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  • Abstract Number: 1735 • 2018 ACR/ARHP Annual Meeting

    Diagnostic Accuracy of MR Angiography in a Cohort with Systemic Sclerosis Compared to Other Rheumatic Diseases with Acral Hypoperfusion

    Diana Dan1,2, Simon Saner1, Andrea D. Gloor1,3, Harald Bonel4, Jennifer L. Cullmann4 and Peter M. Villiger1, 1Department of Rheumatology, Immunology and Allergology, Inselspital, University Hospital of Bern, Bern, Switzerland, 2Service of Rheumatology, Lausanne University Hospital, Lausanne, Switzerland, 3Dermatology, Inselspital, University Hospital of Bern, Bern, Switzerland, 4Institute of Diagnostic, Interventional and Pediatric Radiology, Inselspital, University Hospital of Bern, Bern, Switzerland

    Background/Purpose: Vasculopathy is a major feature of systemic sclerosis (SSc). It leads to intimal proliferation and adventitial fibrosis of small and large arterial blood vessels.…
  • Abstract Number: 1736 • 2018 ACR/ARHP Annual Meeting

    Incidence and Risk Factors for Gangrene in Patients with Systemic Sclerosis from the Eustar Cohort

    Carina Mihai1,2, Oliver Distler1, Ana Maria Gheorghiu2,3, Paul Iulian Constantin2, Rucsandra Dobrota1,2, Suzana Jordan4, Vanessa Smith5, Eric Hachulla6, Joerg C. Henes7, Elise Siegert8, Serena Vettori9, Ulf Müller-Ladner10, Marco Matucci-Cerinic11 and Yannick Allanore12, 1Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 2Carol Davila University of Medicine and Pharmacy, Bucharest, Romania, 3Internal Medicine and Rheumatology, Carol Davila University of Medicine and Pharmacy, Cantacuzino Hospital, Bucharest, Romania, 4Rheumatology, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 5Department of Rheumatology, Ghent University Hospital, Ghent, Belgium, 6Department of Internal Medicine and Clinical Immunology, Hôpital Claude Huriez, University of Lille, Lille, France, 7Department of Internal Medicine II, Division of Rheumatology, University Hospital Tuebingen, Tuebingen, Germany, 8Rheumatology and Clinical Immunology, Charité - University Medicine Berlin, Berlin, Germany, 9Rheumatology Unit, Department of Internal and Experimental Medicine University of Campania "Luigi Vanvitelli", Napoli, Italy, 10Dept. of Rheumatology and Clinical Immunology, Campus Kerckhoff, Justus-Liebig-University Giessen, Germany, Bad Nauheim, Germany, 11Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, 12Cochin Hospital, Paris Descartes University, Paris, France, Paris, France

    Background/Purpose: In patients with systemic sclerosis (SSc), peripheral vasculopathy can promote critical ischemia and gangrene that are very severe complications with potential life-threatening consequences. Recently…
  • Abstract Number: 1737 • 2018 ACR/ARHP Annual Meeting

    Multiplexed Autoantibody Profiles in a Systemic Sclerosis Clinical Trial Comparing Autologous Hematopoietic Stem Cell Transplantation and Cyclophosphamide

    Paul J. Utz1 and Burcu Ayoglu2, 1Medicine, Stanford University School of Medicine, Stanford, CA, 2Medicine, Stanford University, STANFORD, CA

     Background/Purpose: In a randomized, open-label, phase II clinical trial (SCOT, Scleroderma: Cyclophosphamide or Transplantation)1, subjects were randomly assigned to treatment with myeloablative CD34+ selected autologous…
  • Abstract Number: 1738 • 2018 ACR/ARHP Annual Meeting

    Detect Outperforms Echocardiography Based Screening Guidelines for Early Detection of Systemic Sclerosis Associated Pulmonary Arterial Hypertension

    Amber Young1, Victor M. Moles2,3, Vivek Nagaraja1, Dharshan Vummidi4, Scott H. Visovatti3, Vallerie McLaughlin3 and Dinesh Khanna1, 1Division of Rheumatology, Department of Internal Medicine, University of Michigan Scleroderma Program, University of Michigan, Ann Arbor, MI, 2Hospital Jose Maria Cullen, Santa Fe, Argentina, 3Division of Cardiology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, 4Department of Radiology, University of Michigan, Ann Arbor, MI

    Background/Purpose: Pulmonary arterial hypertension (PAH) is a leading cause of mortality in patients with systemic sclerosis (SSc). Active screening can detect SSc-PAH earlier and may…
  • Abstract Number: 1739 • 2018 ACR/ARHP Annual Meeting

    What Do Patient Reported Outcomes for Routine Monitoring of Gastrointestinal Tract Symptoms in a Systemic Sclerosis Center Tell Us about Clinical Features of Potential Small Intestinal Bacterial Overgrowth?

    Jessica Zhu1, Craig Gale2, Joshua Biber3, Mandana Nikpour4, Murray Baron5 and Tracy M. Frech6, 1School of Medicine, University of Utah, Salt Lake City, UT, 2Bioinformatics, University of Utah, Salt Lake City, UT, 3University of Utah, Salt Lake City, UT, 4The University of Melbourne, Melbourne, Australia, Melbourne, Australia, 5Department of Medicine, McGill University, Montreal, QC, Canada, 6Division of Rheumatology, University of Utah, Salt Lake City, UT

    Background/Purpose: The implementation of patient reported outcome measures (PROMs) is increasingly recognized as a critical aspect of clinical practice.  The use of gastrointestinal (GI) PROMs…
  • Abstract Number: 1740 • 2018 ACR/ARHP Annual Meeting

    Effectiveness and Safety of Tocilizumab for the Treatment of Refractory Systemic Sclerosis Associated Interstitial Lung Disease: A Case Series

    Judit Lluch1, Ivan Castellvi2, J Alegre3, Pablo Juárez1, Joan Miquel Nolla1 and Francisco Javier Narváez4, 1Rheumatology, Hospital Universitario de Bellvitge, Barcelona, Spain, 2Rheumatology, Hospital Santa Creu i San Pau, Barcelona, Spain, 3Rheumatology, Hospital Universitario Doctor Peset. Valencia. Spain, Valencia, Spain, 4Rheumatology Department, Hospital Universitario de Bellvitge, Idibell, Barcelona, Spain

    Background/Purpose: To evaluate the efficacy and safety of TCZ as as a rescue therapy in patients with refractory scleroderma-associated interstitial lung disease (SSc-ILD). Methods: We…
  • Abstract Number: 1741 • 2018 ACR/ARHP Annual Meeting

    Disease Duration and Autoantibodies Predict Distinct Skin Score Trajectories in Diffuse Cutaneous Systemic Sclerosis

    Svetlana I. Nihtyanova1, Alper Sari2, Voon H. Ong3 and Christopher P. Denton4, 1Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, 2Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey, 3Division of Medicine, University College London, London, United Kingdom, 4University College London, London, United Kingdom

    Background/Purpose: Although severity of skin involvement and change in skin thickness over time vary substantially between patients with diffuse cutaneous systemic sclerosis (dcSSc), for the…
  • Abstract Number: 1742 • 2018 ACR/ARHP Annual Meeting

    Disease-Specific Autoantibodies Associate with Remarkably Different Risk of Development of Significant Lung Fibrosis in Systemic Sclerosis

    Svetlana I. Nihtyanova1, Alper Sari2, Anna Leslie3, Voon H. Ong4 and Christopher P. Denton5, 1Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, 2Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey, 3Division of Medicine, Centre for Rheumatology and Connective Tissue Disease, University College London, London, United Kingdom, 4Division of Medicine, University College London, London, United Kingdom, 5University College London, London, United Kingdom

    Background/Purpose: Pulmonary fibrosis (PF) is a leading cause of disease-related death in SSc patients. Some studies suggest that the timing of PF development differs between…
  • Abstract Number: 1743 • 2018 ACR/ARHP Annual Meeting

    Serum Markers Potentially Associated with PAH in Systemic Sclerosis; A Targeted Screening Approach

    Anders Heiervang Tennøe1, Håvard Fretheim2, Oyvind Midtvedt2, Torhild Garen2, Thor Ueland3, Pål Aukrust2, Arne K Andreassen2, Einar Gude2, Øyvind Molberg2 and Anna-Maria Hoffmann-Vold2, 1Rheumatology, Oslo University Hospital, Oslo, Norway, 2Oslo University Hospital, Oslo, Norway, 3Research Institute of Internal Medicine Research, Oslo University Hospital, Oslo, Norway

    Background/Purpose: Pulmonary arterial hypertension (PAH) is a feared complication in systemic sclerosis (SSc). Detection of PAH at a preclinical stage is important as early diagnosis…
  • Abstract Number: 1744 • 2018 ACR/ARHP Annual Meeting

    Prognostic Value of Right Heart Involvement in Systemic Sclerosis:Not Only Pulmonary Arterial Hypertension

    Luna Gargani1, Cosimo Bruni2, Antonella Moreo3, Laura Belloli3, Silvia Bellando-Randone2, Oscar Massimiliano Epis3, Francesco Ferrara4, Olga Vriz5, Eduardo Bossone6, Petar M. Seferovic7, Daniel E. Furst8 and Marco Matucci-Cerinic9, 1Institute of Clinical Physiology, Consiglio Nazionale delle Ricerche (CNR), Pisa, Italy, 2Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Firenze, Italy, 3Niguarda Ca’ Granda Hospital, Milan, Italy, 4"Cava de' Tirreni and Amalfi Coast", University Hospital, Salerno, Italy, 5King Faisal Hospital, Riyadh, Saudi Arabia, 6Cardarelli Hospital, Naples, Italy, 7Department of Cardiology, , Clinical Center of Serbia and Belgrade University School of Medicine, Belgrade, Serbia, 8University of California Los Angeles, Los Angeles, CA, 9Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy

    Background/Purpose: In SSc, cardiac dysfunction is associated with poor prognosis. Right ventricular involvement is frequently secondary to PAH. Echocardiography is the routine imaging tool to…
  • Abstract Number: 1745 • 2018 ACR/ARHP Annual Meeting

    Efficacy of Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss) Treatments According to the Type of Manifestations Based on Analysis of 376 Patients

    Matthias Papo1, Giacomo Emmi2, Franco Schiavon3, Matthieu Groh4, Maria-Letizia Urban2, Chiara Marvisi5, Jean-Emmanuel Kahn4, Alberto Sinico6, Maxime Samson7, Pascal Cohen1, Xavier Puéchal1, Luc Mouthon1, Loïc Guillevin1, Augusto Vaglio5 and Benjamin Terrier8, 1Department of Internal Medicine, INSERM Unité 1016, Centre de Référence pour les Maladies Auto-immunes Rares, Hôpital Cochin, Université Paris Descartes, Sorbonne Paris Cité, Paris, France, 2Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, 3Operative Unit of Rheumatology, Department of Medicine DIMED, University of Padova, Padova, Italy, 4Service de Médecine Interne, Centre de Référence des Syndromes Hyperéosinophiliques-CEREO, Hôpital Foch, Université Versailles–Saint-Quentin-en-Yvelines, Suresnes, France, 5Nephrology Unit, Parma University Hospital, Parma, Italy, 6UOS di Immunologia Clinica e UOC di Nefrologia, Azienda Ospedaliera San Carlo Borromeo, Milan, Italy, 7Department of Internal Medicine and Clinical Immunology, François-Mitterrand Teaching Hospital, University of Bourgogne-Franche-Comté, Dijon, France, 8Department of Internal Medicine, Centre de Référence pour les Maladies Auto-immunes Rares, Paris, France, Hôpital Cochin, Université Paris Descartes, Sorbonne Paris Cité, Paris, France

    Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg–Strauss) is a small-vessel necrotizing vasculitis characterized by blood and tissue eosinophilia and asthma. Glucocorticoids (GCs) represent the cornerstone…
  • Abstract Number: 1746 • 2018 ACR/ARHP Annual Meeting

    Impact of Interstitial Lung Disease on the Long-Term Survival in 76 Japanese Patients with Microscopic Polyangiitis

    Hiromi Matsushita1, Yoshioki Yamasaki2, Yukiko Takakuwa3, Hidehiro Yamada4 and Kimito Kawahata3, 1Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Japan, 2Internal Medicie, St. Marianna University School of Medicine, Kawasaki, Japan, 3Division of Rheumatology and Allergology, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Japan, 4Rheumatology, Seirei Yokohama Hospital, Yokohama, Japan

    Background/Purpose: ANCA-associated vasculitis including microscopic polyangiitis (MPA) involves multiple organs including lungs. Clinical characteristics and impact on long-term survival in MPA patients with interstitial lung disease (ILD) have not…
  • Abstract Number: 1747 • 2018 ACR/ARHP Annual Meeting

    Long-Term Outcomes of Patients with Nonsevere Eosinophilic Granulomatosis with Polyangiitis Given Azathioprine and Glucocorticoids for Remission Induction

    Xavier Puéchal1, Christian Pagnoux2, Gabriel Baron3, François Lifermann4, Loïk Geffray5, Thomas Quémeneur6, Jean-Luc Saraux7, Marie Wislez8, Vincent Cottin9, Marc Ruivard10, Nicolas Limal11, Achille Aouba12, Bernard Bonnotte13, Antoine Neel14, Christian Agard15, Pascal Cohen16, Benjamin Terrier17, Claire Le Jeunne17, Luc Mouthon1, Philippe Ravaud3 and Loïc Guillevin for the French Vasculitis Study Group17, 1Department of Internal Medicine, INSERM Unité 1016, Centre de Référence pour les Maladies Auto-immunes Rares, National Referral Center for Rare Systemic Autoimmune Diseases, Paris Cochin, France, Paris, France, 2Division of Rheumatology, Division of Rheumatology, Mount Sinai Hospital, Toronto, ON, Canada, 3Paris Hôtel Dieu, Paris, France, 4Dax, Dax, France, 5Lisieux, Lisieux, France, 6Department of Internal Medicine, CH of Valenciennes, France, Valenciennes, France, 7Eaubonne, Eaubonne, France, 8Paris Tenon, Paris, Gambia, 9Lyon Louis Pradel, Lyon, France, 10Clermont-Ferrand, Clermont Ferrand, France, 11Créteil Henri Mondor, Créteil, France, 12Caen, Caen, France, 13Dijon, Dijon, France, 14Medecine Interne, Nantes Hôtel Dieu, Nantes, France, 15Nantes Hôtel Dieu, Nantes, France, 16Department of Internal Medicine, INSERM Unité 1016, Centre de Référence pour les Maladies Auto-immunes Rares, Hôpital Cochin, Université Paris Descartes, Sorbonne Paris Cité, Paris, France, 17National Referral Center for Rare Systemic Autoimmune Diseases, Paris Cochin, France, Paris, France

    Background/Purpose: At month 24 (M24), CHUSPAN2-trial patients with nonsevere eosinophilic granulomatosis with polyangiitis (EGPA), who received 1 yr of azathioprine (AZA) and glucocorticoids (GC) as…
  • Abstract Number: 1748 • 2018 ACR/ARHP Annual Meeting

    Interstitial Lung Disease in ANCA-Positive Vasculitis Patients

    Andy Abril1, Minkyung Kwon,2, Isabel Mira-Avendano3, Carlos Rojas4 and Andras Khoor5, 1Rheumatology, Mayo Clinic Florida, Jacksonville, FL, 2Pulmonary and Critical Care, Mayo Clinic, Jacksonville, FL, 3Pulmonary and Critical Care, Mayo Clinic Florida, Jacksonville, FL, 4Radiology, Mayo Clinic, Jacksonville, FL, 5Pathology, Mayo Clinic, Jacksonville, FL

    Background/Purpose: Rarely, ANCA-positive vasculitis patients are found to have interstitial lung disease (ILD). Clinical characteristics and prognosis are not well known in these patients. The…
  • Abstract Number: 1749 • 2018 ACR/ARHP Annual Meeting

    Long-Term Renal Outcome in Pulmonary-Limited Microscopic Polyangiitis

    Hironari Hanaoka, Harunobu Iida, Tomofumi Kiyokawa, Yukiko Takakuwa and Kimito Kawahata, Division of Rheumatology and Allergology, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Japan

    Background/Purpose: Patients with pulmonary-renal or renal-limited microscopic polyangiitis (MPA) frequently manifested with rapidly progressive glomerular nephritis resulting in chronic renal failure if clinical response to…
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