Abstracts tagged "ANCA"

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: 1928 • ACR Convergence 2022
Idiopathic Pulmonary Capillaritis Within the Spectrum of Pediatric Diffuse Alveolar Hemorrhage
Angela Chun¹, Tiphanie vogel¹, Andrea Ramirez¹, Marietta De Guzman², Eyal Muscal¹ and Manuel Silva-Carmona³, ¹Baylor College of Medicine, Houston, TX, ²Baylor College of Medicine/ Texas Children's Hospital, Houston, TX, ³Baylor College of Medicine, Texas Children's Hospital, Houston, TX
Background/Purpose: Diffuse alveolar hemorrhage (DAH) is a life-threatening syndrome with bleeding from the pulmonary microvasculature. It is classified as pulmonary capillaritis if histology shows neutrophilic…
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: 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: 0443 • ACR Convergence 2022
Prognosis of Non-PR3 ANCA-Associated Vasculitis with Glomerulonephritis
Aurélien Chepy¹, Hélène Béhal², Alexandre Karras³, Xavier Puéchal⁴, Benjamin Terrier⁴, David Jayne⁵, Thomas Quéméneur⁶ and Mary-Jane Guerry⁷, ¹CHU Lille, Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Auto-immunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Lille, France, ²University of Lille, CHU Lille, ULR 2694 - METRICS: Évaluation des Technologies de Santé et des Pratiques Médicales, Lille, France, ³HEGP, Paris, France, ⁴National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, ⁵University of Cambridge, Cambridge, United Kingdom, ⁶Valenciennes Hospital, Valenciennes, France, ⁷Department of Nephrology, Centre Hospitalier de Valenciennes, Valenciennes., Valenciennes, France
Background/Purpose: Immunosuppressive treatments have improved the prognosis of anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV), transforming these formerly fatal diseases into chronic conditions, with periods of…
Abstract Number: 0450 • ACR Convergence 2022
Incidence of Solid and Hematologic Tumors in Patients with ANCA: A 10-year Retrospective Cohort Study
Enrico Brunetta¹, Giacomo Ramponi², marco folci², Maria De Santis³, Giacomo Maria Guidelli¹, Angela Ceribelli⁴, Nicoletta Luciano¹, Marta Caprioli⁵, Arianna Sonaglia¹ and Carlo Selmi⁶, ¹IRCCS Humanitas Research Hospital, Rheumatology and Clinical Immunology, Rozzano, Italy, ²IRCCS Humanitas Research Hospital, Internal Medicine, Rozzano, Italy, ³Humanitas University, IRCCS Humanitas Research Hospital, Rheumatology and Clinical Immunology, Pieve Emanuele, Italy, ⁴Humanitas Research Hospital, Humanitas University, Rozzano (MI) Italy, Borgo San Giacomo, Italy, ⁵IRCCS Humanitas Research Hospital, Rheumatology and Clinical Immunology, Pavia, Italy, ⁶IRCCS Humanitas Research Hospital, Humanitas University, Rozzano (MI), Italy, Rozzano, Italy
Background/Purpose: Serum antineutrophil cytoplasmic antibodies (ANCA) are significantly associated with the development of ANCA-associated vasculitides (AAV) but may also be detected in patients with other…
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: 0513 • ACR Convergence 2022
The Association Between Age of Diagnosis and Disease Characteristics and Damage in Patients with Antineutrophil Cytoplasmic Antibody-Associated Vasculitis
Jessica Bloom¹, Kaci Pickett², Lori Silveira², David Cuthbertson³, Nader Khalidi⁴, Curry Koening⁵, Carol Langford⁶, Carol McAlear⁷, Paul Monach⁸, Larry Moreland¹, Christian Pagnoux⁹, Rennie Rhee¹⁰, Philip Seo¹¹, Ulrich Specks¹², Kenneth J. Warrington¹², Robert Fuhlbrigge¹ and Peter Merkel¹⁰, ¹University of Colorado, Denver, CO, ²University of Colorado Anschutz Medical Campus, Aurora, CO, ³University of South Florida, Tampa, FL, ⁴McMaster University, Hamilton, ON, Canada, ⁵University of Texas Dell Medical School, Austin, TX, ⁶Cleveland Clinic, Cleveland, OH, ⁷University of Pennsylvania, Philadelphia, ⁸VA Boston Healthcare System, Boston, MA, ⁹Mount Sinai Hospital, Toronto, ON, Canada, ¹⁰University of Pennsylvania, Philadelphia, PA, ¹¹Johns Hopkins University, Baltimore, MD, ¹²Mayo Clinic, Rochester, MN
Background/Purpose: Clinical characteristics and outcomes often differ between children and adults diagnosed with the same rheumatic condition; however, such comparative data is limited in ANCA-associated…
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: 1072 • ACR Convergence 2022
Efficacy and Safety Assessment of a Rituximab Biosimilar in Patients with Granulomatosis with Polyangiitis (GPA)
Adil Vural¹, Komal Mushtaq¹, Chao Zhang¹, Mahmoud Alwakeel¹, Vickie Sayles¹, Kristine Duly², Carol Langford¹ and Rula Hajj-Ali³, ¹Cleveland Clinic, Cleveland, OH, ²Cleveland Clinic, Fairview Park, OH, ³Cleveland Clinic, Hunting Valley, OH
Background/Purpose: A biosimilar is a medication that is highly similar to a biologic agent that is already approved by a regulatory organization. Considering the share…
Abstract Number: 1073 • ACR Convergence 2022
Analysis of Clinical Outcomes in ANCA-associated Vasculitis Treated with Rituximab: Eighty Years a Single Center Experience in Japan
Soko Kawashima¹, Mitsumasa Kishimoto², Yoshinori Komagata³ and Shinya kaname⁴, ¹Kyorin University, Mitaka Tokyo, Japan, ²Kyorin University School of Medicine, Yokohama, Japan, ³Kyorin University School of Medicine, Tokyo, Japan, ⁴Kyorin University, Tokyo, Japan
Background/Purpose: Contrary to many Western countries, MPO-ANCA-associated vasculitis (MPO-AAV) is dominant in Japan. The therapeutic response to rituximab (RTX) may differ. Therefore, we conducted a…
Abstract Number: 0418 • ACR Convergence 2021
Longitudinal Patterns of Renal Function in ANCA-Associated Vasculitis
Jennifer Hanberg¹, Xiaoqing Fu¹, Claire Cook², John Stone³, Hyon K. Choi⁴ and Zachary Wallace⁵, ¹Massachusetts General Hospital, Boston, MA, ²Division of Rheumatology, Allergy, and Immunology, Mass General Hospital, Boston, MA, ³Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA, ⁴Massachusetts General Hospital, Lexington, MA, ⁵Massachusetts General Hospital, Newton, MA
Background/Purpose: Renal involvement is common in ANCA-associated vasculitis (AAV). Prior analyses show that end stage renal disease (ESRD) in AAV may be multifactorial. Little is…
Abstract Number: 0428 • ACR Convergence 2021
ANCA Positivity and ANCA Associated Vasculitis in Patients with Rheumatoid Arthritis
Courtney O'Brien, Veena Ranganath and Tannaz Kermani, University of California Los Angeles, Los Angeles, CA
Background/Purpose: Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) with rheumatoid arthritis (RA) has been reported rarely, and, studies have found ANCA positivity in RA. The…
Abstract Number: 1439 • ACR Convergence 2020
Proteinase 3-Reactive B Cell Pool Restructuring After Rituximab and Risk of Relapse in Severe PR3-ANCA-Associated Vasculitis
Alvise Berti¹, Sophie Hillion², Marta Casal Moura¹, Amber Hummel¹, Eva Carmona¹, Tobias Peikert¹, Carol Langford³, Peter A. Merkel⁴, Paul Monach⁵, Philip Seo⁶, Robert Spiera⁷, Eugene St Clair⁸, Fernando C. Fervenza¹, Kristina Harris⁹, John H. Stone¹⁰, Jacques-Olivier Pers¹¹, Ulrich Specks¹ and Divi Cornec¹², ¹Mayo Clinic, Rochester, MN, ²Brest University, Brest, Bretagne, France, ³Cleveland Clinic, Moreland Hills, OH, ⁴Division of Rheumatology University of Pennsylvania, Philadelphia, PA, ⁵Brigham and Women's, Boston, ⁶Johns Hopkins University, Baltimore, MD, ⁷Division of Rheumatology, Hospital for Special Surgery, New York, New York, USA, New York, NY, ⁸Duke University, Durham, NC, ⁹Immune Tolerance Network, Bethesda, MD, ¹⁰Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA, ¹¹University of Brest, Brest, France, ¹²Rheumatology Department, Brest University Hospital, Brest, France
Background/Purpose: In ANCA-associated vasculitis (AAV) B cells play a central pathogenic role and are instrumental for the production of ANCA, which are thought to mediate…
Abstract Number: 1440 • ACR Convergence 2020
The Myeloperoxidase (MPO) Anti-Neutrophilic Cytoplasmic Antibody (ANCA) Binding Epitope, MPO447-459 Induces CD4 T-cell Proliferation in Patients with MPO-ANCA-associated Vasculitis
Matthew Terrill¹, Hendrik Nel², Yassmin Musthaffa³, Wong Richard⁴, Ross Francis⁵, David Johnson⁵, Greg Keir⁶, David Gillis⁷ and Ranjeny Thomas⁸, ¹University of Queensland Diamantina Institute and Princess Alexandra Hospital, Rheumatology Department, Brisbane- Australia, Moffat beach, Queensland, Australia, ²University of Queensland Diamantina Institute, Brisbane, Queensland, Australia, ³University of Queensland Diamantina Institute, Brisbane, Australia, ⁴Immunology Department, Princess Alexandra Hospital, Brisbane- Australia, Brisbane, Australia, ⁵Renal Department, Princess Alexandra Hospital, Brisbane- Australia., Brisbane, Queensland, Australia, ⁶Respiratory Department, Princess Alexandra Hospital, Brisbane- Australia., Brisbane, Queensland, Australia, ⁷Immunopathology Department, Royal Women’s and Children’s Hospital, Brisbane- Australia., Brisbane, Queensland, Australia, ⁸University of Queensland Diamantina Institute and Rheumatology Department, Princess Alexandra Hospital, Brisbane – Australia., Brisbane, Australia
Background/Purpose: In Myeloperoxidase (MPO) Anti Neutrophilic Cytoplasmic Antibody (ANCA)-Associated Vasculitis (MPO-AAV), murine and human studies suggest that the MPO435-465 region, which includes ANCA-binding MPO447-459, the…

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