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  • Abstract Number: 1930 • 2016 ACR/ARHP Annual Meeting

    Factors Associated with Glucocorticoid Exposure in ANCA-Associated Vasculitis

    Matthew D. Cascino1, Ulrich Specks2, Peter A. Merkel3, Philip Seo4, Robert F. Spiera5, Carol A. Langford6, Gary S. Hoffman6, Cees G.M. Kallenberg7, E. William St Clair8, Paul A. Monach9, John H. Stone10 and Paul Brunetta11, 1Division of Rheumatology, University of California, San Francisco, San Francisco, CA, 2Mayo Clinic, Rochester, MN, 3Penn Vasculitis Center, Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, 4Rheumatology Division, Johns Hopkins Vasculitis Center, Johns Hopkins University, Baltimore, MD, 5Rheumatology, Hospital for Special Surgery, New York, NY, 6Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, 7Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, Netherlands, 8Rheumatology, Duke University Medical Center, Durham, NC, 9Rheumatology, Boston University School of Medicine, Boston, MA, 10Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA, 11Genentech, Inc., South San Francisco, CA

    Background/Purpose: Factors associated with cumulative glucocorticoid exposure in granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) have not previously been described. We examined the association…
  • Abstract Number: 1931 • 2016 ACR/ARHP Annual Meeting

    MRI Findings in Granulomatosis with Polyangiitis: Pachymeningitis and Implications in Quality of Life

    Violeta Higuera-Ortiz1, Natllely Ruiz1, Daniel Carrillo2, Abraham Reynoso2, Rosa Delia Delgado-Hernández2 and Luis F. Flores-Suarez1, 1Primary Systemic Vasculitides Clinic, Instituto Nacional de Enfermedades Respiratorias, Mexico City, Mexico, 2Radiology and Molecular Imaging, Instituto Nacional de Ciencias Médicas y Nutrición, Mexico City, Mexico

    Background/Purpose: The frequency of CNS involvement in granulomatosis with polyangiitis (GPA) is uncommon (4-18%). Among them, pachymeningitis (PM) has been reported in 0.6-8%. Improvement of…
  • Abstract Number: 1932 • 2016 ACR/ARHP Annual Meeting

    Precapillary Pulmonary Hypertension in Granulomatosis with Polyangiitis: A Case Series with Long-Term Follow-up

    Xavier Puéchal1, Xavier Jaïs2, Claire Le Hello3, Anne Grasland4, Assia Eslami1, Jesús Rolando de la Jara Cordero1, Benjamin Terrier1,5, David Launay6, Loïc Guillevin1, Marc Humbert2 and Luc Mouthon for the French Vasculitis Study Group1, 1National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, 2Le Kremlin–Bicêtre University Hospital, Bicêtre, France, 3Caen University Hospital, caen, France, 4Béclère University Hospital, Clamart, France, 5Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, 6Lille University Hospital, Lille, France

    Background/Purpose:  A few isolated case reports of precapillary pulmonary hypertension (PH) in ANCA-associated vasculitides, mostly granulomatosis with polyangiitis (GPA), have been published. The characteristics and…
  • Abstract Number: 1933 • 2016 ACR/ARHP Annual Meeting

    M2 Macrophage Is the Predominant Phenotype in Airways Inflammatory Lesions in Patients with Granulomatosis with Polyangiitis

    Alexandre W.S. Souza1, Mirjan van Timmeren2, Jan-Stephan Sanders3, Coen A. Stegeman4, Peter Heeringa5, Cees G.M. Kallenberg6 and Johanna Westra6, 1Universidade Federal de São Paulo, São Paulo, Brazil, 2University of Groningen, Groningen, Netherlands, 3University of Groningen, University Medical Center Groningen, Groningen, Netherlands, 4Nephrology, University of Groningen, University Medical Center Groningen, Groningen, Netherlands, 5Pathology and Medical Biology, University of Groningen, University Medical Center Groningen, Groningen, Netherlands, 6Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, Netherlands

    Background/Purpose:  Macrophages may present two main phenotypes indicated as M1 and M2 under different stimuli. M1- and M2-macrophages have divergent functions that range from enhancement…
  • Abstract Number: 1934 • 2016 ACR/ARHP Annual Meeting

    Cutaneous Manifestations of ANCA-Associated Vasculitis

    Zelma ChiesaFuxench1, Robert Micheletti2, Raashid Luqmani3, Richard Watts4, Anthea Craven5 and Peter A. Merkel6, 1University of Pennsylvania, Philadelphia, PA, 2Department of Dermatology, Hospital of the University of Pennsylvania, Philadelphia, PA, 3Oxford, Oxford, United Kingdom, 4Norwich Medical School, University of East Anglia, Norwich, United Kingdom, 5Nuffield Department of Orthopaedics Rheumatology and Musculoskeletal Sciences, Botnar Research Centre, University of Oxford, Oxford, United Kingdom, 6Division of Rheumatology, University of Pennsylvania, Philadelphia, PA

    The cutaneous manifestations of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), are varied…
  • Abstract Number: 1935 • 2016 ACR/ARHP Annual Meeting

    Hypertrophic Pachymeningitis in a Population with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Retrospective Study in a Single Japanese Institution

    Yasuhiro Shimojima, Dai Kishida, Akiyo Hineno, Masahide Yazaki, Yoshiki Sekijima and Shu-ichi Ikeda, Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan

    Background/Purpose: Hypertrophic pachymeningitis (HP) is an inflammatory disorder causing focal and diffuse thickening of the dura mater. It is realized that anti-neutrophil cytoplasmic antibody (ANCA)-associated…
  • Abstract Number: 1936 • 2016 ACR/ARHP Annual Meeting

    Clinical Characteristics of Inflammatory Eye Disease in Patients with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Retrospective Cohort Study

    Patompong Ungprasert1, Cynthia S. Crowson2, Rodrigo Cartin-Ceba3, James A. Garrity4, Wendy M. Smith5, Ulrich Specks6, Eric L. Matteson1 and Ashima Makol7, 1Rheumatology, Mayo Clinic, Rochester, MN, 2Health Sciences Research, Mayo Clinic, Rochester, MN, 3Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, 4Ophthalmology, Mayo Clinic, Rochester, MN, 5Department of Ophthalmology, Mayo Clinic, Rochester, MN, 6Mayo Clinic, Rochester, MN, 7Division of Rheumatology, Department of Internal Medicine, Mayo Clinic, Rochester, MN

    Clinical Characteristics of Inflammatory Eye Disease in Patients with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Retrospective Cohort Study Background/Purpose: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is…
  • Abstract Number: 1937 • 2016 ACR/ARHP Annual Meeting

    Analysis of Innate and Adaptive Immune Responses in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)

    Jeremie Dion1,2, Jonathan London2,3, Benjamin Chaigne2,4, Nicolas Dumoitier2, Bertrand Dunogué5, Pascal Cohen3, Matthieu Groh4, Claire Le Jeunne4, Luc Mouthon2,5 and Benjamin Terrier2,5, 1Internal medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, 2INSERM U1016, Institut Cochin, Equipe Neutrophiles et Vascularites, Paris, France, 3Internal Medecine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, 4National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, 5Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France

    Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA, formerly called Churg-Strauss syndrome) belongs to ANCA-associated vasculitis and is characterized by late onset asthma, blood and tissue eosinophilia…
  • Abstract Number: 1938 • 2016 ACR/ARHP Annual Meeting

    Bronchiectasis Are Highly Prevalent in Anti-MPO ANCA-Associated Vasculitis and Associate with a Distinct Disease Phenotype

    Antoine Néel1, Alexandra Espitia1, Pierre Paul Arrigoni2, Christelle Volteau3, Agathe Masseau1, Marie Rimbert4, Christian Agard1, Fadi Fakhouri5, Renan Liberge2 and Mohamed Hamidou6, 1Internal Medicine Department, Nantes University Hospital, Nantes, France, 2Radiology Department, Nantes University Hospital, Nantes, France, 3Clinical Research Department, Nantes University Hospital, Nantes, France, 4Immunology Laboratory, Nantes University Hospital, Nantes, France, 5Nephrology Department, Nantes University Hospital, Nantes, France, 6Internal Medicine Department, Internal Medicine Department, Nantes University Hospital, Nantes, France

    Background/Purpose: To assess the prevalence of bronchiectasis in a western cohort of ANCA positive GPA or MPA and its correlations with disease phenotype and outcome.…
  • Abstract Number: 1939 • 2016 ACR/ARHP Annual Meeting

    Immunologic, Clinical and Demographic Correlates in 51 Cocaine Users with Serum Anti-Neutrophil Cytoplasmic Antibodies

    Grant Hughes1 and Meredith Barnes2, 1University of Washington, Seattle, WA, 2Medicine, University of Washington, Seattle, WA

    Background/Purpose: Exposure to illicit cocaine and its frequent adulterant, levamisole, is associated with the development of serum anti-neutrophil cytoplasmic autoantibodies (ANCA) and a variety of…
  • Abstract Number: 1940 • 2016 ACR/ARHP Annual Meeting

    High Prevalence of Inflammatory Heart Disease in Eosinophillic Granulomatosis with Polyangiitis (Churg Strauss) Patients

    Eloi Garcia Vives1, Len Harty2 and David Jayne3, 1Vall d'Hebrón Hospital, Barcelona, Spain, 2Vasculitis & Lupus, Addenbrookes Hospital University of Cambridge, Cambridge, United Kingdom, 3Vasculitis and Lupus Clinic, Addenbrookes Hospital University of Cambridge, Cambridge, United Kingdom

    Background/Purpose: To establish EGPA/Churg Strauss inflammatory heart disease prevalence and develop an algorithm for heart disease screening in EGPA patients. Methods: An audit of all…
  • Abstract Number: 1941 • 2016 ACR/ARHP Annual Meeting

    Retrospective Survey of Concomitant Autoimmune Diseases and Autoantibodies in a Cohort of Patients with ANCA-Associated Vasculitis (AAV)

    Marta Casal Moura1,2, Sergio Prieto-González2, Georgina Espígol-Frigolé2, Giuseppe Murgia2,3, Marco Alba2, Jose Hernández-Rodríguez2 and Maria C. Cid2, 1Department of Internal Medicine, São João Hospital Center, Porto, Portugal, 2Hospital Clínic. University of Barcelona. IDIBAPS, Vasculitis Research Unit. Department of Autoimmune Diseases, Spain, Barcelona, Spain, 3University Clinic for Visceral Surgery and Medicine Bauchzentrum Bern, Bern, Switzerland

    Background/Purpose: Anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) - granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA) have heterogenous clinic…
  • Abstract Number: 1942 • 2016 ACR/ARHP Annual Meeting

    Presentation and Clinical Features of ANCA-Associated Vasculitis in US African Americans: Experience from a Single Center  

    Sebastian Sattui1, Andrew Westfall2 and Angelo L. Gaffo3, 1Tinsley Harrison Internal Medicine Residency Program, Department of Medicine, University of Alabama at Birmingham, Birmingham, AL, 2Department of Biostatistics, School of Public Health, University of Alabama at Birmingham, Birmingham, AL, 3Medicine, Birmingham VA Medical Center, Birmingham, AL

    Title: Presentation and Clinical Features of ANCA-associated Vasculitis in US African Americans: Experience From a Single Center   Background/Purpose: Little is known about the presentation,…
  • Abstract Number: 1943 • 2016 ACR/ARHP Annual Meeting

    Factors Predictive of ANCA-Associated Vasculitis Relapse

    Maxime Samson1, Hervé Devilliers2, Xavier Puéchal3, Christian Pagnoux4, Pascal Cohen3, Luc Mouthon3, Benjamin Terrier5 and Loïc Guillevin3, 1Department of Internal Medicine and Clinical Immunology, Hôpital François Mitterrand, CHU de Dijon, Dijon, France, 2Department of Internal Medicine and Systemic Diseases, Hôpital François Mitterrand, CHU de Dijon, Dijon, France, 3Department of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, Paris, France, 4Division of Rheumatology, Mount Sinai Hospital, University Health Network, University of Toronto, Toronto, Canada, Toronto, ON, Canada, 5National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France

    Background/Purpose:  To identify associations between patients’ clinical and biological characteristics at diagnosis of antineutrophil cytoplasmic antibody-associated vasculitides (AAVs), and their relapse during follow-up. Methods:  Long-term…
  • Abstract Number: 1944 • 2016 ACR/ARHP Annual Meeting

    Disease Activity, Glucocorticoid Exposure, and Rituximab Determine Body Composition Changes during Induction Treatment of ANCA-Associated Vasculitis

    Zachary Wallace1, Eli Miloslavsky2, Sebastian H. Unizony3, Na Lu4, Gary S. Hoffman5, Cees G.M. Kallenberg6, Carol A. Langford7, Peter A. Merkel8, Paul A. Monach9, Philip Seo10, Robert F. Spiera11, Eugene William St.Clair12, Paul Bruntetta13, Matthew Cascino14, Hyon K. Choi15 and John H. Stone3, 1Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Boston, MA, 2Division of Rheumatology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, 3Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA, 4Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, 5Rheumatology, Cleveland Clinic, Cleveland, OH, 6Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, Netherlands, 7Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, 8Division of Rheumatology, Univ of Pennsylvania; Perelman School of Med, Philadelphia, PA, 9Rheumatology, Boston University School of Medicine, Boston, MA, 10Medicine, Johns Hopkins University, Baltimore, MD, 11Hospital for Special Surgery, Cornell, New York, NY, 12Rheumatology and Immunology, Duke University, Durham, NC, 13Genentech, Inc., South San Francisco, CA, 14University of California-San Francisco, San Francisco, CA, 15Rheumatology, Allergy and Immunology, Massachusetts General Hospital and Harvard Medical School, Boston, MA

    Background/Purpose: ANCA-associated vasculitis (AAV) treatment includes high dose glucocorticoids (GCs), which are associated with increased body-mass index (BMI), a complication abhorred by patients and associated…
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