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  • Abstract Number: 2726 • 2017 ACR/ARHP Annual Meeting

    Differentiating Features of Primary Angiitis of Central Nervous System and Reversible Cerebral Vasoconstriction Syndrome: Clinical and Radiological Evaluation

    Didem Saygin1, Russell Cerejo2, Priya Sundaram-Simonelli3, Gabor Toth4, Stephen Jones3, Leonard H. Calabrese5 and Rula A Hajj-Ali6, 1Internal Medicine, Department of Internal Medicine, Cleveland Clinic, Cleveland, Cleveland, OH, 2Cleveland Clinic, Cleveland, OH, 3Neuroradiology, Cleveland Clinic, Cleveland, OH, 4Cerebrovascular Center, Cleveland Clinic, Cleveland, OH, 5Rheumatic & Immunologic Disease and Infectious Disease, Cleveland Clinic Foundation, Cleveland, OH, 6Rheumatic and Immunologic Disease, Cleveland Clinic, Cleveland, OH

    Background/Purpose: Primary angiitis of central nervous system (PACNS) is an isolated vasculitis affecting small-sized cerebral blood vessels. One of the major mimickers of PACNS is…
  • Abstract Number: 2727 • 2017 ACR/ARHP Annual Meeting

    Assessment of Damage and Prognosis in Patients with Adult IgA Vasculitis: Retrospective Multicentered Cohort Study

    Fatma Alibaz-Oner1, Ahmet Omma2, Alper Sari3, Omer Karadag4, Dondu Uskudar Cansu5, Cemal Bes6, Fatih Yildiz7, Mustafa Ferhat Oksuz8, Sema Yilmaz9, Atalay Dogru10, Ayse Balkarli8, Sibel Bakirci11 and Haner Direskeneli12, 1Marmara University, School of Medicine, Rheumatology, Istanbul, Turkey, 2Department of Internal Medicine, Division of Rheumatology, Ankara Numune Training and Research Hospital, Ankara, Turkey, 3Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey, 4Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey, 5Rheumatology, Osmangazi University, School of Medicine, Eskısehir, Turkey, 6Rheumatology, Bakırköy Dr. Sadi Konuk Training and Research Hospital, İstanbul, Turkey, 7Rheumatology, Van EAH, Adana, Turkey, 8PsART study group, Ankara, Turkey, 9Department of Rheumatology, Selcuk University School of Medicine, Konya, Turkey, 10Rheumatology, Suleyman Demirel University, School of Medicine, Isparta, Turkey, 11Fellow in Rheumatology, Antalya, Turkey, 12Rheumatology, Marmara University, School of Medicine, Rheumatology, Istanbul, Turkey

    Background/Purpose: IgA Vasculitis is a leukocytoclastic vasculitis involving small vessels with depositions of immune complexes containing IgA. IgA Vasculitis is a predominantly pediatric vasculitis. There…
  • Abstract Number: 2728 • 2017 ACR/ARHP Annual Meeting

    Serum Levels of Interleukin-36 Receptor Antagonist in Behçet’s Patients

    Pelin Ünsal1, Pamir Cerci2, Şükrü Alper Açıkgöz2, Göksal Keskin2 and Ümit Ölmez2, 1Ankara University School of Medicine, Ankara, Turkey, 2Immunology and Allergy, Ankara University School of Medicine, Ankara, Turkey

    Background/Purpose: Behcet's disease (BD) is a systemic vasculitis disorder of unknown etiology with recurrent exacerbations and remissions. The etiopathogenesis of the disease is still unclear.…
  • Abstract Number: 2729 • 2017 ACR/ARHP Annual Meeting

    Surgical Therapies in the Treatment of Pulmonary Artery Involvement in Behcet’s Syndrome

    Hasan Tuzun1, Gul Guzelant2, Ozkan Demirhan3, Buge Oz4, Izzet Fresko2, Vedat Hamuryudan2, Hasan Yazici2 and Emire Seyahi2, 1Istanbul University, Cerrahpasa Medical Faculty, Department of Cardiovascular Surgery, Istanbul, Turkey, 2Istanbul University, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, 3Istanbul Bilim University, Medical Faculty, Department of Thoracic Surgery, Istanbul, Turkey, 4Istanbul University, Cerrahpasa Medical Faculty, Department of Pathology, Istanbul, Turkey

    Background/Purpose: The mainstay treatment of pulmonary artery involvement (PAI) in Behcet’s syndrome (BS) is immunosuppresion and corticosteroids (1). The role of surgical intervention in the…
  • Abstract Number: 2730 • 2017 ACR/ARHP Annual Meeting

    Clinical Features and Treatment of Central Nervous System Vasculitis Associated with Acute Posterior Multifocal Placoid Pigment Epitheliopathy

    Hiromichi Tamaki1 and Rula A Hajj-Ali2, 1Department of Rheumatic and Immunologic Diseases, Cleveland Clinic Foundation, Cleveland, OH, 2Rheumatic and Immunologic Disease, Cleveland Clinic, Cleveland, OH

    Background/Purpose: Acute posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) is a rare inflammatory eye disease and some appear to develop central nervous system vasculitis (CNSV). This…
  • Abstract Number: 2731 • 2017 ACR/ARHP Annual Meeting

    The Utility of Unbiased Metagenomic Next Generation Sequencing in the Management of Patients with CNS Vasculitis

    Hiromichi Tamaki1, Michael R Wilson2, Leonard H. Calabrese3, Joseph L. DeRisi4 and Rula A Hajj-Ali5, 1Department of Rheumatic and Immunologic Diseases, Cleveland Clinic Foundation, Cleveland, OH, 2Department of Neurology, UCSF, San Francisco, CA, 3Rheumatic & Immunologic Disease, Cleveland Clinic Foundation, Cleveland, OH, 4Biochemistry and Biophysics, UCSF, San Francisco, CA, 5Rheumatology, Cleveland Clinic Foundation, Cleveland, OH

    Background/Purpose: In the clinical approach to CNS vasculitis, exclusion of infection is of major concern as some microbes can cause vasculitis, and infections can complicate…
  • Abstract Number: 2732 • 2017 ACR/ARHP Annual Meeting

    Recommendations for the Management of Neuro-Behcet’s Disease By the Japanese National Research Committee for Behcet’s Disease

    Hirotoshi Kikuchi1, Tetsuji Sawada2, Masato Okada3, Mitsuhiro Takeno4, Masataka Kuwana4, Yoshiaki Ishigatsubo5, Izumi Kawachi6, Hideki Mochizuki7, Susumu Kusunoki8 and Shunsei Hirohata9, 1Department of Internal Medicine, Teikyo University School of Medicine, Tokyo, Japan, 2Rheumatology, Tokyo Medical University, Shinjuku Tokyo, Japan, 3Immuno-Rheumatology Center, St. Luke’s International Hospital, Tokyo, Japan, 4Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, 5Department of Internal Medicine and Clinical Immunology, Yokohama City University Graduate School of Medicine, Yokohama, Japan, 6Department of Neurology, Niigata University Medical and Dental Hospital, Niigata, Japan, 7Department of Neurology, Osaka University Graduate School of Medicine, Osaka, Japan, 8Department of Neurology, Kinki University School of Medicine, Osaka, Japan, 9Rheumatology and Infectious Diseases, Kitasato University School of Medicine, Kanagawa, Japan

    Background/Purpose: Central nervous system involvement is one of the most serious complications in Behçet’s disease (BD). This condition is referred to as neuro-Behçet’s disease (NB)…
  • Abstract Number: 2733 • 2017 ACR/ARHP Annual Meeting

    Clinical Characteristics and Treatment Outcomes of Patients with Behçet’s Disease and Vascular Involvement

    In Young Kim1, Yeong Hee Eun2, Ji young Chai3, Hyungjin Kim1, Jaejoon Lee1, Eun-Mi Koh1, Duk-Kyung Kim4 and Hoon-Suk Cha1, 1Department of Medicine,Division of Rheumatology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea, Republic of (South), 2Department of Medicine,Division of Rheumatology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, MN, Korea, Republic of (South), 3Department of Rheumatology, Bundang Jesaeng Hospital, Seongnam, Korea, Republic of (South), 4Department of Medicine,Division of Cardiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea, Republic of (South)

    Background/Purpose: The international Chapel Hill Consensus Conference (CHCC) categorized Behçet’s disease (BD) as variable vessel vasculitis, which was defined as vasculitis that can affect vessels…
  • Abstract Number: 2734 • 2017 ACR/ARHP Annual Meeting

    A Comparison of Current Practice to New Vasculitis Treatment Guidelines

    Ishrat Gill1, Norman Madsen2, Imran Hassan3 and Elaine Yacyshyn4, 1Medicine, University of Alberta, Sherwood Park, AB, Canada, 2Medicine, Rheumatology, University of Alberta, Edmonton, AB, Canada, 3EPICORE Centre, University of Alberta, Edmonton, AB, Canada, 4Medicine, University of Alberta, Edmonton, AB, Canada

    Background/Purpose: Appropriate treatment for Anti-Neutrophilic Cytoplasm Antibodies (ANCA) Associated Vasculitis (AAV) requires induction and maintenance of remission, while balancing side effects from treatment. The CanVasc…
  • Abstract Number: 2735 • 2017 ACR/ARHP Annual Meeting

    Is There an Association between Adult IgA Vasculitis and Cancer?

    Jaka Ostrovrsnik1, Ziga Rotar1, Rok Jese1, Matija Tomšič1,2 and Alojzija Hočevar1, 1Department of Rheumatology, University Medical Centre Ljubljana, Ljubljana, Slovenia, 2University of Ljubljana, Faculty of Medicine, Ljubljana, Slovenia

    Background/Purpose: An increased incidence rate of cancer has been reported in adult patients with IgA vasculitis (IgAV). These conclusions are mostly based on observations in…
  • Abstract Number: 2736 • 2017 ACR/ARHP Annual Meeting

    Long-Term Survival in Systemic Necrotizing Vasculitides

    Sabine Jardel1, Xavier Puéchal2, Alain Le Quellec3, Matthieu Groh2, Mohamed Hamidou4, Francois Maurier5, Olivier Aumaître6, Achille Aouba7, Thomas Quémeneur8, Jean-François Subra9, Vincent Cottin10, Pascal Godmer11, Patrice Cacoub12, Philippe Delaval13, Anne-Laure Fauchais14, Eric Hachulla15, Delphine Maucort-Boulch16, Loïc Guillevin2 and Jean-Christophe Lega17, 1Lyon 1 University, Lyon, France, 2National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, 3Montpellier, Montpellier, France, 4Internal Medicine Department, Internal Medicine Department, Nantes University Hospital, Nantes, France, 5Internal Medicine, Sainte-Blandine de Metz Hospital, Metz, France, 6CHU Pitié-Salpêtrière - Department of Internal Medicine 2. Referal center for SLE/APS, Paris, France, 7Department of Internal Medicine, Caen University Hospital, Caen, France, 8Service de néphrologie, médecine interne et vasculaire, Hôpital de Valenciennes, Valenciennes, France, 9Angers, Angers, France, 10Louis Pradel Hospital, Claude Bernard University Lyon 1, Lyon, France, 11Medecine Interne, CH Vannes, Vannes, France, 12Department of Internal Medicine and Clinical Immunology, Groupe Hospitalier Pitié-Salpêtrière, Paris, France, 13Rennes, Rennes, France, 14Internal Medicine, Internal Medicine, Limoges, France, 15CHU Lille, Département de Médecine Interne et Immunologie Clinique, F-59000 Lille, France, Lille, France, 16Biostatistics and bioinformatics, Centre Hospitalier Lyon Sud - Claude Bernard University Lyon 1, Lyon, France, 17Department of Internal and Vascular Medicine, Lyon Sud Hospital, Hospices Civils de Lyon, Lyon, France

    Background/Purpose: The aim of the study was to describe the evolution of mortality over recent decades in systemic necrotizing vasculitides, including polyarteritis nodosa (PAN), granulomatosis…
  • Abstract Number: 2737 • 2017 ACR/ARHP Annual Meeting

    Pulmonary Manifestations of Primary Systemic Vasculitides

    Jean-Paul Makhzoum1, Raashid Luqmani2, Richard A. Watts3, Anthea Craven4, Peter A. Merkel5 and Christian Pagnoux6, 1Rheumatology, Vasculitis Clinic, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, 2Botnar Research Centre, University of Oxford, Oxford, United Kingdom, 3Rheumatology Department, The Ipswich Hospital, Ipswich, Great Britain, 4Nuffield Department of Orthopaedics Rheumatology and Musculoskeletal Sciences, Botnar Research Centre, Oxford University Hospitals, Oxford, United Kingdom, 5Division of Rheumatology, University of Pennsylvania; Perelman School of Medicine, Philadelphia, PA, 6Rheumatology-Vasculitis clinic, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada

    Background/Purpose: Pulmonary involvement in systemic primary vasculitides is diverse and occurs with variable incidence depending on the type of vasculitis. This study aimed to describe…
  • Abstract Number: 2738 • 2017 ACR/ARHP Annual Meeting

    Infectious Complications in Systemic Necrotizing Vasculitides: Pooled Analysis of Five Prospective, Randomized, Controlled Trials

    Lafarge Antoine1, Christian Pagnoux2, Xavier Puéchal3, Maxime Samson4, Mohamed Hamidou5, Alexandre Karras6, Thomas Quémeneur7, Matthieu Groh8, Luc Mouthon9, Loïc Guillevin for the French Vasculitis Study Group3 and Benjamin Terrier10, 1Medecine Interne, Hôpital Cochin, Paris, France, 2Mount Sinai Hospital, Toronto, ON, Canada, 3Service de Médecine Interne, Centre de Référence Maladies Auto-Immunes et Auto-Inflammatoires Systémiques Rares, Hôpital Cochin, Paris, France, 4Department of Internal Medicine and Clinical Immunology, Hôpital François Mitterrand, CHU de Dijon, Dijon, France, 5Medecine Interne, CHU Hôtel Dieu, Nantes, France, 6nephrology, HEGP, Paris, France, 7Service de néphrologie, médecine interne et vasculaire, Hôpital de Valenciennes, Valenciennes, France, 8National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, 9Université Paris Descartes Sorbonne Paris, Paris, France, 10Internal Medicine, Cochin University Hospital, Paris, France

    Background/Purpose: Prognosis of patients with systemic necrotizing vasculitides has been markedly improved during the last 2 decades. However, infectious complications remain a major cause of…
  • Abstract Number: 2739 • 2017 ACR/ARHP Annual Meeting

    Onco-Hematological Malignancies in Systemic Necrotizing Vasculitides: Pooled Analysis of Five Prospective, Randomized, Controlled Trials

    Lafarge Antoine1, Christian Pagnoux2, Xavier Puéchal3, Maxime Samson4, Mohamed Hamidou5, Alexandre Karras6, Thomas Quémeneur7, Matthieu Groh8, Luc Mouthon9, Loïc Guillevin for the French Vasculitis Study Group3 and Benjamin Terrier10, 1Medecine Interne, Hôpital Cochin, Paris, France, 2Mount Sinai Hospital, Toronto, ON, Canada, 3Service de Médecine Interne, Centre de Référence Maladies Auto-Immunes et Auto-Inflammatoires Systémiques Rares, Hôpital Cochin, Paris, France, 4Department of Internal Medicine and Clinical Immunology, Hôpital François Mitterrand, CHU de Dijon, Dijon, France, 5Medecine Interne, CHU Hôtel Dieu, Nantes, France, 6nephrology, HEGP, Paris, France, 7Service de néphrologie, médecine interne et vasculaire, Hôpital de Valenciennes, Valenciennes, France, 8National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, 9Université Paris Descartes Sorbonne Paris, Paris, France, 10Internal Medicine, Cochin University Hospital, Paris, France

    Background/Purpose: The use of long-term immunosuppressive agents in patients with systemic necrotizing vasculitides has dramatically improved the overall prognosis, but expose patients to potential severe…
  • Abstract Number: 2740 • 2017 ACR/ARHP Annual Meeting

    Cyclophosphamide Treatment Modulates Circulating Cell Populations in Patients with Vasculitis and Autoimmune Systemic Diseases

    Martina Skácelová1, Gabriela Gabčová2, Pavel Horak3, Zuzana Mikulková2, František Mrázek4, Eva Kriegová5 and Andrea Smržová6, 1III. Department of Internal Medicine, Faculty of Medicine and Dentistry, Palacký University of Olomouc, Olomouc, Czech Republic, 2Department of Immunology, Faculty of Medicine and Dentistry, Palacky University Olomouc, Olomouc, Czech Republic, 3III. Department of internal medicine, III. Department of Internal Medicine, Faculty of Medicine and Dentistry, Palacký University of Olomouc, Olomouc, Czech Republic, 4Department of Immunology, Faculty of Medicine and Dentistry, Palacky University of Olomouc, Olomouc, Czech Republic, 5Department of Immunology, Department of Immunology, Faculty of Medicine and Dentistry, Palacky University of Olomouc, Olomouc, Czech Republic, 6III. Department of Internal Medicine, III. Department of Internal Medicine, Faculty of Medicine and Dentistry, Palacký University of Olomouc, Olomouc, Czech Republic

    Background/Purpose: Although cyclophosphamide (CFA) remain the cornerstone for treatment of patients with severe manifestations of systemic autoimmune diseases, the knowledge about the effect of CFA…
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