Abstracts tagged "Wegener’s granulomatosis"

Abstract Number: 095 • 2020 Pediatric Rheumatology Symposium
Pyoderma Gangrenosum Ulceration as a Presenting Feature of Pediatric Granulomatosis with Polyangiitis (GPA)
Rotem Semo¹, Rotem Tal ², Tomas Dallos ³, Liora Harel ⁴, Lukas Plank ³ and Linda Wagner-Weiner ¹, ¹university of chicago, chicago, Illinois, ²schneider medical center, Ramat Gan, Israel, ³National Institute of Child Diseases, Bratislava, Slovakia, ⁴Schneider Hospital, Tel Aviv University, Petah-Tiqva, Israel
Background/Purpose: GPA is a form of anti-neutrophilic cytoplasmic antibody vasculitis affecting small to medium sized vessels and involves most commonly the kidneys and the respiratory…
Abstract Number: 872 • 2019 ACR/ARP Annual Meeting
Granulomatosis with Polyangiitis Sustained Remission Off-Therapy: Data from the French Vasculitis Study Group Registry
Xavier Puéchal for the French Vasculitis Study Group¹, Michele Iudici ², Christian Pagnoux ³, Alexandre Karras ⁴, Pascal Cohen ¹, François Maurier ⁵, Thomas Quémeneur ⁶, François Lifermann ⁷, Mohamed Hamidou ⁸, Luc Mouthon ¹, Benjamin Terrier ¹ and Loic Guillevin ¹, ¹National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France, ²National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin and Geneva Switzerland, Paris, France, ³National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin and Toronto Canada, Paris, France, ⁴Paris HEGP, Paris, France, ⁵Service de Médecine Interne, Hôpital Belle Isle, Metz, Metz, France, ⁶CH Valenciennes, Valenciennes, France, ⁷CH Dax, Dax, France, ⁸CHU Nantes, Nantes, France
Background/Purpose: Data on granulomatosis with polyangiitis (GPA) sustained remission off-therapy (SROT) are limited and it is unknown whether disease characteristics or treatment regimen may affect…
Abstract Number: 1677 • 2019 ACR/ARP Annual Meeting
Management of Severe Renal Disease in Anti-Neutrophil-Cytoplasmic-Antibodies Associated Vasculitis: Role of Rituximab and Plasma Exchange?
Pauline Morel ¹, Alexandre Karras ², Raphael Porcher ³, Xavier Belenfant ⁴, Vincent Audard ⁵, Cédric Rafat ⁶, Guillaume Hanouna ⁷, Séverine Beaudreuil ⁸, Cédric Vilain ⁹, Aurélie Hummel ¹⁰, Benjamin Terrier ¹¹, Evangeline Pillebout ¹², Matthieu Groh ¹³, Romain Jouenne ¹⁴, Robin Dhote ¹⁵, Olivier Fain ¹⁶, Matthieu Ponsoye ¹⁷, Nicolas Noel ¹⁸, Nicolas Limal ¹⁹, Loïc Guillevin ¹¹, Luc Mouthon ¹¹ and Alexis Régent¹¹, ¹Cochin Hospital, Paris, France, ²Paris HEGP, Paris, France, ³Hotel Dieu Hospital, Paris, France, ⁴Service de néphrologie, Hôpital André Grégoire, 56 Boulevard de la Boissière Montreuil, France, Montreuil, Ile-de-France, France, ⁵Henri Mondor Hospital, Créteil, France, ⁶Tenon Hospital, Paris, France, ⁷Service de néphrologie, Hôpital Bichat-Claude Bernard, AP-HP, 46 rue Henri Huchard, Paris, France., Paris, Ile-de-France, France, ⁸Bicetre Hospital, Le Kremlin Bicetre, France, ⁹Service de néphrologie hémodialyse, Hôpital Ambroise Paré, APHP, 9 avenue Charles de Gaulle, Boulogne-Billancourt, France, Boulogne-Billancourt, Ile-de-France, France, ¹⁰APHP, Paris, France, ¹¹National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France, ¹²Service de néphrologie et transplantation, Hôpital Saint-Louis, AP-HP, 1 avenue Claude Vellefaux, Paris, France., Paris, Ile-de-France, France, ¹³Service de médecine interne, maladies systémiques, Hôpital Saint Louis, AP-HP, 1 avenue Claude Vellefaux, Paris, France, Paris, Ile-de-France, France, ¹⁴Service de médecine interne, Hôpital Bichat-Claude Bernard, AP-HP, 46 rue Henri Huchard, Paris, France, Paris, Ile-de-France, France, ¹⁵Service de médecine interne, Hôpital Avicenne, AP-PH, 125 rue de Stalingrad, Bobigny, France, Bobigny, Ile-de-France, France, ¹⁶Service de médecine interne, Hôpital Saint-Antoine, AP-HP, 184 rue du Faubourg Saint-Antoine, Paris, France, Paris, Ile-de-France, France, ¹⁷Service de médecine interne, Hopital Ambroise Paré, Boulogne-Billancourt, Boulogne-Billancourt, Ile-de-France, France, ¹⁸Service de médecine interne et immunologie clinique, Hôpital Bicêtre, AP-HP, 78 rue du Général Leclerc, Kremlin-Bicêtre, France, Kremil-Bicêtre, Ile-de-France, France, ¹⁹Service de médecine interne, Hôpital Henri-Mondor, AP-HP, 51 Avenue du Maréchal de Lattre de Tassigny, Créteil, France, Créteil, Ile-de-France, France
Background/Purpose: Induction therapy for severe ANCA-associated vasculitides (AAVs) is based on the combination of glucocorticoids and cyclophosphamide (CYC) or rituximab (RTX). For patients with severe…
Abstract Number: 1682 • 2019 ACR/ARP Annual Meeting
Inpatient Epidemiology of Granulomatosis with Polyangiitis in the United States
Patompong Ungprasert¹, Matthew Koster ², Wisit Cheungpasitporn ³, Karn Wijarnpreecha ⁴, Charat Thongprayoon ² and Paul Kroner ⁵, ¹Cleveland Clinic, Cleveland, OH, Bangkok, Thailand, ²Mayo Clinic Rochester, Rochester, MN, ³University of Mississippi Medical Center, Jackson, MS, ⁴Mayo Clinic Florida, Jacksonville, FL, ⁵Mayo Clinic Florida, Jacksonville
Background/Purpose: Granulomatosis with polyangiitis (GPA, formerly known and Wegener’s granulomatosis) is a major subtype of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis characterized by granulomatous inflammation of…
Abstract Number: 2626 • 2019 ACR/ARP Annual Meeting
Cell-free Mitochondrial DNA Levels in Granulomatosis with Polyangiitis
Lindsay Lally¹, Sebastian E. Sattui ², Jackie Finik ³, Augustine Choi ⁴, Rennie Rhee ⁵, Peter Merkel ⁶, W. Joseph McCune ⁷, E William St. Clair ⁸, Ulrich Specks ⁹, John Stone ¹⁰ and Robert Spiera ¹, ¹Hospital for Special Surgery, New York, NY, ²Hospital for Special Surgery, New York, ³Hospital for Special Surgery, New York, NY, New York, NY, ⁴Weill Cornell Medicine, New York, ⁵University of Pennsylvania, Division of Rheumatology, Philadelphia, PA, ⁶University of Pennsylvania, Philadelphia, PA, ⁷University of Michigan, Ann Arbor, MI, ⁸Duke, Raleigh, ⁹Mayo Clinic College of Medicine, Rochester, MN, ¹⁰Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA
Background/Purpose: Circulating cell-free mitochondrial DNA (mtDNA) is released during non-apoptotic processes such as NETosis. Increased levels of mtDNA are associated with autophagic dysfunction and inflammasome…
Abstract Number: 2788 • 2019 ACR/ARP Annual Meeting
Longitudinal Changes in the Nasal Microbiome and Disease Activity in Patients with Granulomatosis with Polyangiitis
Rennie Rhee¹, Antoine Sreih ², Jung-Jin Lee ³, Kyle Bittinger ³, Lisa Mattei ³, Ronald Collman ⁴ and Peter Merkel ², ¹University of Pennsylvania, Division of Rheumatology, Philadelphia, PA, ²University of Pennsylvania, Philadelphia, PA, ³Children's Hospital of Philadelphia, Philadelphia, ⁴University of Pennsylvania, Philadelphia
Background/Purpose: Microbial organisms have been theorized to contribute to disease activity in granulomatosis with polyangiitis (GPA) but little is known about what changes occur in…
Abstract Number: 2789 • 2019 ACR/ARP Annual Meeting
Neutrophil Extracellular Traps Induce Tissue-Invasive Macrophages in Granulomatosis with Polyangiitis Dominated by Ear, Nose and Throat Manifestations
Mitsuhiro Akiyama¹, Nour Ibrahim ², Markus Zeisbrich ¹, Peter Hwang ¹, Jorg Goronzy ¹ and Cornelia Weyand ¹, ¹Stanford University, Stanford, CA, ²Stanford University, Stanford University
Background/Purpose: Granulomatosis with polyangiitis (GPA) is a small vessel vasculitis characterized by acute and chronic tissue destruction in the nose, sinuses, lungs and kidneys. In…
Abstract Number: L04 • 2018 ACR/ARHP Annual Meeting
Pediatric Open-Label Clinical Study of Rituximab for the Treatment of Granulomatosis with Polyangiitis (GPA) and Microscopic Polyangiitis (MPA)
Paul Brogan¹, Gavin Cleary², Aimee O. Hersh³, Ozgur Kasapcopur⁴, Satyapal Rangaraj⁵, Rae S.M. Yeung⁶, Andrew Zeft⁷, Simone Melega⁸, Paul Brunetta⁹, Jennifer Cooper¹⁰, Pooneh Pordeli¹¹ and Patricia B. Lehane¹², ¹Infection Inflammation and Rheumatology, UCL GOL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ²Alder Hey Children's Hospital, Liverpool, United Kingdom, ³Pediatrics/Rheumatology, University of Utah, Salt Lake City, UT, ⁴Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey, ⁵Nottingham University Hospitals NHS Trust, Nottingham, United Kingdom, ⁶Paediatrics, Immunology and Medical Science, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada, ⁷Center for Pediatric Rheumatology & Immunology, The Cleveland Clinic Foundation, Cleveland, OH, ⁸F. Hoffmann-La Roche, Basel, Switzerland, ⁹Genentech, Inc., South San Francisco, CA, ¹⁰Pediatric Rheumatology, Univ. of California San Francisco, San Francisco, CA, ¹¹Hoffmann-La Roche Ltd., Mississauga, ON, Canada, ¹²Roche Products Ltd., Welwyn Garden City, United Kingdom
Background/Purpose: PePRS is a Phase IIa international, multicenter, open-label single arm study of rituximab in pediatric patients with newly diagnosed or relapsing GPA or MPA.…
Abstract Number: 1769 • 2018 ACR/ARHP Annual Meeting
Efficacy and Safety of Biomimic Rituximab in Granulomatosis with Polyangiitis – Experience from a Single Tertiary Care Centre in India
Aman Sharma¹, Sakshi Mittal², GSRSNK Naidu³, Saket Jha², Manish Rathi⁴, Vikas Sharma², Arghya Chattopadhyay⁵, Varun Dhir⁶, Kusum Sharma⁷, Ranjana Minz⁸, Ritambhra Nada⁹ and Sanjay Jain¹⁰, ¹Clinical Immunology and Rheumatology Services, Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India, Chandigarh, India, ²Internal Medicine, PGIMER, Chandigarh, India, ³PGIMER, cHANDIGARH, India, ⁴Department of Nephrology,, Postgraduate Institute of Medical Education and Research, Chandigarh, India, ⁵PGIMER, Chandigarh, India, ⁶Internal Medicine (Rheumatology Unit), Postgraduate Institute of Medical Education and Research, Chandigarh, India, Chandigarh, India, ⁷Department of Medical Microbiology,, PGIMER,, Chandigarh, India, ⁸Department of Immunopathology,, PGIMER,, Chandigarh, India, ⁹Postgraduate Institute of Medical Education and Research, Chandigarh, India, ¹⁰Postgraduate Institute of Medical Education and Research, Chandigarh, India, CHANDIGARH, India
Background/Purpose: Though rituximab(RTX) has become a standard of care for remission induction and maintenance in Granulomatosis with Polyangiitis(GPA), there is very limited data on the…
Abstract Number: 2720 • 2018 ACR/ARHP Annual Meeting
30-Day Hospital Readmission for Granulomatosis with Polyangiitis: Analysis from National Readmission Database
Yiming Luo¹, Changchuan Jiang¹, Ana Belen Arevalo Molina¹, Shane Murray¹, Maria Salgado² and Jiehui Xu³, ¹Department of Medicine, Mount Sinai St Luke's and Mount Sinai West Hospitals, Icahn School of Medicine at Mount Sinai, New York, NY, ²Department of Medicine, Jacobi Medical Center, Albert Einstein College of Medicine, New York, NY, ³Department of Biostatistics, Mailman School of Public Health, Columbia University Medical Center, New York, NY
Background/Purpose: Granulomatosis with polyangiitis (GPA) is a systemic vasculitis with multi-organ involvement which can lead to frequent hospitalizations. Our study is to investigate characteristics and…
Abstract Number: 2728 • 2018 ACR/ARHP Annual Meeting
Validation of the Draft Classification Criteria of Granulomatosis with Polyangiitis (GPA) Amongst Indian Patients with ANCA Associated Vasculitis
Aman Sharma¹, Adarsh MB², GSRSNK Naidu³, Manish Rathi⁴, Kusum Sharma⁵, Varun Dhir⁶, Ritambhra Nada⁷, Ranjana Minz⁸ and Sanjay Jain⁹, ¹Clinical Immunology and Rheumatology Services, Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India, Chandigarh, India, ²Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India, Chandigarh, India, ³PGIMER, cHANDIGARH, India, ⁴Department of Nephrology,, Postgraduate Institute of Medical Education and Research, Chandigarh, India, ⁵Department of Medical Microbiology,, PGIMER,, Chandigarh, India, ⁶Internal Medicine (Rheumatology Unit), Postgraduate Institute of Medical Education and Research, Chandigarh, India, Chandigarh, India, ⁷Postgraduate Institute of Medical Education and Research, Chandigarh, India, ⁸Department of Immunopathology,, PGIMER,, Chandigarh, India, ⁹Postgraduate Institute of Medical Education and Research, Chandigarh, India, CHANDIGARH, India
Background/Purpose: A draft classification criteria of GPA was proposed at ACR meeting in San Diego in 2017. The present criteria is a modification of ACR/EULAR…
Abstract Number: 2729 • 2018 ACR/ARHP Annual Meeting
Increased Risk of Cerebrovascular Accident Among Patients with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Systematic Review and Meta-Analysis of Cohort Studies
Patompong Ungprasert¹, Karn Wijarnpreecha² and Wisit Cheungpasitporn³, ¹Clinical Epidemiology Unit, Department of Research and Development, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand, ²Internal medicine, Bassett medical center, cooperstown, NY, ³Medicine, University of Mississippi Medical center, Jackson, MS
Increased Risk of Cerebrovascular Accident among Patients with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Systematic Review and Meta-analysis of Cohort StudiesAbstractBackground/Purpose: An increased risk of cardiovascular…
Abstract Number: 2737 • 2018 ACR/ARHP Annual Meeting
Opportunistic Infections in Medium and Small-Sized Vessel Vasculitis: Based on a Retrospectivestudy on 108 Patients
Raphaele Mestiri¹, Lafarge Antoine², Jean-Jacques Tudesq³, Marc Pineton de Chambrun⁴, Melanie Roriz⁵, Stéphane Bally⁶, Laurent Alric⁷, Damien Sène⁸, Thomas Quémeneur⁹, Cécile-Audrey Durel¹⁰, Brigitte Gilson¹¹, Alice Berezne¹², Anne-Marie Ruppert¹³, Raphaele Seror¹⁴, Philippe Guilpain¹⁵, Xavier Puéchal¹⁶, Luc Mouthon¹⁷, Loïc Guillevin¹⁶ and Benjamin Terrier¹⁸, ¹Internal Medicine, Cochin hospital, Paris, France, ²Medecine Interne, National Referral Center for Rare Systemic Autoimmune Diseases, Paris Cochin, France, Paris, France, ³Internal Medicine, CHU Montpellier, Montpellier, France, ⁴Université Pierre et Marie Curie, Paris, France, ⁵Université Paris-Diderot, Paris, France, ⁶Nephrology, CH Chambery, Chambery, France, ⁷Internal Medicine, CHU Toulouse, Toulouse, France, ⁸Lariboisière Hospital, Paris Diderot University, Paris, France, ⁹Department of Internal Medicine, CH of Valenciennes, France, Valenciennes, France, ¹⁰Internal Medicine, Hôpital Edouard Herriot, lyon, France, ¹¹Internal Medicine, CH Verdun, Verdun, France, ¹²Internal Medicine, CH Annecy, Annecy, France, ¹³Pulmonology, Tenon hospital, Paris, France, ¹⁴Hopitaux Universitaires Paris Sud, Kremlin Bicetre, France, Rheumatology, Université Paris Sud, Le Kremlin Bicetre, France, ¹⁵Montpellier, Montpellier, France, ¹⁶Department of Internal Medicine, INSERM Unité 1016, Centre de Référence pour les Maladies Auto-immunes Rares, Hôpital Cochin, Université Paris Descartes, Sorbonne Paris Cité, Paris, France, ¹⁷Department of Internal Medicine, INSERM Unité 1016, Centre de Référence pour les Maladies Auto-immunes Rares, National Referral Center for Rare Systemic Autoimmune Diseases, Paris Cochin, France, Paris, France, ¹⁸Centre de Référence des Maladies Auto-immunes et Systémiques Rares, Vascularites Nécrosantes et Sclérodermie Systémique, Service de Médecine Interne, Hôpital Cochin, APHP, INSERM U1016, Université Paris Descartes, Paris, France
Background/Purpose: Overall survival has been markedly improved during the last decades in systemic vasculitis. In contrast, the use of immunosuppressive agents led to an increased…
Abstract Number: 893 • 2017 ACR/ARHP Annual Meeting
Efficacy and Safety of Belimumab in Combination with Azathioprine for Remission Maintenance in Granulomatosis with Polyangiitis and Microscopic Polyangiitis: A Multicenter Randomized, Placebo-Controlled Study
David Jayne¹, Daniel Blockmans², Raashid Luqmani³, Beulah Ji⁴, Yulia Green⁵, Leanne Hall⁶, David Roth⁷ and Peter A. Merkel⁸, ¹Vasculitis and Lupus Clinic, Department of Medicine, University of Cambridge, Cambridge, United Kingdom, ²General Internal Medicine, University Hospital Gasthuisberg, Leuven, Belgium, ³Botnar Research Centre, University of Oxford, Oxford, United Kingdom, ⁴GSK Stockley Park, Uxbridge, United Kingdom, ⁵GSK Stockley Park, Stockley Park, United Kingdom, ⁶GSK Stevenage, Stevenage, United Kingdom, ⁷GSK Collegeville, Collegeville, PA, ⁸Division of Rheumatology, University of Pennsylvania, Philadelphia, MN
Background/Purpose: GPA (Wegener’s) and MPA are organ- and life-threatening systemic vasculitides characterized by the presence of ANCA-associated vasculitis (AAV), implicating B cells in disease pathogenesis.…
Abstract Number: 1010 • 2017 ACR/ARHP Annual Meeting
Identification of Circulating Biomarkers of Disease Activity and Organ Involvement in ANCA-Associated Vasculitis By Targeted Proteomics
Jun Ishizaki¹, Ayako Takemori², Koichiro Suemori¹, Takuya Matsumoto¹, Yoko Akita¹, Masaki Yasukawa¹, Nobuaki Takemori² and Hitoshi Hasegawa¹, ¹Department of Hematology, Clinical Immunology and Infectious Diseases, Ehime University Graduate School of Medicine, Ehime, Japan, ²Division of Proteomics Research, Proteo-Science Center, Ehime University, Ehime, Japan
Background/Purpose: Targeted proteomics, which involves quantitative analysis of targeted proteins using selected reaction monitoring (SRM) mass spectrometry, has emerged as a new methodology for discovery…

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