Abstracts tagged "Vasculitis"

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…
Abstract Number: 0509 • ACR Convergence 2020
Classification of Patients with Relapsing Polychondritis Based on Somatic Mutations in UBA1
Marcela Ferrada¹, Keith Sikora², Sinisa Savic³, Yiming Luo⁴, Kristina Wells⁵, Emily Rose¹, Kaitlin Quinn⁶, Wendy Goodspeed¹, Anne Jones⁷, Mimi Le⁸, Amanda Ombrello⁹, Zuoming Deng¹⁰, Massimo Gadina¹¹, Wanxia Tsai¹², Ivona Aksentijevich⁷, Daniel Kastner¹³, David Beck¹⁴ and Peter C. Grayson¹⁵, ¹Systemic Autoimmunity Branch, Vasculitis Translational Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ²National Institutes of Health Clinical Center, Bethesda, MD, ³University of Leeds, England, United Kingdom, ⁴Rheumatology Fellowship and Training Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, ⁵Systemic Autoimmunity Branch, Vasculitis Translational Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, ⁶Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Washington, DC, ⁷National Human Genome Research Institute, Bethesda, MD, ⁸Oregon Health & Science University, Portland, ⁹National Institutes of Health, Bethesda, ¹⁰National Institute of Arthritis Musculoskeletal and Skin diseases, Bethesda, ¹¹National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), NIH, Bethesda, MD, ¹²National Institute of Arthritis Musculoskeletal and Skin diseases, Bethesda, MD, ¹³National Human Genome Research Institute (NHGRI), NIH, Bethesda, MD, ¹⁴National Human Genome Research Institute, Bethesda, ¹⁵Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Bethesda, MD
Background/Purpose: Relapsing polychondritis (RP) is a rare, heterogenous, systemic inflammatory disease with a predilection for cartilaginous structures. Recently we discovered myeloid-restricted somatic mutations in UBA1 in…
Abstract Number: 1429 • ACR Convergence 2020
An Evaluation of Real World Use of Biologics in Rare Systemic Vasculitides During Routine Clinical Care in the US
Kathryn Starzyk¹, Kimberly Milberg¹, Ashish Deshpande¹ and Gary Curhan¹, ¹OM1, Inc, Boston, MA
Background/Purpose: The mainstay of treatment for vasculitis has been corticosteroids and other cytotoxic and immunosuppressive medications (e.g., cyclophosphamide, methotrexate) to promptly achieve and maintain remission.…
Abstract Number: 1922 • ACR Convergence 2020
Association of Leukocyte Populations in Peripheral Blood and Arterial Wall Inflammation Assessed by FDG-PET in Takayasu’s Arteritis and Giant Cell Arteritis
K. Bates Gribbons¹, Kaitlin Quinn², Mark Ahlman³, Peter Merkel⁴ and Peter C. Grayson⁵, ¹McGovern Medical School UT Houston, Houston, TX, ²Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Bethesda, ³National Institutes of Health, Clinical Center, Radiology and Imaging Sciences, Bethesda, MD, ⁴University of Pennsylvania, Philadelphia, PA, ⁵Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Bethesda, MD
Background/Purpose: Positron emission tomography (PET) is useful to demonstrate fluorodeoxyglucose (FDG) uptake in the large arteries in both Takayasu’s arteritis (TAK) and giant cell arteritis…
Abstract Number: 2052 • ACR Convergence 2020
Extended Follow-Up of Patients Recruited to a Randomized, Controlled Trial of Rituximab versus Azathioprine After Induction of Remission with Rituximab for Patients with ANCA-Associated Vasculitis and Relapsing Disease
Rona Smith¹, David Jayne² and Peter A. Merkel³, ¹University of Cambridge, Cambridge, United Kingdom, ²Department of Medicine, Vasculitis and Lupus Research Group, University of Cambridge, Cambridge, UK, Cambridge, United Kingdom, ³Division of Rheumatology University of Pennsylvania, Philadelphia, PA
Background/Purpose: Rituximab is an effective therapy for induction of remission in ANCA-associated vasculitis (AAV). However, the effect of rituximab is not sustained, and subsequent relapse…
Abstract Number: 051 • 2020 Pediatric Rheumatology Symposium
Pediatric Providers’ Perspectives on Suspected Immune-Mediated Diffuse Alveolar Hemorrhage and Clinical Care Pathways
Jessica Bloom¹ and Robert Fuhlbrigge ², ¹Children's Hospital Colorado, Aurora, Colorado, ²University of Colorado, Aurora
Background/Purpose: Immune-mediated diffuse alveolar hemorrhage (iDAH) is a life-threatening complication of pediatric rheumatologic diseases such as vasculitis, systemic lupus erythematosus, and antiphospholipid antibody syndrome. Delay…
Abstract Number: 068 • 2020 Pediatric Rheumatology Symposium
Development of CARRA Consensus Treatment Plans for Severe ANCA-associated Vasculitis – Final CARRA-wide Consensus
Linda Wagner-Weiner ¹, Vidya Sivaraman ², Karen James ³, Eric Yen ⁴, Kathleen O'Neil ⁵, Dana Gerstbacher ⁶, Ann Marie Szymanski⁷, Kimberly Morishita ⁸ and David Cabral ⁹ for the CARRA investigators, ¹University of Chicago, Chicago, Illinois, ²Division of Rheumatology, Nationwide Children's Hospital, Bexley, ³University of Utah, 84113, Utah, ⁴UCLA, Los Angeles, California, ⁵University of Indiana School of Medicine, Indianapolis, Indiana, ⁶Stanford University School of Medicine, Palo Alto, California, ⁷Tampa, Florida, ⁸University of British Columbia - Vancouver, Vancouver, British Columbia, Canada, ⁹BC Children's Hospital and University of British Columbia, Vancouver, British Columbia, Canada
Background/Purpose: Randomized trials for pediatric ANCA-associated vasculitis (ped-AAV) have not been feasible because of its rarity, therefore adult treatment strategies are usually adapted for children.…
Abstract Number: 084 • 2020 Pediatric Rheumatology Symposium
Kawasaki Disease Shock Syndrome: A Single Center Cohort
Monica Bray¹, Jennifer Rammel ², Andrea Ramirez ³, Kristen Sexson ⁴, Fong Lam ⁵, Eyal Muscal ¹ and Marietta De Guzman ³, ¹Section of Rheumatology, Department of Pediatrics, Baylor College of Medicine, Houston, ²Section of Nephrology and Rheumatology, Department of Pediatrics, University of Florida Health Jacksonville, Jacksonville, Florida, ³Section of Rheumatology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas, ⁴Section of Cardiology, Department of Pediatrics, Baylor College of Medicine, Houston, ⁵Section of Critical Care, Department of Pediatrics, Baylor College of Medicine, Houston
Background/Purpose: Kawasaki disease (KD) is a well described vasculitis of childhood that is the leading cause of acquired heart disease in developed countries. Kawasaki disease…
Abstract Number: 102 • 2020 Pediatric Rheumatology Symposium
Implications of Positive Tests for ANCA in a Pediatric Population
Karen James¹, Peter Merkel ² and Aimee Hersh ³, ¹University of Utah, 84113, Utah, ²University of Pennsylvania, Philadelphia, ³University of Utah Primary Children's Hospital, Salt Lake City
Background/Purpose: Testing for ANCA, particularly performed by ELISA (anti-MPO/PR3) is highly sensitive and specific for ANCA-associated vasculitis (AAV). However ANCA testing may be used in…
Abstract Number: 138 • 2020 Pediatric Rheumatology Symposium
Serious Infection Risk in Pediatric Patients with Low Immunoglobulin Levels Following Rituximab Treatment for Granulomatosis with Polyangiitis (GPA) or Microscopic Polyangiitis (MPA)
Simone Melega ¹, Paul Brogan², Gavin Cleary ³, Aimee Hersh ⁴, Ozgur Kasapcopur ⁵, Satyapal Rangaraj ⁶, Rae Yeung ⁷, Andrew Zeft ⁸, Jennifer Cooper ⁹, Pooneh Pordeli ¹⁰, Petra Kirchner ¹¹ and Patricia Lehane ¹², ¹F. Hoffmann-La Roche, Ltd., Basel, Switzerland, ²UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ³Alder Hey Children's Hospital, Liverpool, United Kingdom, ⁴University of Utah Primary Children's Hospital, Salt Lake City, ⁵Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Cerrahpasa, Turkey, ⁶Nottingham University Hospitals NHS Trust, Nottingham, United Kingdom, ⁷The Hospital for Sick Children, Toronto, Canada, ⁸The Cleveland Clinic - Center for Pediatric Rheumatology & Immunology, Cleveland, Ohio, ⁹University of Colorado, Children's Hospital Colorado, Denver, ¹⁰F. Hoffmann-La Roche, Ltd., Mississauga, Canada, ¹¹F. Hoffmann-La Roche Ltd., Birsfelden, Switzerland, ¹²Roche Products Ltd., Welwyn Garden City, United Kingdom
Background/Purpose: Low immunoglobulin (Ig) levels can occur after rituximab treatment, but the clinical significance is not completely understood. Not all patients (pts) who develop low…
Abstract Number: 312 • 2019 ACR/ARP Annual Meeting
Timely Glucocorticoid Tapering in Vasculitis: A Need for Improved Knowledge Translation to Limit Toxicity
Arielle Mendel¹, Daniel Ennis ², Shirley Lake ³, Simon Carette ⁴ and Christian Pagnoux ⁴, ¹Mount Sinai Hospital, Toronto, ON, Canada, ²University of Toronto, Toronto, ON, Canada, ³Division of Rheumatology, Sunnybrook Hospital, Toronto, ON, Canada, ⁴Mount Sinai Hospital and University Health Network, Toronto, ON, Canada
Background/Purpose: High dose glucocorticoids (GC) are part of the initial treatment of ANCA-associated (AAV) and large vessel vasculitides (LVV). Prompt subsequent tapering limits toxicity. Adherence…
Abstract Number: 1679 • 2019 ACR/ARP Annual Meeting
Adaptive Study Design of a Randomized, Multicenter, 2-Part Phase 2 Trial of Replacement of Glucocorticoids by IFX-1, a C5a Inhibitor, in Active Granulomatosis with Polyangiitis and Microscopic Polyangiitis
Peter Merkel¹, Bernhard Hellmich ², David Jayne ³, Simon Rückinger ⁴, Zsuzsanna Tamas ⁵, Claus Thielert ⁵ and Othmar Zenker ⁶, ¹University of Pennsylvania, Philadelphia, PA, ²Department of Internal Medicine, Rheumatology and Immunology, Vasculitis-Center Tübingen-Kirchheim, Medius Klinik Kirchheim, University of Tübingen, Kirchheim-Teck, Germany, Kirchheim, Germany, ³Vasculitis and Lupus Clinic, Addenbrooke’s Hospital, University of Cambridge, UK, Cambridge, United Kingdom, ⁴Metronomia Clinical Research GmbH, Munich, Germany, ⁵InflaRx GmbH, Planegg, Germany, ⁶InflaRx GmbH, Jena, Germany
Background/Purpose: Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are life-threatening rare autoimmune diseases are both forms of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). Standard of…
Abstract Number: 2627 • 2019 ACR/ARP Annual Meeting
Association of Work Productivity Assessed by Absenteeism and Presenteeism with Disease Activity, Damage and Health-related Quality of Life in Patients with ANCA-associated Vasculitis
Shinya Hirahara¹, Yasuhiro Katsumata ¹, Ken-ei Sada ², Hiroko Nagafuchi ³, Eiichi Tanaka ¹ and Masayoshi Harigai ¹, ¹Department of Rheumatology, Tokyo Women's Medical University School of Medicine, Shinjuku-ku, Tokyo, Japan, ²Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Kitaku, Okayama, Japan, ³Division of Rheumatology and Allergology, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki City, Kanagawa, Japan
Background/Purpose: Chronic inflammatory diseases such as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) substantially affects patients’ daily lives, including their ability to work. Although the management…
Abstract Number: 806 • 2019 ACR/ARP Annual Meeting
A Randomized, Controlled Trial of Rituximab versus Azathioprine After Induction of Remission with Rituximab for Patients with ANCA-associated Vasculitis and Relapsing Disease
Rona Smith¹, David Jayne ² and Peter Merkel ³, ¹University of Cambridge, Cambridge, England, United Kingdom, ²Vasculitis and Lupus Clinic, Addenbrooke’s Hospital, University of Cambridge, UK, Cambridge, United Kingdom, ³University of Pennsylvania, Philadelphia
Background/Purpose: Rituximab is an effective therapy for induction of remission in ANCA-associated vasculitis (AAV). However, the effect of rituximab is not sustained, and relapse rates…
Abstract Number: 1680 • 2019 ACR/ARP Annual Meeting
Design of a Phase 2, Multicenter, Randomized, Double-Blind, Placebo-Controlled Trial of 2 Different Dose Regimens of IFX-1, a C5a Inhibitor, as an Add-On Therapy for Granulomatosis with Polyangiitis or Microscopic Polyangiitis
Peter Merkel¹, Bernhard Hellmich ², David Jayne ³, Simon Rückinger ⁴, Zsuzsanna Tamas ⁵, Claus Thielert ⁵ and Othmar Zenker ⁶, ¹University of Pennsylvania, Philadelphia, PA, ²Department of Internal Medicine, Rheumatology and Immunology, Vasculitis-Center Tübingen-Kirchheim, Medius Klinik Kirchheim, University of Tübingen, Kirchheim-Teck, Germany, Kirchheim, Germany, ³Vasculitis and Lupus Clinic, Addenbrooke’s Hospital, University of Cambridge, UK, Cambridge, United Kingdom, ⁴Metronomia Clinical Research GmbH, Munich, Germany, ⁵InflaRx GmbH, Planegg, Germany, ⁶InflaRx GmbH, Jena, Germany
Background/Purpose: Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are life-threatening rare autoimmune diseases to anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Standard of care (SOC)…

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