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  • Abstract Number: 1776 • 2014 ACR/ARHP Annual Meeting

    Factors Predictive of ANCA-Associated Vasculitis Relapse in Patients Given Rituximab-Maintenance Therapy

    Benjamin Terrier1, Christian Pagnoux2, Guillaume Geri3, Alexandre Karras4, Chahéra Khouatra5, Olivier Aumaitre6, Pascal Cohen7, Francois Maurier8, Olivier Decaux9, Hélène Desmurs-Clavel10, Pierre Gobert11, Thomas Quemeneur12, Claire Blanchard-Delaunay13, Pascal Godmer14, Xavier Puéchal7, Luc Mouthon7 and Loïc Guillevin for the French Vasculitis Study Group7, 1National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, 2Division of Rheumatology, University of Toronto, Toronto, ON, Canada, 3Intensive Care Unit, Cochin Hospital, Paris, France, 4Nephrology, Hôpital Européen Georges Pompidou, APHP, Paris, France, 5CHU Louis Pradel, Lyon, Lyon, France, 6Internal Medicine, CHU, Clermont-Ferrand, France, 7National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Paris, France, 8HP Metz Belle Isle Hospital, Department of Internal Medicine, Metz, France, 9Department of Internal Medicine, Rennes University Hospital, Rennes, France, 10University of Lyon, LYON, France, 11Nephrology, Centre Hospitalier d'Avignon, Avignon, France, 12Internal Medicine, CH, Valenciennes, France, 13Internal Medicine, Hôpital de Niort, Niort, France, 14Department of Internal Medicine, Centre Hospitalier Bretagne Atlantique de Vannes, Vannes, France

    Background/Purpose Rituximab (RTX) was shown to be as effective as cyclophosphamide to induce remission in patients with ANCA-associated vasculitis (AAV). The prospective MAINRITSAN trial compared…
  • Abstract Number: 1775 • 2014 ACR/ARHP Annual Meeting

    Predicting Relapse in Patients with Granulomatosis with Polyangiitis – the Potential Use of Monitoring in Vitro ANCA Production

    Judith Land1, Wayel H. Abdulahad1, Coen A. Stegeman2, Peter Heeringa3 and Abraham Rutgers1, 1Rheumatology and Clinical Immunology, University Medical Center Groningen, Groningen, Netherlands, 2Nephrology, University Medical Center Groningen, Groningen, Netherlands, 3Pathology and Medical Biology, University Medical Center Groningen, Groningen, Netherlands

    Background/Purpose Patients with granulomatosis with polyangiitis (GPA) suffer from frequent disease relapses, with up to 50% of patients relapsing within 5 years. Several risk factors…
  • Abstract Number: 1774 • 2014 ACR/ARHP Annual Meeting

    Granulomatosis with Polyangiitis (Wegener’s): Endoscopic Management of Tracheobronchial Stenosis – Results from a Multicenter Experience in 47 Patients

    Benjamin Terrier1, Agnes Dechartres2, Charlotte Girard3, Stéphane Jouneau4, Jean-Emmanuel Kahn5, Robin Dhote6, Jean Cabane7, Estibaliz Lazaro8, Thomas Papo9, Nicolas Schleinitz10, Guillaume Le Guenno11, Luc Mouthon12 and Loïc Guillevin for the French Vasculitis Study Group12, 1National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, 2Biostatistics, Hotel Dieu, Paris, France, 3Internal Medicine, CHU, Lyon, France, 4Pulmonology, CHU, Rennes, France, 5Internal Medicine, Foch Hospital, Suresnes, France, 6Internal Medicine, Avicenne University Hospital, Bobigny, France, 7Internal Medicine, Saint-Antoine, Paris, France, 8Centre François Magendie, CHU, Pessac, France, 9Internal Medicine, Bichat Hospital, Paris, Paris, France, 10internal medicine, APHM, marseille, France, 11Internal Medicine department, Clermont-Ferrand, France, 12National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Paris, France

    Background/Purpose Tracheobronchial stenosis (TBS) is noted in 12 to 23% in patients with granulomatosis with polyangiitis (GPA), and includes subglottic stenosis (SGS) and bronchial stenosis.…
  • Abstract Number: 1773 • 2014 ACR/ARHP Annual Meeting

    Otolaryngologic Lesions Are Not Rare and Closely Related with Pachymeningitis and Cranial Neuropathy in MPO-ANCA Associated Vasculitis

    Takahiro Nunokawa1, Naoto Yokogawa2, Kota Shimada1 and Shoji Sugii2, 1Department of Rheumatic diseases, Tokyo Metropolitan Tama Medical Center, Tokyo, Japan, 2Department of Rheumatic Diseases, Tokyo Metropolitan Tama Medical Center, Tokyo, Japan

    Background/Purpose: Recently several case reports of serous otitis media (SOM), hypertrophic pachymeningitis (HP) and cranial neuropathy (CN) have been reported in connection with MPO-ANCA associated…
  • Abstract Number: 1772 • 2014 ACR/ARHP Annual Meeting

    Venous Thromboembolic Events in Eosinophilic Granulomatosis with Polyangiitis (EGPA)

    Chiara Baldini1, Francesco Ferro1, Nicoletta Luciano1, Antonio Tavoni2, Francesca Sernissi1, Daniela Martini1, Sara L'Abbate1, Marta Mosca3 and Stefano Bombardieri3, 1University of Pisa, Rheumatology Unit, Pisa, Italy, 2University of Pisa, Immunoallergology Unit, Pisa, Italy, 3Rheumatology Unit, University of Pisa, Pisa, Italy

    Background/Purpose: Previous studies have documented an increased risk of venous thromboembolic events in patients with antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides (AAV) as compared to…
  • Abstract Number: 1771 • 2014 ACR/ARHP Annual Meeting

    Arterial Thrombotic Events in Systemic Vasculitis

    Alexander Tsoukas1, Sasha Bernatsky2, Lawrence Joseph3, David Buckeridge4, Patrick Belisle5 and Christian A. Pineau1, 1Rheumatology, McGill University Health Centre, Montreal, QC, Canada, 2Divisions of Rheumatology and Clinical Epidemiology, McGill University Health Centre, Montreal, QC, Canada, 3McGill University, Montreal, QC, Canada, 4Department of Epidemiology, Biostatistics and Occupational Health, McGill University, Montreal, QC, Canada, 5Clinical Epidemiology, Research Institute of the McGill University Health Centre, Montreal, QC, Canada

    Background/Purpose To estimate the incidence rate of clinically apparent arterial thrombotic events and associated comorbidities in patients with primary systemic vasculitis. Methods Using large-cohort administrative…
  • Abstract Number: 1770 • 2014 ACR/ARHP Annual Meeting

    Increased Risk of Chronic Obstructive Pulmonary Disease in Granulomatosis with Polyangiitis: A General Population-Based Study

    Neda Amiri1, Mohsen Sadatsafavi2, Eric C. Sayre3, John M. Esdaile4 and J. Antonio Avina-Zubieta2,5, 1Rheumatology, University of British Columbia, Vancouver, BC, Canada, 2University of British Columbia, Vancouver, BC, Canada, 3Arthritis Research Centre of Canada, Richmond, BC, Canada, 4Rheumatology, University of British Columbia, Department of Medicine, Division of Rheumatology, Vancouver, BC, Canada, 5Rheumatology, Arthritis Research Centre of Canada, Richmond, BC, Canada

    Background/Purpose             Chronic obstructive pulmonary disease (COPD) is increasingly recognized as an inflammatory condition. We aimed to identify the risk of newly recorded COPD among…
  • Abstract Number: 1769 • 2014 ACR/ARHP Annual Meeting

    Abdominal Visceral Adipose Tissue Measured By DXA As a Novel Surrogate Marker of Cardiovascular Risk in Primary Necrotizing Vasculitides

    Bertrand Dunogué1, Karine Briot2, Sami Kolta3, Alexis Regent4, Pascal Cohen5, Alice Berezne6, Xavier Puéchal5, Claire Le Jeunne5, Luc Mouthon5, Christian Roux7, Loïc Guillevin for the French Vasculitis Study Group5 and Benjamin Terrier8, 1Internal Medicine, Hôpital Cochin, Paris, France, 2Cochin Hospital, Paris Descartes University, Paris, France, 3Rheumatology B, Paris Descartes University, Cochin hospital, Paris, France, 4Internal Medicine, Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes, Paris, France, Paris, France, 5National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Paris, France, 6Paris Descartes University, Internal Medicine department, Cochin Hospital, Paris, France, 7Paris Descartes University, Cochin Hospital, Paris, France, 8National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France

    Background/Purpose Studies have shown a strong prevalence of cardiovascular events among patients with primary necrotizing vasculitides. Recent studies indicate that visceral adipose tissue (VAT) is…
  • Abstract Number: 1768 • 2014 ACR/ARHP Annual Meeting

    Cardiac Involvement in Granulomatosis with Polyangiitis: A Magnetic Resonance Imaging Study of 31 Consecutive Patients

    Grégory Pugnet1,2, Xavier Puéchal1, Benjamin Terrier1, Hervé Gouya3, Andre Kahan4, Paul Legmann3, Loïc Guillevin1 and Olivier Vignaux5, 1National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Paris, France, 2Toulouse University Hospital, Internal Medicine Department, Toulouse, France, 3AP-HP Cochin Hospital, Department of Radiology B, Paris, France, 4Paris Descartes University, Rheumatology A department, Cochin Hospital, Paris, France, 5Radiology, Cochin University Hospital, Paris, France

    Background/Purpose Cardiac manifestations in granulomatosis with polyangiitis (GPA) patients are usually considered to be rare but may be life-threatening.  However specific cardiac involvement in GPA is…
  • Abstract Number: 1767 • 2014 ACR/ARHP Annual Meeting

    Granulomatosis with Polyangiitis (Wegener’s) According to Geographic Origin and Ethnicity: Clinical-Biological Presentation and Outcome

    Benjamin Terrier1, Christophe Deligny2, Xavier Puéchal3, Pascal Godmer4, Pierre Charles5, Gilles Hayem6, Bertrand Dunogué7, Pascal Cohen3, Serge Arfi8, Luc Mouthon3 and Loïc Guillevin for the French Vasculitis Study Group3, 1National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, 2Internal Medicine, CHU Fort de France, Fort de France, France, 3National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Paris, France, 4Department of Internal Medicine, Centre Hospitalier Bretagne Atlantique de Vannes, Vannes, France, 5Internal Medicine, Institut Mutualiste Montsouris, Paris, France, 6Rhumatologie, Hopital Bichat, Paris Cedex 18, France, 7Internal Medicine, Hôpital Cochin, Paris, France, 8Rhumatologie Et Médecine Interne, Centre hospitalier Universitaire de Fort de France, Fort de France, Martinique

    Background/Purpose Granulomatosis with polyangiitis (Wegener’s) (GPA) is an ANCA-associated vasculitis (AAV) predominantly affecting small-sized vessels, involving mainly the upper and lower respiratory tracts and kidneys.…
  • Abstract Number: 1766 • 2014 ACR/ARHP Annual Meeting

    Comparison of Clinicopathologically- and Serologically-Based Classification Systems for ANCA-Associated Vasculitis

    Sebastian Unizony1, Eli M. Miloslavsky1, Miguel Villarreal2, Peter A. Merkel3, Paul Monach4, E. William St. Claire5, Cees Kallenberg6, David Ikle2, Robert F. Spiera7, Nadia Tchao8, Deborah J. Phippard8, Linna Ding9, Carol A. Langford10, Philip Seo11, Gary S. Hoffman12, John H. Stone1 and Ulrich Specks13, 1Rheumatology, Massachusetts General Hospital, Boston, MA, 2Rho, Chapel Hill, NC, 3University of Pennsylvania, Philadelphia, PA, 4Rheumatology, Boston University, Boston, MA, 5Rheumatology, Duke University School of Medicine, Durham, NC, 6Rheumatology and Clinical Immunology, University of Groningen, Groningen, Netherlands, 7Rheumatology, Hospital for Special Surgery, New York, NY, 8Immune Tolerance Network, Bethesda, MD, 9NIAID, Bethesda, MD, 10Center for Vasculitis Care and Research, Cleveland Clinic, Cleveland, OH, 11Rheumatology Division, Johns Hopkins Vasculitis Center, Johns Hopkins University, Baltimore, MD, 12Center for Vasculitis Care and Research, Cleveland Clinic Foundation, Cleveland, OH, 13Frederichs Dr NW, Mayo Clinic, Rochester, MN

    Background/Purpose Genome-wide association studies suggest that PR3-ANCA-positive ANCA-associated vasculitis (AAV) is genetically distinct from MPO-ANCA AAV.  We evaluated patients enrolled in the RAVE trial according…
  • Abstract Number: 1765 • 2014 ACR/ARHP Annual Meeting

    Comparison of Clinical Characteristics of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis By the Serotype Specificity to Myeloperoxidase and Proteinase-3

    Takamasa Murosaki, Takeo Sato, Yoichiro Akiyama, Katsuya Nagatani and Seiji Minota, Division of Rheumatology and Clinical Immunology, Jichi Medical University, Tochigi, Japan

    Background/Purpose: To correlate the clinical characteristics of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with myeloperoxidase (MPO)-ANCA and proteinase-3 (PR3)-ANCA, and to detect clinical characteristics of…
  • Abstract Number: 1784 • 2014 ACR/ARHP Annual Meeting

    Prognostic Factors for Interstitial Lung Disease with Microscopic Polyangiitis

    Takeshi Shoda, Tohru Takeuchi, Takaaki Ishida, Hideyuki Shiba, Youhei Fujiki, Daisuke Wakura, Shuzo Yoshida, Takuya Kotani, Shigeki Makino and Toshiaki Hanafusa, Department of Internal Medicine (I), Osaka medical college, Osaka, Japan

    Background/Purpose Many patients with interstitial lung disease (ILD) complicated by microscopic polyangiitis (MPA) show a UIP pattern on chest CT. The prognosis is poorer than…
  • Abstract Number: 1783 • 2014 ACR/ARHP Annual Meeting

    The Importance of Histopathological Classification of ANCA-Associated Glomerulonephritis in Renal Function and Renal Survival

    Valeria Scaglioni1, Marina Scolnik1, Luis J. Catoggio1, Carlos Federico Varela2, Gustavo Greloni2, Silvia Christiansen3 and Enrique R. Soriano1, 1Rheumatology Unit, Internal Medicine Service, Hospital Italiano de Buenos Aires, Instituto Universitario Hospital Italiano de Buenos Aires, and Fundacion PM Catoggio, Buenos Aires, Argentina, 2Nephrology Service. Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, 3Pathology Service. Hospital Italiano de Buenos Aires, Buenos Aires, Argentina

    Background/Purpose : Histological changes in renal biopsy are the gold standard for establishing the diagnosis of antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (GN). In 2010 a…
  • Abstract Number: 1782 • 2014 ACR/ARHP Annual Meeting

    Long-Term Follow-up of Non-HBV Polyarteritis Nodosa and Microscopic Polyangiitis with Poor-Prognosis Factors

    Maxime Samson1, Xavier Puéchal2, Hervé Devilliers3, Pascal Cohen2, Boris Bienvenu4, Kim Heang Ly5, Alain Bruet6, Brigitte Gilson7, Marc Ruivard8, Edouard Pertuiset9, Mohamed Hamidou10, Benjamin Terrier2, Christian Pagnoux11, Luc Mouthon2, Loic Guillevin12 and The French Vasculitis Study Group (FVSG)12, 1Department of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France; Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France, 2National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Paris, France, 3Dijon University Hospital, Department of internal medicine and systemic diseases, Dijon, France, 4Médecine interne, CHU Côte de Nacre, CAEN, France, 5CHU Dupuytren, Limoges, Limoges, France, 6Department of Nephrology and Internal Medicine, CH de Poissy Saint-Germain-en-Laye, Poissy, France, 7Department of Nephrology and Internal Medicine, CH de Verdun, Verdun, France, 8CHU Estaing, Clermont–Ferrand, Clermont–Ferrand, France, 9Rheumatology, René Dubos Hospital, Pontoise, France, 10CHU Hôtel Dieu, Nantes, Nantes, France, 11Division of Rheumatology, University of Toronto, Toronto, ON, Canada, 12Department of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France

    Background/Purpose To study the long-term outcomes of 65 patients with non-HBV polyarteritis nodosa (PAN) or microscopic polyangiitis (MPA) enrolled in a prospective, randomized, open-label trial,1…
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