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  • Abstract Number: 1659 • 2019 ACR/ARP Annual Meeting

    Linaclotide for the Treatment of Gastrointestinal Symptoms in Systemic Sclerosis

    Eric Dein1, John Clarke 2, Frederick Wigley 3 and Zsuzsanna McMahan* 3, 1Johns Hopkins, Baltimore, MD, 2Stanford, Pao Alto, 3Johns Hopkins University, Division of Rheumatology, Baltimore

    Background/Purpose: Gastrointestinal (GI) involvement is the most common internal organ affected in systemic sclerosis (SSc). Constipation is a common GI complication in SSc that affects…
  • Abstract Number: 1660 • 2019 ACR/ARP Annual Meeting

    Serial Sublingual Videomicroscopy in Systemic Sclerosis Clinic: Are the Microcirculation Measurements Correlated with Gastrointestinal Symptoms?

    Tracy Frech1, Guo Wei 2 and Maureen Murtaugh 3, 1Division of Rheumatology, University of Utah and Salt Lake VAMC, Salt Lake City, UT, 2University of Utah, Salt Lake City, UT, 3University of Utah and Salt Lake VAMC, Salt Lake City

    Background/Purpose: Intravital microscopy of the sublingual microcirculation provides estimates of perfused barrier region (PBR) and red blood cell fraction (RBCfract), which quantifies vasculopathy and, similar…
  • Abstract Number: 1661 • 2019 ACR/ARP Annual Meeting

    Circulating Cell Free DNA Released from Eosinophils Is a Practical Biomarker in Patients with Eosinophilic Granulomatosis with Polyangiitis

    Teppei Hashimoto1, Yuichi Yokoyama 2, Tetsuya Furukawa 3, Takahiro Yoshikawa 3, Naoto Azuma 2, Kohsuke Yoshida 4, Akira Hashiramoto 4 and Kiyoshi Matsui 3, 1Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine, Kobe, Hyogo, Japan, 2Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine, Nishinomiya, Japan, 3Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan, 4Kobe University Graduate School of Health Sciences, Kobe, Hyogo, Japan

    Background/Purpose: Fragmented cell-free DNA (cfDNA) is released into blood circulation as results of damage or death of peripheral blood cells as well as organ tissues,…
  • Abstract Number: 1662 • 2019 ACR/ARP Annual Meeting

    Does PR3-ANCA+ Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss)Really Exist?

    Matthias Papo1, Renato A. Sinico 2, Vítor Teixeira 3, Maria-Letizia Urban 4, Juliane Mahrhold 5, Francesco Locatelli 6, Giulia Cassone 7, Franco Schiavon 8, Benjamin Seeliger 9, Thomas Neumann 10, Claus Kroegel 11, Matthieu Groh 12, Chiara Marvisi 13, Maxime Samson 14, Thomas Barba 15, David Jayne 16, Bernhard Hellmich 5, Sara Monti 17, Carlomaurizio Montecucco 6, Carlo Salvarani 18, Jean-Emmanuel Kahn 12, Cécile-Audrey Durel 15, Loic Guillevin 19, Giacomo Emmi 4, Augusto Vaglio 20 and Benjamin Terrier 19, 1Department of Internal Medicine, Hôpital Cochin, Université Paris Descartes, Sorbonne Paris Cité, INSERM Unité 1016, Centre de Référence pour les Maladies Auto-immunes Rares, Paris, France, Paris, France, 2Department of Medicine and Surgery, Università degli Studi di Milano - Bicocca , Italy, Milan, Italy, 3Rheumatology and Bone Diseases Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte; Lisbon, Portugal., Lisbon, Portugal, 4Department of Experimental and Clinical Medicine, University of Firenze, Florence, Italy, Florence, Italy, 5Department of Internal Medicine, Rheumatology and Immunology, Vasculitis-Center Tübingen-Kirchheim, Medius Klinik Kirchheim, University of Tübingen, Kirchheim-Teck, Germany, Kirchheim, Germany, 6Department of Rheumatology, IRCCS Policlinico S. Matteo Foundation, University of Pavia, Pavia, Italy, Pavia, Italy, 7Clinical and Experimental Medicine PhD Program, University of Modena and Reggio Emilia, Modena, Italy. Rheumatology Unit, IRCCS Arcispedale Santa Maria Nuova, Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, Reggio Emilia, Italy, Modena and Reggio Emilia, Italy, 8Operative Unit of Rheumatology, Department of Medicine DIMED, University of Padova, Padova, Italy, Padova, Italy, 9Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany, Hannover, Germany, 10Division of Rheumatology and Immunolog, Kantonsspital St. Gallen, St. Gallen, Sankt Gallen, Switzerland, 11Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital, Jena, Germany, Jena, Germany, 12Service de Médecine Interne, Centre de Référence des Syndromes Hyperéosinophiliques-CEREO, Hôpital Foch, Université Versailles–Saint-Quentin-en-Yvelines, Suresnes, France, Suresnes, France, 13Nephrology Unit, Parma University Hospital, Parma, Italy, Parma, Italy, 14CHU Dijon, Dijon, France, 15Department of Internal Medicine, Hôpital Edouard Herriot, Lyon, France, Lyon, France, 16Vasculitis and Lupus Clinic, Addenbrooke’s Hospital, University of Cambridge, UK, Cambridge, United Kingdom, 17University of Pavia, Pavia, Italy, 18Division of Rheumatology, Arcispedale Santa Maria Nuova-IRCCS, Reggio Emilia, Italy, 19National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France, 20Nephrology Unit, Parma University Hospital, Parma, Italy, Nephrology Unit, Parma University Hospital, Parma, Italy, Italy

    Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg–Strauss) is a small-vessel necrotizing vasculitis characterized by blood and tissue eosinophilia and asthma. Only a third of EGPA…
  • Abstract Number: 1663 • 2019 ACR/ARP Annual Meeting

    Assessments of Quality of Life in Patients with Eosinophilic Granulomatosis with Polyangiitis

    Gunnar Tomasson1, Christian Pagnoux 2, David Cuthbertson 3, Simon Carette 2, Nader A. Khalidi 4, Curry L. Koening 5, Carol Langford 6, Carol A. McAlear 7, Larry Moreland 8, Paul Monach 9, Philip Seo 10, Antoine Sreih 11, Steven Ytterberg 12 and Peter Merkel 11, 1Faculty of Medicine, University of Iceland and Landpitali University Hospital, Reykjavik, Iceland, 2Mount Sinai Hospital and University Health Network, Toronto, ON, Canada, 3University of South Florida, Tampa, FL, 4McMaster University, Hamilton, ON, Canada, 5University of Utah Hospital, Salt Lake City, UT, 6Cleveland Clinic, Cleveland, OH, 7University of Pennsylvania - VCRC Project Manager, Philadelphia, PA, 8University of Pittsburgh, PITTSBURGH, PA, 9Brigham and Women's Hospital, Boston, MA, 10Johns Hopkins Medicine, Baltimore, MD, 11University of Pennsylvania, Philadelphia, PA, 12Mayo Clinic College of Medicine, Rochester, MN

    Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by asthma and other manifestations of vasculitis, some of which can be life-threatening, cause major organ damage,…
  • Abstract Number: 1664 • 2019 ACR/ARP Annual Meeting

    Reducing the Number of Rituximab Infusions at Onset of Maintenance Therapy for ANCA-associated Vasculitides: Results of a Post Hoc Analysis from a Randomized–controlled Trial

    Pierre Charles1, Agnes Dechartres 2, Benjamin Terrier 3, Pascal Cohen 3, Stanislas Faguer 4, Antoine Huart 4, Mohamed Hamidou 5, Christian Agard 5, Bernard Bonnotte 6, Maxime Samson 7, Alexandre Karras 8, Noémie Jourde-Chiche 9, François Lifermann 10, Pierre Gobert 11, Catherine Hanrotel-Saliou 12, Pascal Godmer 13, Nicolas Martin-Silva 14, Grégory Pugnet 15, Marie Matignon 16, Olivier Aumaitre 17, Jean-François Viallard 18, François Maurier 19, Nadine Meaux Ruault 20, Sophie Rivière 21, Jean Sibilia 22, Xavier Puéchal for the French Vasculitis Study Group 3, Luc Mouthon 3 and Loic Guillevin 3, 1Institut Mutualiste Montsouris, Paris, France, 2APHP, Paris, France, 3National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France, 4CHU Toulouse, Toulouse, France, 5CHU Nantes, Nantes, France, 6Service de Médecine Interne et Immunologie Clinique, CHU Dijon Bourgogne, Hôpital François Mitterrand, Dijon ; Université Bourgogne-Franche Comté, INSERM, EFS BFC, UMR1098, F-21000 Dijon, Dijon, France, 7CHU Dijon, Dijon, France, 8Paris HEGP, Paris, France, 9APHM, Marseille, Marseille, France, 10CH Dax, Dax, France, 11CH Avignon, Avignon, France, 12CHU Brest, Brest, France, 13CH Bretagne-Atlantique, Vannes, France, 14CHU Caen, Caen, France, 15CHU de Toulouse, Hôpital Purpan, Service de Médecine Interne, Toulouse, France, 16APHP, Créteil, France, 17CHU Clermont Ferrand, Clermont Ferrand, France, 18CHU Bordeaux, Bordeaux, France, 19Service de Médecine Interne, Hôpital Belle Isle, Metz, Metz, France, 20CHU Besançon, Besançon, France, 21CHU Montpellier, Montpellier, France, 22CHU Strasbourg, Strasbourg, France

    Background/Purpose: Rituximab (RTX) superiority over azathioprine to maintain ANCA-associated vasculitis (AAV) remission was demonstrated.The MAINRITSAN2 trial was designed to compare an individually tailored RTX-infusion schedule…
  • Abstract Number: 1665 • 2019 ACR/ARP Annual Meeting

    Glucocorticoids in Incident ANCA-Associated Vasculitis (AAV) Patients – A Study of Routine Clinical Practice in the EU Demonstrates Prolonged Use and Temporal Relationship to Adverse Events and Infections

    Dieter Goette1 and Peter Rutherford 1, 1Vifor Pharma, Zurich, Zurich, Switzerland

    Background/Purpose: AAV is a severe systemic vasculitis and rapid induction of remission is essential and  high dose glucocorticoids (GC) are part of standard of care.…
  • Abstract Number: 1666 • 2019 ACR/ARP Annual Meeting

    Maintenance Treatment in ANCA Associated Vasculitis in Real World Clinical Practice – Burden of Disease, Use of Glucocorticoids and Impact on Patient Functional Status Remain Major Problems

    Dieter Goette1 and Peter Rutherford 1, 1Vifor Pharma, Zurich, Zurich, Switzerland

    Background/Purpose: After successful remission induction AAV is a relapsing remitting long term condition and patients are at risk of organ damage from both active AAV…
  • Abstract Number: 1667 • 2019 ACR/ARP Annual Meeting

    Long Term Outcome of Hydralazine-Associated Vasculitis

    Saja Almaaitah1, Kinanah Yaseen 2, Yuxuan Jin 3 and Rula Hajj-ali 4, 1Internal Medicine Residency/Cleveland Clinic, University Heights, OH, 2Cleveland Clinic, Cleveland, OH, 3Quantitative Health Science, Cleveland Clinic, Cleveland, OH, 4Rheumatologic and Immunologic Disease/Cleveland Clinic, Cleveland, OH

    Background/Purpose: Hydralazine associated vasculitis (HAV) is rare but with potentially detrimental complications, affecting different organ systems. Most case series described short term disease course. Hence,…
  • Abstract Number: 1668 • 2019 ACR/ARP Annual Meeting

    Granulomatosis with Polyangiitis: Data from the French Vasculitis Study Group Registry

    Michele Iudici1, Christian Pagnoux 2, Delphine Courvoisier 3, Pascal Cohen 4, Mohamed Hamidou 5, Achille Aouba 6, François Lifermann 7, Marc Ruivard 8, Olivier Aumaitre 9, Bernard Bonnotte 10, François Maurier 11, Olivier Decaux 12, Eric Hachulla 13, Alexandre Karras 14, Chahéra Khouatra 15, Noémie Jourde-Chiche 16, Jean-François Viallard 17, Claire Blanchard-Delaunay 18, Pascal Godmer 19, Alain Le Quellec 20, Thomas Quémeneur 21, Claire de Moreuil 22, Alexis Régent 4, Benjamin Terrier 4, Luc Mouthon 4, Loic Guillevin 4 and Xavier Puéchal for the French Vasculitis Study Group 4, 1National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, France and Division of Rheumatology, Department of Internal Medicine Specialties, Geneva University Hospitals, Switzerland, Geneva, Geneve, Switzerland, 2National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin and Toronto Canada, Paris, France, 3Division of Rheumatology, Geneva University Hospital, Switzerland, Geneva, Switzerland, 4National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France, 5CHU Nantes, Nantes, France, 6CHU Caen, Caen, France, 7CH Dax, Dax, France, 8CHU Clermont-Ferrand, Clermont-Ferrand, France, 9CHU Clermont Ferrand, Clermont Ferrand, France, 10Service de Médecine Interne et Immunologie Clinique, CHU Dijon Bourgogne, Hôpital François Mitterrand, Dijon ; Université Bourgogne-Franche Comté, INSERM, EFS BFC, UMR1098, F-21000 Dijon, Dijon, France, 11Service de Médecine Interne, Hôpital Belle Isle, Metz, Metz, France, 12CHU Rennes, Rennes, France, 13CHU Lille, Lille, France, 14Paris HEGP, Paris, France, 15CHU Lyon, Lyon, France, 16APHM, Marseille, Marseille, France, 17CHU Bordeaux, Bordeaux, France, 18CH Niort, Niort, France, 19CH Bretagne-Atlantique, Vannes, France, 20CHU Montpellier, Montpellier, France, 21CH Valenciennes, Valenciennes, France, 22CHU Brest, Brest, France

    Background/Purpose: The long-term features of granulomatosis with polyangiitis (GPA), a systemic small-vessel ANCA-associated necrotizing vasculitis, have been mainly reported in small patient series. The aim…
  • Abstract Number: 1669 • 2019 ACR/ARP Annual Meeting

    Comparison of Mizoribine with Azathioprine in Efficacy and Safety for ANCA-Associated Vasculitis

    Sho Fukui 1, Satoshi Kawaai1, Genki Kidoguchi 1, Takehiro Nakai 2, Hiroki Ozawa 1, Ayako Koido 1, Yukihiko Ikeda 1, Masei Suda 1, Haruyuki Yanaoka 1, Hisanori Shimizu 1, Hiromichi Tamaki 1, Tokutaro Tsuda 1, Mitsumasa Kishimoto 3, Kenichi Yamaguchi 1 and Masato Okada 1, 1St.Luke International Hospital Immuno-Rheumatology Center, Tokyo, Japan, 2St.Luke International Hospital Immuno-Rheumatology Center, Tokyo, Tokyo, Japan, 3Immuno-Rheumatology Center, St. Luke’s International Hospital, Tokyo, Japan

    Background/Purpose: Mizoribine (MZR) is an immunosuppressant working as an inhibitor of purine synthesis, which mechanism of action is similar to mycophenolate mofetil. MZR is approved…
  • Abstract Number: 1670 • 2019 ACR/ARP Annual Meeting

    Evaluation of Subclinical Coronary Atherosclerosis in ANCA-associated Small Vessel Vasculitides (AAVs) Compared to Matched Controls Through Visual Assessment of Coronary Arterial Calcium (CAC) Score Using Non-Gated Chest Computed Tomography (CT)

    Sohail Farshad1, Alexandra Halalau 2, Sayf Al-Katib 3, Julie George 4 and Elena Schiopu 5, 1Department of Internal Medicine, Beaumont Health System, Royal Oak, MI, 2Department of Internal Medicine, Beaumont Health System, Royal Oak, 3Department of Radiology, Beaumont Health System, Royal Oak, MI, 4Beaumont Health System, Royal Oak, MI, 5Department of Rheumatology, University of Michigan, Ann Arbor

    Background/Purpose: Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is the most prevalent adult vasculitis and is associated with significant inflammatory burden; chest computed tomography (CT)…
  • Abstract Number: 1671 • 2019 ACR/ARP Annual Meeting

    Hospital Admissions and Mortality in Patients with ANCA-associated Vasculitis

    Jon Golenbiewski1, Amanda Eudy 2, Megan Clowse 2 and Nancy Allen 2, 1Duke University, Durham, NC, 2Duke University, Durham

    Background/Purpose: ANCA-associated vasculitis (AAV) has a high rate of complications, both from disease itself and treatments. Hospital mortality rates for AAV range between 10-20%. There…
  • Abstract Number: 1672 • 2019 ACR/ARP Annual Meeting

    Use of Rituximab for the Treatment of ANCA-Associated Vasculitis in Canada, 2010-2018

    Arielle Mendel1, Simon Carette 2 and Christian Pagnoux 2, 1Mount Sinai Hospital, Toronto, ON, Canada, 2Mount Sinai Hospital and University Health Network, Toronto, ON, Canada

    Background/Purpose: Rituximab (RTX) is an effective therapy for ANCA-Associated Vasculitis (AAV).  The Canadian Vasculitis Research Network (CanVasc) recommends RTX for induction among patients with unacceptable risk…
  • Abstract Number: 1673 • 2019 ACR/ARP Annual Meeting

    Factors Associated with Overall and First-Year Mortality in Turkish Patients with ANCA-Associated Vasculitides: Retrospective, Multicentre Trial

    Onay Gercik1, Emre Bilgin 2, Dilek Solmaz 1, Irfan Ocal 3, Arzu Saglam 4, Riza Kardas 5, Ozge Aybi 5, Gokhan Kabadayi 6, Zeki Soypacaci 7, İdil Kurut Aysin 8, Tolga Yildirim 9, Omer Karadag 10 and Servet Akar 11, 1Izmir Katip Celebi University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, İzmir, Turkey, 2Hacettepe University Vasculitis Centre, Ankara, Turkey, Ankara, Turkey, 3Izmir Katip Celebi University, Faculty of Medicine, Department of Pathology, Izmir, Turkey, 4Hacettepe University, Faculty of Medicine, Department of Pathology, Ankara, Turkey, 5Hacettepe University, Faculty of Medicine, Department of Internal Medicine, Ankara, Turkey, 6Izmir Katip Celebi University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, İzmir, Turkey, 7Izmir Katip Celebi University Faculty of Medicine, Department of Internal Medicine, Division of Nephrology, Izmir, Turkey, 8Izmir Katip Celebi University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, İzmir, Turkey, 9Hacettepe University, Faculty of Medicine, Department of Internal Medicine, Division of Nephrology, Ankara, Turkey, 10Hacettepe University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, ankara, Turkey, 11Izmir Katip Celebi University, Faculty of Medicine, Division of Rheumatology, İzmir, Turkey

    Background/Purpose: Overall mortality in ANCA-associated vasculitides (AAVs) over the last two decades has been reported to be decreasing with the use of immunosuppressive therapies. However,…
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