA-associated vasculitis (AAV) to prevent pneumocystis jirovecii pneumonia (PJP), and may also reduce the risk…
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