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  • Abstract Number: 1678 • 2019 ACR/ARP Annual Meeting

    Interstitial Lung Disease in ANCA Associated Vasculitis: A Single Center Retrospective Analysis

    Haidy Adel Youssef1 and Mehrnaz Hojjati 2, 1Riverside Community Hospital/ UCR School of Medicine Internal Medicine Residency Program, Corona, CA, 2Department of Rheumatology, Loma Linda University, Loma Linda, CA

    Background/Purpose: ANCA associated vasculitis patients have a wide spectrum of pulmonary involvement in the form nodular disease (especially peri-bronchial nodules), cavitating lesions, diffuse alveolar hemorrhage and less…
  • 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: 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: 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: 1682 • 2019 ACR/ARP Annual Meeting

    Inpatient Epidemiology of Granulomatosis with Polyangiitis in the United States

    Patompong Ungprasert1, Matthew Koster 2, Wisit Cheungpasitporn 3, Karn Wijarnpreecha 4, Charat Thongprayoon 2 and Paul Kroner 5, 1Cleveland Clinic, Cleveland, OH, Bangkok, Thailand, 2Mayo Clinic Rochester, Rochester, MN, 3University of Mississippi Medical Center, Jackson, MS, 4Mayo Clinic Florida, Jacksonville, FL, 5Mayo Clinic Florida, Jacksonville

    Background/Purpose: Granulomatosis with polyangiitis (GPA, formerly known and Wegener’s granulomatosis) is a major subtype of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis characterized by granulomatous inflammation of…
  • Abstract Number: 1683 • 2019 ACR/ARP Annual Meeting

    Cardiac Involvement of Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss): Initial Manifestations and Outcomes Based on Data from a Monocenter Patient Cohort

    Silvia Sartorelli 1, Pascal Cohen 2, Bertrand Dunogue 2, Alexis Régent 2, Xavier Puéchal for the French Vasculitis Study Group 2, Luc Mouthon 2, Loic Guillevin 2 and Benjamin Terrier2, 1Vita-Salute San Raffaele University, IRCCS San Raffaele Hospital, Milan, Italy, 2National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France

    Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis characterized by blood and tissue eosinophilia, and asthma. Its cardiac involvement is a major…
  • Abstract Number: 1684 • 2019 ACR/ARP Annual Meeting

    Remission and Low Disease Activity State in Patients with Granulomatosis with Polyangiitis and Microscopic Polyangiitis: Prevalence and Impact on Damage Accrual

    Paolo Delvino 1, Federica Sardanelli 2, Pascal Cohen 3, Xavier Puéchal for the French Vasculitis Study Group 3, Luc Mouthon 3, Loic Guillevin 3 and Benjamin Terrier3, 1Rheumatology Department, IRCCS Policlinico S. Matteo Foundation, University of Pavia, Vercelli, Italy, 2Department of Internal Medicine , Clinical Immunology Unit of Genoa and Policlinico San Martino, Genova, Italy, 3National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France

    Background/Purpose: Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) require glucocorticoids (GCs) and immunosuppressants (IS) to induce and maintain remission. At the era of highly…
  • Abstract Number: 1685 • 2019 ACR/ARP Annual Meeting

    Inpatient Burden, Expenditures and Comorbidities of Polyarteritis Nodosa: National Inpatient Sample 2014

    Patompong Ungprasert1, Matthew Koster 2, Wisit Cheungpasitporn 3, Karn Wijarnpreecha 4, Charat Thongprayoon 2 and Paul Kroner 5, 1Cleveland Clinic, Cleveland, OH, Bangkok, Thailand, 2Mayo Clinic Rochester, Rochester, MN, 3University of Mississippi Medical Center, Jackson, MS, 4Mayo Clinic Florida, Jacksonville, FL, 5Mayo Clinic Florida, Jacksonville

    Background/Purpose: Due to the rarity of polyarteritis nodosa (PAN), no study has ever investigated inpatient characteristics, healthcare utilization and frequency of comorbidities of patients with…
  • Abstract Number: 1686 • 2019 ACR/ARP Annual Meeting

    Risk of Vasculitis Associated with Inflammatory Bowel Diseases: Evidence for a Role of TNF-α Blockers

    Benjamin Terrier1, Laurent Beaugerie 2, Philippe Seksik 2, Harry Sokol 2 and Julien Kirchgesner 2, 1National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France, 2Saint-Antoine Hospital, Paris, France

    Background/Purpose: An increased risk of side effects, especially infections, has been reported among patients receiving tumor necrosis factor (TNF)-α blockers. Some leukocytoclastic cutaneous vasculitides reportedly occurred…
  • 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: 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: 1689 • 2019 ACR/ARP Annual Meeting

    Off-Label Use of Biotherapies to Treat Relapsing And/or Refractory Polyarteritis Nodosa

    Alice Canzian 1, Omer Karadag 2, Anne Contis 3, François Maurier 4, Silvia Sartorelli 5, Laure Denis 6, Sébastien Sanges 7, Claire de Moreuil 8, Cécile-Audrey Durel 9, Stéphane Durupt 10, Marie Jachiet 11, Diane Rouzaud 12, Carlo Salvarani 13, Franco Schiavon 14, Lorenzo Dagna 15, Fabrice Bonnet 16, David Jayne 17, Loic Guillevin 18 and Benjamin Terrier1, 1Cochin Hospital, Paris, France, 2Hacettepe University Vasculitis Centre, Ankara, Turkey / Vasculitis and Lupus Clinic, Addenbrooke’s Hospital, University of Cambridge, UK, Ankara, Turkey, 3Department of Internal Medicine and Clinical Immunology , Hôpital Saint-André, CHU de Bordeaux , Bordeaux , France, Bordeaux, France, 4Service de Médecine Interne, Hôpital Belle Isle, Metz, Metz, France, 5Vita-Salute San Raffaele University, IRCCS San Raffaele Hospital, Milan, Italy, 6Clermont-Ferrand Hospital, Clermont-Ferrand, France, 7CHRU Lille, Lille, France, 8CHU Brest, Brest, France, 9Department of Internal Medicine, Hôpital Edouard Herriot, Lyon, France, Lyon, France, 10Lyon Hospital, Lyon, France, 11Saint Louis Hospital, Paris, France, 12Bichat Hospital, Paris, France, 13Division of Rheumatology, Arcispedale Santa Maria Nuova-IRCCS, Reggio Emilia, Italy, 14Operative Unit of Rheumatology, Department of Medicine DIMED, University of Padova, Padova, Italy, Padova, Italy, 15Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, Vita-Salute San Raffaele University, Milan, Italy, 16CHU Bordeaux, Bordeaux, France, 17Vasculitis and Lupus Clinic, Addenbrooke’s Hospital, University of Cambridge, UK, Cambridge, United Kingdom, 18National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France

    Background/Purpose: Polyarteritis nodosa (PAN) is a rare systemic necrotizing vasculitis of medium- and small-sized arteries, not associated with antineutrophil cytoplasmic antibodies (ANCA). Conventional treatments include…
  • Abstract Number: 1690 • 2019 ACR/ARP Annual Meeting

    The Long-term Outcome of Patients with Arthritis of Behçet’s Disease

    Hirotoshi Kikuchi1, Takafumi Tomizuka 1, Kurumi Asako 1, Hajime Kono 1 and Shunsei Hirohata 2, 1Teikyo University School of Medicine, Tokyo, Japan, 2Nobuharra Hospital & Institute of Biomechanics, Tokyo, Japan

    Background/Purpose: Behçet’s disease (BD) is characterized by recurrent aphthous stomatitis, eye lesions, skin lesions, and genital ulcer. In addition to these symptoms, Behçet’s disease is…
  • Abstract Number: 1691 • 2019 ACR/ARP Annual Meeting

    Efficacy of TNF α Inhibitors for Refractory Vascular Behcet’s Disease: A Multicenter Observational Study of 27 Patients

    Aysun Aksoy 1, Ayten Yazici 2, Ahmet Omma 3, Ayşe Cefle 4, Fatos Onen 5, Unal Tasdemir 6, Tulin Ergun 7, Haner Direskeneli 8 and Fatma Alibaz-Oner9, 1Marmara University Faculty of Medicine, Division of Internal Medicine, Department of Rheumatology, Istanbul, Turkey; University of Manchester, Division of Musculoskeletal and Dermatological Sciences, Manchester, United Kingdom, Istanbul, Istanbul, Turkey, 2Kocaeli University, Faculty of Medicine, Division of Rheumatology, Kocaeli, Kocaeli, Turkey, 3Health Sciences University, Ankara Numune Training and Research Hospital, Department of Rheumatology, Ankara, Turkey, 4Division of Rheumatology, Kocaeli University School of Medicine, Kocaeli, Turkey, Kocaeli, Turkey, 5Dokuz Eylul University School of Medicine, Division of Rheumatology, İzmir, Turkey, 6Dokuz Eylul University, School of Medicine Division of Internal Medicine Rheumatology Department, izmir, Turkey, 7Marmara University School of Medicine Dermatology Department, istanbul, Turkey, 8Marmara University, School of Medicine, Division of Rheumatology, Istanbul, Turkey, 9Marmara University Faculty of Medicine,Department of Rheumatology,Istanbul,Turkey, Istanbul, Turkey

    Background/Purpose: Vascular involvement is one of the major causes of morbidity and mortality in Behcet’s Disease (BD) patients. Immunosuppressive (IS) agents are the mainstay of…
  • Abstract Number: 1692 • 2019 ACR/ARP Annual Meeting

    Long-Term Follow-Up of Anti-IL6-Receptor Tocilizumab in Refractory Uveitis in Patients with Behçet Disease: Multicenter Study of 14 Patients in Clinical Practice

    Iñigo Gonzalez-Mazon1, Lara Sanchez-Bilbao 2, Belén Atienza-Mateo 2, Jose Luis Martín-Varillas 2, DIANA PRIETO- PENA 1, Vanesa Calvo-Río 3, Rosalia Demetrio 4, Natalia Palmou-Fontana 1, Javier Loricera 5, Emma Beltrán Catalán 6, Marisa Hernández Garfella 7, Elia Valls-Pascual 8, Antonio Atanés 9, Miguel Cordero Coma 9, Joan M Nolla 10, Carmen carrasco-Cubero 11, Julio Sánchez 12, Miguel A Gonzalez-Gay 2 and Ricardo Blanco 2, 1Rheumatology, Hospital Universitario Marques de Valdecilla, Santander, Cantabria, Spain, 2Rheumatology, Hospital Universitario Marques de Valdecilla, Santander, Spain, 3Hospital Marqués de Valdecilla, Santander, Cantabria, Spain, 4Hospital Universitario Marqués de Valdecilla, Santander, Spain, 5H.U. Marques de Valdecilla, Santander, Spain, 6Hospital del Mar, Barcelona, Spain, 7H.G.U. Valencia, Valencia, 8Dr Peset University Hospital, Valencia, Spain, 9HUAC La Coruña, Coruña, Spain, 10Hospital Universitari de Bellvitge, Hospitalet, Spain, 11CHU de Badajoz, Badajoz, Spain, 12Hospital 12 de Octubre, Madrid, Spain

    Background/Purpose: Ocular involvement in Behçet’s disease (BD) is a potential severe and disabling complication. Anti-TNF-α agents have shown an improvement of visual outcome in BD-related…
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