Abstracts tagged "Granulomatosis with Polyangiitis (GPA)"

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: 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: 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…
Abstract Number: 1421 • ACR Convergence 2020
Clinical Features, Disease Activity and Prognosis of ANCA-Associated Vasculitis in US African Americans
Luis Palomino¹, Angelo Gaffo², Sebastian Sattui³ and Dongmei Sun², ¹University of Alabama at Birmingham Hospital, Birmingham, AL ²University of Alabama at Birmingham, Birmingham, AL, ³Hospital for Special Surgery, New York, NY
Background/Purpose: ANCA-associated vasculitis (AAV), including Granulomatosis with Polyangiitis (GPA), Eosinophilic Granulomatosis with Polyangiitis (EGPA) and Microscopic Polyangiitis (MPA), affect mostly Caucasians of European descent. Clinical…
Abstract Number: 1425 • ACR Convergence 2020
Reduced Risk of Cardiovascular Diseases Events with Renal Transplantation in Granulomatosis with Polyangiitis in the United States: Data from the US Renal Data System
Zachary Wallace¹, April Jorge², Xiaoqing Fu³, Yuqing Zhang⁴ and Hyon Choi⁵, ¹Massachusetts General Hospital, Newton, MA, ²Massachusetts General Hospital, Boston, MA, ³Massachusetts General Hospital, Boston, ⁴Massachusetts General Hospital, Quincy, MA, ⁵Massachusetts General Hospital, Department of Medicine, Division of Rheumatology, Lexington, MA
Background/Purpose: Granulomatosis with polyangiitis (GPA) is a common cause of glomerulonephritis and leads to end-stage renal disease (ESRD) in approximately 25% of patients. Both GPA…
Abstract Number: 1430 • ACR Convergence 2020
The Role of Lung Biopsy in Pediatric ANCA-associated Vasculitis
Edouard Sayad¹, Tiphanie Vogel¹, David Moreno McNeill¹, Nahir Cortes-Santiago¹, David Spielberg¹, Kalyani Patel¹ and Manuel Silva Carmona¹, ¹Baylor College of Medicine/Texas Children's Hospital, Houston, TX
Background/Purpose: Anca-associated vasculitis (AAV) is characterized by vascular inflammation in multiple organs. The diagnosis can be made clinically using a number of different criteria. The…
Abstract Number: 1438 • ACR Convergence 2020
Rituximab Immunogenicity in ANCA-associated Vasculitis (RITUXIMAV)
Jason Springer¹ and Ryan Funk², ¹University of Kansas Medical Center, Overland Park, KS, ²UNIVERSITY OF KANSAS, Kansas City, KS
Background/Purpose: Rituximab (RTX), an anti-CD20 chimeric monoclonal antibody, has been shown to be an effective maintenance therapy for granulomatosis with polyangitiis (GPA) and microscopic polyangiitis…
Abstract Number: 1524 • ACR Convergence 2020
Interstitial Lung Disease, Kidney Inflammation and Myositis Are Induced by Transfer of PBMC Derived from Systemic Sclerosis Patients into Rag2-/-/ IL2rg-/- mice
Xiaoyang Yue¹, Frank Petersen¹, Xinhua Yu¹, Gabriela Riemekasten², Peter Lamprecht³, Antje Müller³ and Junping Yin⁴, ¹Priority Area Asthma & Allergy, Research Center Borstel, Airway Research Center North (ARCN), Members of the German Center for Lung Research (DZL), Borstel, Germany, ²University of Lübeck, Department of Rheumatology and Clinical Immunology,, Lübeck, Germany, ³University of Lübeck, Department of Rheumatology and Clinical Immunology, Lübeck, Germany, ⁴1 Priority Area Asthma & Allergy, Research Center Borstel, Airway Research Center North (ARCN), Members of the German Center for Lung Research (DZL),, Borstel, Germany
Background/Purpose: To explore the pathogenic potential of lymphocytes in systemic sclerosis (SSc) and granulomatosis with polyangiitis (GPA), a humanized mouse model was generated by transferring…
Abstract Number: 2048 • ACR Convergence 2020
Comparison of Two Rituximab Regimens for Induction of Remission in Antineutrophil Cytoplasm Antibody-associated Vasculitis: Systematic Review and Meta-analysis
Valerie Benard¹, Cynthia Farhat², Melissa Zarandi-Nowroozi², Madeleine Durand³, Christian Pagnoux⁴, Pierre Charles⁵, Xavier Puechal⁶, Loïc Guillevin⁷ and Jean-Paul Makhzoum¹, ¹Vasculitis Clinic, Canadian Network for Research on Vasculitides (CanVasc), Department of Internal Medicine, Hopital du Sacre-Coeur de Montreal, University of Montreal, Montreal, QC, Canada, ²Department of Medecine, University of Montreal, Montreal, QC, Canada, ³Department of Internal Medicine, Centre Hospitalier de l’Universite de Montreal (CHUM) and Centre de Recherche du Centre Hospitalier de l’Universite de Montreal (CRCHUM), Montreal, QC, Canada, ⁴Vasculitis Clinic, Canadian Network for Research on Vasculitides (CanVasc), Department of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, ⁵Department of Internal Medicine, Institut Mutualiste Montsouris, Paris, France, ⁶National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris-Descartes University, Paris, France, ⁷Department of Internal Medecine, National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris-Descartes University, Paris, France
Background/Purpose: Organ or life-threatening granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), two of the antineutrophil cytoplasm antibody-associated vasculitis (AAV), are treated with cyclophosphamide or…

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