Abstracts tagged "Vasculitis"

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)…
Abstract Number: 2630 • 2019 ACR/ARP Annual Meeting
A Dutch Consensus Statement on the Diagnosis and Treatment of ANCA Associated Vasculitis
Ebru Dirikgil¹, Peter Verhoeven ², Sander Tas ³, Abraham Rutgers ⁴, Hein Bernelot Moens ⁵, Jacob van Laar ⁶, Willem Jan Bos ⁷ and Onno Teng ¹, ¹LUMC, Leiden, Netherlands, ²The Dutch Vasculitis Foundation, Silvolde, Netherlands, ³Amsterdam Rheumatology & immunology Center, Amsterdam UMC/University of Amsterdam, Amsterdam, Netherlands, ⁴University Medical Center Groningen, Groningen, Netherlands, ⁵Ziekenhuisgroep Twente, Almelo, Netherlands, ⁶UMC Utrecht, Department of Rheumatology & Clinical Immunology, Utrecht, The Netherlands, Utrecht, Netherlands, ⁷St. Antoniusziekenhuis, Nieuwegein, Netherlands
Background/Purpose: Several guidelines have been published on the diagnosis and treatment of anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV). These guidelines provide an evidence-based approach…
Abstract Number: 810 • 2019 ACR/ARP Annual Meeting
Small Vessel Vasculitis Syndrome with Autoinflammation Caused by De Novo Mutations in LYN Kinase
Adriana de Jesus¹, Gina Montealegre Sanchez ², Helen Freeman ³, Neil Martin ⁴, Ebun Omoyinmi ⁵, Katherine Calvo ⁶, Richard Chyi-chia Lee ⁷, Murray Passo ⁸, Natasha Ruth ⁸, David Kleiner ⁷, Yan Huang ⁹, Nirali Shah ¹⁰, Paul Brogan ¹¹, SuJin Hwang ¹², HyeSun Kuehn ¹², Sergio Rosenzweig ¹², Zuoming Deng ¹³, Anna Huttenlocher ¹⁴, Susan Moir ¹⁵, Douglas Kuhns ¹⁶ and Raphaela Goldbach-Mansky ¹⁷, ¹Translation Autoinflammatory Diseases Section/NIAID/NIH, Silver Spring, MD, ²Translational Autoinflammatory Disease Section/NIAID/NIH, Bethesda, ³Raigmore Hospital, Inverness, United Kingdom, ⁴Royal Hospital for Children, Glasgow, United Kingdom, ⁵University College London Institute of Child Health, London, United Kingdom, ⁶Hematology Service/Department of Laboratory Medicine/NIH, Bethesda, MD, ⁷Laboratory of Pathology/NCI/NIH, Bethesda, MD, ⁸Medical University of South Carolina, Charleston, SC, ⁹Translational Autoinflammatory Disease Section/NIAID/NIH, Bethesda, MD, ¹⁰Pediatric Oncology Branch/NCI/NIH, Bethesda, MD, ¹¹Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, ¹²Immunology Service/Department of Laboratory Medicine/NIH, Bethesda, MD, ¹³Biomining and Discovery Section/NIAMS/NIH, Bethesda, MD, ¹⁴Department of Pediatrics/University of Wisconsin, Madison, WI, ¹⁵Immunopathogenesis Section/NIAID/NIH, Bethesda, MD, ¹⁶Collaborative Clinical Research Branch/NIAID/NIH, Bethesda, MD, ¹⁷Translational Autoinflammatory Diseases Section/NIAID/NIH, Bethesda, MD
Background/Purpose: Lyn kinase is a member of the Src family of non-receptor tyrosine-protein kinases that modifies signals from various cell surface receptors and regulates innate…
Abstract Number: 1681 • 2019 ACR/ARP Annual Meeting
The Association of Reduced Low-Density Lipoprotein (LDL) Cholesterol Levels with ANCA-Associated Vasculitis (AAV)
Zachary Wallace¹, Xiaoqing Fu ¹, Yuqing Zhang ², John Stone ³ and Hyon K. Choi ², ¹Massachusetts General Hospital, Boston, ²Massachusetts General Hospital, Boston, MA, ³Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA
Background/Purpose: ANCA-associated vasculitis (AAV) is a small vessel vasculitis associated with an intense inflammatory state. AAV patients are at a 2-fold higher risk of cardiovascular…
Abstract Number: 2632 • 2019 ACR/ARP Annual Meeting
Changing Trends in the Management of ANCA-associated Vasculitis at an Academic Medical Center
Sana Afroz¹ and Najia Shakoor ², ¹RUMC, Chicago, IL, ²Rush University Medical Center, Chicago, IL
Background/Purpose: For years, cyclophosphamide (CYC) has been the primary treatment for severe anca-associated vasculitides (AAV) and has significantly improved disease related mortality. In addition, maintenance…
Abstract Number: 869 • 2019 ACR/ARP Annual Meeting
Clinical Manifestations of Patients with Eosinophilic Granulomatosis with Polyangiitis in a Large North American Cohort
Irena Doubelt¹, David Cuthbertson ², Simon Carette ¹, Nader A. Khalidi ³, Curry L. Koening ⁴, Carol Langford ⁵, Carol A. McAlear ⁶, Larry W. Moreland ⁷, Paul Monach ⁸, Philip Seo ⁹, Ulrich Specks ¹⁰, Antoine Sreih ¹¹, Steven Ytterberg ¹⁰, Peter A. Merkel ¹², Christian Pagnoux ¹ and VCRC Vasculitis Clinical Research Consortium ¹³, ¹Mount Sinai Hospital and University Health Network, Toronto, ON, Canada, ²University of South Florida, Tampa, FL, ³McMaster University, Hamilton, ON, Canada, ⁴University of Utah Hospital, Salt Lake City, UT, ⁵Cleveland Clinic, Cleveland, OH, ⁶University of Pennsylvania - VCRC Project Manager, Philadelphia, PA, ⁷University of Pittsburgh, Pittsburgh, PA, ⁸Brigham and Women's Hospital, Boston, MA, ⁹Johns Hopkins Medicine, Baltimore, MD, ¹⁰Mayo Clinic College of Medicine, Rochester, MN, ¹¹University of Pennsylvania, Philadelphia, PA, ¹²Univeristy of Pennsylvania, Philadelphia, PA, ¹³University of Pennsylvania, Division of Rheumatology, philadelphia
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, systemic necrotizing small-vessel vasculitis, with only a few published North American series. This project aimed to…
Abstract Number: 1687 • 2019 ACR/ARP Annual Meeting
Dramatic but Suspensive Effect of interleukin-1 Inhibitors on Persistent Urticarial Vasculitis
Thomas Bettuzzi ¹, Alban Deroux ², Marie Jachiet ³, Meryem-Maud Farhat ⁴, Julien Wipff ¹, Marc Fabre ⁵, Laurence Bouillet ², Nora Kramkimel ¹, Selim Aractingi ¹, Nicolas Dupin ¹ and Benjamin Terrier⁶, ¹Cochin Hospital, Paris, France, ²CHU, Grenoble, France, ³Saint Louis Hospital, Paris, France, ⁴CHRU, Lille, France, ⁵CH, Bourgoin-Jailleu, France, ⁶National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France
Background/Purpose: Urticarial vasculitis (UV) is a rare disease characterized by dermal capillary inflammation responsible for long-lasting urticarial lesions. UV can be separated into 2 different…
Abstract Number: 2634 • 2019 ACR/ARP Annual Meeting
ANCA Response upon Rituximab or Cyclophosphamide in ANCA-associated Vasculitis Patients
Laura van Dam¹, Ebru Dirikgil ², Edwin Bredewold ², Argho Ray ², Ton Rabelink ², Cees van Kooten ² and Onno Teng ², ¹LUMC, Leiden, Zuid-Holland, Netherlands, ²LUMC, Leiden, Netherlands
Background/Purpose: Recent studies have demonstrated that in patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) with successful remission-induction (RI) after cyclophosphamide (CYC), maintenance treatment with…
Abstract Number: 871 • 2019 ACR/ARP Annual Meeting
ANCA-Associated Vasculitis Management in the United States: Data from the RISE Registry
Zachary Wallace¹, Huifeng Yun ², Jeffrey Curtis ², Shuo Yang ², Lang Chen ², John Stone ³ and Hyon K. Choi ⁴, ¹Massachusetts General Hospital, Boston, ²University of Alabama at Birmingham, Birmingham, AL, ³Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA, ⁴Massachusetts General Hospital, Boston, MA
Background/Purpose: The management of ANCA-associated vasculitis (AAV) evolved substantially in recent years because of evidence supporting the efficacy of various treatment regimens. As such, treatment…
Abstract Number: 1688 • 2019 ACR/ARP Annual Meeting
Glucocorticoids Plus Rituximab versus Glucocorticoids Plus Placebo in Non-infectious Active Mixed Cryoglobulinemia Vasculitis: Results of a Placebo-Controlled Randomized Trial
Benjamin Terrier¹, Jonathan London ², Fabrice Bonnet ³, Diane Cerutti ⁴, Nathalie Costedoat-Chalumeau ⁵, Elisabeth Diot ⁶, Yasmina Ferfar ⁷, Aurélie Hummel ⁸, Gilles Kaplanski ⁹, Isabelle Marie ¹⁰, Thomas Quémeneur ¹¹, Patricia Rullier ¹², Patricia Senet ¹³, Noémie Le Gouellec ¹⁴, Julie Lejeune ⁷, David Saadoun ¹⁵ and Patrice Cacoub ¹⁵, ¹National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France, ²Croix Saint-Simon Hospital, Paris, France, ³CHU, Bordeaux, France, ⁴CH, Toulon, France, ⁵Cochin University Hospital, Paris, France, ⁶APHP, Tours, France, ⁷Pitié-Salpétrière Hospital, Paris, France, ⁸APHP, Paris, France, ⁹CHU, Marseille, France, ¹⁰CHU, Rouen, France, ¹¹CH Valenciennes, Valenciennes, France, ¹²CHU, Montpellier, France, ¹³Tenon Hospital, Paris, France, ¹⁴CH, Valenciennes, France, ¹⁵AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Department of Internal Medicine and Clinical Immunology, F-75013, Paris, France, Paris, France
Background/Purpose: A previous retrospective study suggested superiority of glucocorticoids (GCs) plus rituximab (RTX) compared to GCs alone to induce complete clinical response in non-infectious cryoglobulinemia…
Abstract Number: 2635 • 2019 ACR/ARP Annual Meeting
Accuracy of Self-Reported Diagnosis of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis
Tanaz Kermani¹, Jason Springer ², Antoine Sreih ³, Dianne Shaw ⁴, Kalen Young ⁵, Cristina Burroughs ⁶ and Peter Merkel ³, ¹University of California Los Angeles, Los Angeles, CA, ²Kansas University Medical Center, Kansas, MO, ³University of Pennsylvania, Philadelphia, PA, ⁴Vasculitis Foundation, North Carolina, ⁵Vasculitis Foundation, Kansas City, MO, ⁶University of South Florida, Tampa, FL
Background/Purpose: To determine the reliability of self-reported diagnosis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with…
Abstract Number: 873 • 2019 ACR/ARP Annual Meeting
Survival After Lung Transplantation in Adults with Primary Systemic Vasculitides: Analysis of the United Network of Organ Sharing (UNOS) Database
Sebastian E. Sattui¹, Zafir Abutalib ², Jackie Finik ² and Robert Spiera ³, ¹Hospital for Special Surgery, New York, ²Hospital for Special Surgery, New York, NY, New York, NY, ³Hospital for Special Surgery, New York, NY
Background/Purpose: Pulmonary involvement, including pulmonary vascular disease and interstitial lung disease can be an important cause of morbidity and even mortality in patients with primary…

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