Abstracts tagged "Granulomatosis with Polyangiitis (GPA)"

Abstract Number: 0686 • ACR Convergence 2023
Safety and Efficacy of Avacopan in Patients 65 Years and Older with ANCA-Associated Vasculitis
David Jayne¹, Duvuru Geetha², Christian Pagnoux³, Sebastian Sattui⁴ and Peter Merkel⁵, ¹University of Cambridge, Cambridge, United Kingdom, ²Johns Hopkins University, Baltimore, MD, ³Mount Sinai Hospital, Toronto, ON, Canada, ⁴University of Pittsburgh, Pittsburgh, PA, ⁵University of Pennsylvania, Philadelphia, PA
Background/Purpose: Older adults are at increased risk of glucocorticoid (GC)-related toxicity; minimization of GCs is a major focus for treatment of patients with ANCA-associated vasculitis…
Abstract Number: 0689 • ACR Convergence 2023
Use of Avacopan in Patients with ANCA-associated Vasculitis and Estimated Glomerular Filtration Rate < 15 mL/min/1.73m2
Bryce Barr, Kim Cheema, Aurore Fifi-Mah, Stephanie Garner, Louis Girard and Jeffrey Ma, University of Calgary, Calgary, AB, Canada
Background/Purpose: Glomerulonephritis represents a severe manifestation of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) and is associated with substantial morbidity and mortality. Therefore, maximal recovery…
Abstract Number: 0690 • ACR Convergence 2023
Treating Patients with ANCA-Associated Vasculitis and Very Severe Renal Injury with an Intensified B Cell Depletion Therapy: Comparison with a Control Cohort Receiving a Conventional Therapy
Dario Roccatello¹, Savino Sciascia², SILVIA GRAZIETTA FODDAI³, gIACOMO QUATTROCCHIO⁴, MASSIMO RADIN³, IRENE CECCHI³, ALICE BARINOTTI³, ELENA RUBINI³, MICHELA FERRO⁴, EMANUELE DE SIMONE⁴, carla naretto⁴, ANTONELLA BARRECA⁵, ANDREA SAMMARTINO⁴, DANIELA ROSSI⁴ and ROBERTA FENOGLIO⁴, ¹University of Torino, Torino, Italy, ²University of Turin, Torino, Italy, ³University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy, ⁴University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, San Giovanni Bosco Hub Hospital, Turin, Italy, ⁵Pathology Unit, Città della Salute e della Scienza, Turin, Italy
Background/Purpose: Rituximab (RTX), an anti-CD20 monoclonal antibody, has shown to be an effective induction treatment for small-vessel vasculitides associated with antineutrophil cytoplasm antibodies (AAV) in…
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…
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: 1080 • ACR Convergence 2022
Performance of Risk Prediction Models for Relapse and Severe Infection at the End of 18-month Rituximab Maintenance Therapy in ANCA-associated Vasculitides: Data from the 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: Long-term follow-up analysis of the three MAINRITSAN trials confirmed the efficacy of rituximab (RTX) in preventing relapses in ANCA-associated vasculitis (AAV), especially RTX administration…
Abstract Number: 1082 • ACR Convergence 2022
Outcomes of Methotrexate as an Initial Induction and Maintenance Regimen for Localized Granulomatosis with Polyangiitis: A Retrospective Review
Pratyusha Banik¹, Marcia Friedman², Daniela Ghetie³, Dongseok Choi⁴ and Margaret Brophy¹, ¹Oregon Health & Science University, Portland, OR, ²Oregon Health and Science University, Division of Arthritis and Rheumatic Diseases, Portland, OR, ³Oregon Health & Science University, Division of Arthritis and Rheumatic Diseases, Lake Oswego, OR, ⁴OHSU-PSU School of Public Health, Portland, OR
Background/Purpose: While there is established data regarding the induction and maintenance treatment of systemic or organ threatening granulomatosis with polyangiitis (GPA), there is limited data…
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)…

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