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  • Abstract Number: 2677 • 2017 ACR/ARHP Annual Meeting

    Impact of Rheumatologic Evaluation and Serologic Testing on Patients with Fibrotic Interstitial Lung Disease: A Single Center Retrospective Experience

    Lindsey A. MacFarlane1 and Paul F. Dellaripa2, 1Rheumatology, Brigham & Women's Hospital, Boston, MA, 2Rheumatology, Brigham and Women’s Hospital, Boston, MD

    Background/Purpose: The evaluation of patients who develop interstitial lung disease (ILD) includes an assessment for underlying rheumatic disease or connective tissue disease (CTD). Delineating a…
  • Abstract Number: 2678 • 2017 ACR/ARHP Annual Meeting

    Comparison of Scleroderma Associated Isolated Pulmonary Arterial Hypertension and Pulmonary Hypertension with Concomitant Interstitial Lung Disease

    Alexander Hannan1, Raed Dweik2, Kristin B. Highland3, Gustavo Heresi4, Adriano Tonelli5, William Messner6 and Soumya Chatterjee1,7, 1Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, 2Respiratory Institute, Cleveland Clinic, Cleveland, OH, 3Rheumatology.org, Cleveland Clinic, Cleveland, OH, 4Respiratory Institute - Pulmonary Medicine, Cleveland Clinic, Cleveland, OH, 5Pulmonary Medicine - Respiratory Institute, Cleveland Clinic, Cleveland, OH, 6Quantitative Health Sciences, Cleveland Clinic, Cleveland, OH, 7Rheumatic and Immunologic Ds, Cleveland Clinic, Cleveland, OH

    Background/Purpose: Relatively little data exist in the literature to characterize the differences between patients with scleroderma-associated isolated pulmonary arterial hypertension (SSc-PAH, WHO Group 1) and…
  • Abstract Number: 2679 • 2017 ACR/ARHP Annual Meeting

    Mortality Is Increased in Scleroderma Associated Pulmonary Arterial Hypertension Patients with Younger Age, Lower Systolic Blood Pressure, and Lower Cardiac Index, but Not in Idiopathic Pulmonary Arterial Hypertension

    Alexander Hannan1, Raed Dweik2, Kristin B. Highland3, Gustavo Heresi4, Adriano Tonelli5, William Messner6 and Soumya Chatterjee1, 1Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, 2Respiratory Institute, Cleveland Clinic, Cleveland, OH, 3Rheumatology.org, Cleveland Clinic, Cleveland, OH, 4Respiratory Institute - Pulmonary Medicine, Cleveland Clinic, Cleveland, OH, 5Pulmonary Medicine - Respiratory Institute, Cleveland Clinic, Cleveland, OH, 6Quantitative Health Sciences, Cleveland Clinic, Cleveland, OH

    Background/Purpose: Survival in scleroderma associated pulmonary arterial hypertension (SSc-PAH) is known to be significantly worse compared to that in idiopathic pulmonary arterial hypertension (iPAH). However,…
  • Abstract Number: 2680 • 2017 ACR/ARHP Annual Meeting

    Analysis of Prognostic Determinants of Scleroderma-Associated Pulmonary Arterial Hypertension and Idiopathic Pulmonary Arterial Hypertension

    Alexander Hannan1, Raed Dweik2, Kristin B. Highland3, Gustavo Heresi4, Adriano Tonelli5, William Messner6 and Soumya Chatterjee1, 1Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, 2Respiratory Institute, Cleveland Clinic, Cleveland, OH, 3Rheumatology.org, Cleveland Clinic, Cleveland, OH, 4Respiratory Institute - Pulmonary Medicine, Cleveland Clinic, Cleveland, OH, 5Pulmonary Medicine - Respiratory Institute, Cleveland Clinic, Cleveland, OH, 6Quantitative Health Sciences, Cleveland Clinic, Cleveland, OH

    Background/Purpose: Systemic sclerosis (SSc) associated pulmonary arterial hypertension (SSc-PAH) is a serious and often life-threatening complication of SSc. Perhaps the most important enigma in the…
  • Abstract Number: 2681 • 2017 ACR/ARHP Annual Meeting

    Epidemiology and Survival of Systemic Sclerosis-Systemic Lupus Erythematosus Overlap Syndrome

    Samar Alharbi1, Zareen Ahmad2, Zahi Touma3, Arthur Bookman4, Jorge Sánchez-Guerrero5 and Sindhu Johnson6, 1Division of Rheumatology, University of Toronto, Toronto Scleroderma Program, Sinai Health System, University Health Network,, Toronto, ON, Canada, 2Toronto Scleroderma Program, Division of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, 3Rheumatology, University of Toronto, Division of Rheumatology, Institute of Health Policy, Management and Evaluation, Toronto, ON, Canada, 4Division of Rheumatology, University Health Network, University of Toronto, Toronto, ON, Canada, Toronto, ON, Canada, 5Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, Mexico, 6Division of Rheumatology, University of Toronto, Toronto Scleroderma Program, Sinai Health Systems and University Health Network, Toronto, ON, Canada

    Background/Purpose: Systemic sclerosis (SSc) is an immune disorder characterized by vasculopathy and fibrosis. SSc may overlap with another disease such as systemic lupus erythematosus (SLE).…
  • Abstract Number: 2682 • 2017 ACR/ARHP Annual Meeting

    Correlation between Capillaroscopic Patterns of Nailfold Microangiopathy and Three Different Methods to Assess Dermal Thickness in Systemic Sclerosis Patients

    Barbara Ruaro1, Alberto Sulli2, Carmen Pizzorni1, Elisa Alessandri1, Sabrina Paolino1, Massimo Ghio1, Veronica Tomatis3, Vanessa Smith4 and Maurizio Cutolo1, 1Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino, Genoa, Italy, Genoa, Italy, 2Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino, Genoa, Italy, Genova, Italy, 3Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Genoa, Italy, Genoa, Italy, 4Department of Rheumatology, Ghent University Hospital, Faculty of Internal Medicine, Ghent University, Ghent, Belgium, Ghent, Belgium

    Background/Purpose: Systemic sclerosis (SSc) is characterized by increase of dermal thickness (DT) and impairment of microvascular system (1). Several studies demonstrate that nailfold capillaroscopy (NVC)…
  • Abstract Number: 2683 • 2017 ACR/ARHP Annual Meeting

    Factors Associated with Disease Progression in Early-Diagnosed Pulmonary Arterial Hypertension Associated with Systemic Sclerosis

    Carina Mihai1, Milos Antic2, Rucsandra Dobrota3, Diana Bonderman4, Harbajan Chadha-Boreham5, J. Gerry Coghlan6, Christopher Denton7, Martin Doelberg5, Ekkehard Gruenig8, Dinesh Khanna9, Vallerie McLaughlin10, Ulf Müller-Ladner11, Janet E. Pope12, Daniel M Rosenberg5, James R Seibold13, Madelon C Vonk14 and Oliver Distler15, 1Internal Medicine and Rheumatology Dept., Cantacuzino Clinical Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania, 2University Hospital Zurich, Zurich, Switzerland, 3Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland, 4Department of Internal Medicine II, Division of Cardiology, Medical University of Vienna, Vienna, Austria, 5Actelion Pharmaceuticals Ltd., Alschwil, Switzerland, 6National Pulmonary Hypertension Service, Royal Free Hospital, London, United Kingdom, 7Department of Rheumatology, University College London, Royal Free Hospital, London, United Kingdom, 8Centre for Pulmonary Hypertension, Thoraxclinic, University Hospital Heidelberg, Heidelberg, Germany, 9Division of Rheumatology and Clinical Autoimmune Center of Excellence, University of Michigan, Ann Arbor, MI, Ann Arbor, MI, 10Internal Medicine, Division of Cardiology, University of Michigan, Ann Arbor, MI, 11Justus-Liebig-University Giessen, Department of Internal Medicine and Rheumatology, Kerckhoff-Klinik, Bad Nauheim, Germany, Bad-Nauheim, Germany, 12Department of Medicine, Division of Rheumatology, University of Western Ontario, St Joseph's Health Care, London, ON, Canada, 13Scleroderma Research Consultants LLC, Litchfield, CT, 14Rheumatology, Radboud University Medical Centre, Nijmegen, Netherlands, 15Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland

    Background/Purpose: Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis (SSc), and its diagnosis requires right heart catheterization (RHC). The DETECT study developed…
  • Abstract Number: 2684 • 2017 ACR/ARHP Annual Meeting

    Regional and Whole Gut Transit Times in Patients with Systemic Sclerosis Using the Wireless Motility Capsule

    Neetu Bali1, Isela Valera2, Aly Aly3, Jeffrey Conklin4, Daniel E. Furst5 and Suzanne Kafaja6, 1Pediatric Gastroenterology, Hepatology, and Nutrition, David Geffen School of Medicine at UCLA, Los Angeles, CA, 2Autoimmunity and Tolerance Laboratory, Division of Rheumatology, Department of Medicine, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, CA, 3Chamblion St,, Alexandria Faculty of Medicine, Alexandria, Egypt, 4Medicine, Division of Digestive Diseases at UCLA, Los Angeles, CA, Los Angeles, CA, 5David Geffen School of Medicine at UCLA, Los Angeles, CA, 6Department of Internal Medicine, University of California Los Angeles, David Geffen School of Medicine, Division of Rheumatology, Los Angeles, CA

    Background/Purpose: The Gastrointestinal tract (GI) is one of the most commonly affected systems in systemic sclerosis (SSc), impacting the lives of up to 90% of…
  • Abstract Number: 2685 • 2017 ACR/ARHP Annual Meeting

    Sensitivity and Specificity of YKL-40 for the Presence of Pulmonary Arterial Hypertension in Systemic Sclerosis

    Tetsuya Furukawa1, Kiyoshi Matsui2, Masayasu Kitano2, Yuichi Yokoyama3, Naoto Azuma2 and Hajime Sano2, 1Division ofRheumatology Department of internal medicine, Hyogo College of Medicine, Nishinomiya, 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, Japan

    Background/Purpose: Systemic sclerosis (SSc) is an intractable connective tissue disease that causes fibrosis of the skin and organs. The prognosis of this disease is affected…
  • Abstract Number: 2686 • 2017 ACR/ARHP Annual Meeting

    In Established Pulmonary Arterial Hypertension the Follow-up Cardiac Catheterization-Derived  Pulmonary Artery Systolic Pressure and Vascular Resistence May be Predicted By Echocardiography- a Longitudinal Study in Two Connective Tissue Disease Cohorts

    Vasiliki-Kalliopi Bournia1, Iraklis Tsangaris2, Loukianos Rallidis2, Anastasia Anthi2, Stylianos Orfanos2, Eftychia Demerouti3, Panayiotis Karyofyllis3, Vasilis Voudris3, Stylianos Panopoulos4 and Petros P Sfikakis4,5, 1First Department of Propedeutic and Internal Medicine and Joined Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece, 2Pulmonary Hypertension Clinic, National and Kapodistrian University of Athens Medical School, Athens, Greece, 3Cardiology Department, Onassis Cardiac Surgery, Athens, Greece, 4First Department of Propaedeutic and Internal Medicine and Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece, 5First Department of Propaedeutic and Internal Medicine and Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, athens, Greece

    Background/Purpose: Pulmonary arterial hypertension (PAH) associated with connective tissue disease should always be diagnosed with the gold-standard method of right heart catheterization (RHC). On the…
  • Abstract Number: 2687 • 2017 ACR/ARHP Annual Meeting

    Characteristic of Pulmonary Arterial Hypertension in Patients with Anti-U1RNP Antibody-Positive-Connective Tissue Diseases Is Determined By the Underlying Disease Rather Than Autoantibody Profile

    Hidekata Yasuoka1, Hiroshi Takei1, Yuichiro Shirai2, Kunihiro Yamaoka1, Masataka Kuwana1,2 and Tsutomu Takeuchi1, 1Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan, 2Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan

    Background/Purpose: Patients with connective tissue diseases (CTDs) positive for anti-U1RNP antibody (U1) is characterized by overlapping feature of systemic sclerosis (SSc), systemic lupus erythematosus (SLE),…
  • Abstract Number: 2688 • 2017 ACR/ARHP Annual Meeting

    Collagen Metabolite Biomarker Levels Are Associated with the Amount of Fibrosis in Internal Organs in Systemic Sclerosis Patients

    Anne Sofie Siebuhr1, Satoshi Kubo2, Pernille Juhl3, K Nakano4, S Nakayamada5, Morten Karsdal6, Anne-C. Bay-Jensen7 and Yoshiya Tanaka8, 1the first department of internal medicine, University of occupational and environmental health, Fukuoka, Japan, 2The first department of internal medicine, University of Occupational en environmental Health, Fukuoka, Japan, 3Nordic Bioscience, Herlev, Denmark, 4the first department of internal medicin, University of occupational and environmental health, fukuoka, Japan, 5The first department of internal medicine, University of Occupational and environmental Health, fukuoka, Japan, 6Biomarkers and Reseacrh, Nordic Bioscience, Herlev, Denmark, 7Biomarkers and Reseach, Nordic Bioscience, Herlev, Denmark, 8The First Department of Internal Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan

    Background/Purpose: Biomarkers that can monitor the fibrotic burden will aid in trial enrichment and personalized health care in SSc. SSc is associated with internal organ…
  • Abstract Number: 2689 • 2017 ACR/ARHP Annual Meeting

    Troponinemia Independently Associates with Mortality in Systemic Sclerosis

    Julie J. Paik1, Debbie Choi1, Fredrick M. Wigley2, Laura K. Hummers3 and Ami A. Shah1, 1Johns Hopkins University, Baltimore, MD, 2Rheum Div/Mason F Lord, Johns Hopkins University, Baltimore, MD, 3Medical and Rheumatology, Johns Hopkins University, Baltimore, MD

    Title: Troponinemia independently associates with mortality in systemic sclerosis Background/Purpose: Cardiac involvement is common in systemic sclerosis (SSc) and in the early asymptomatic stages, elevated…
  • Abstract Number: 2690 • 2017 ACR/ARHP Annual Meeting

    Plasma D-Dimer Levels Are More Frequently Elevated in Limited Than Diffuse Cutaneous Systemic Sclerosis but Do Not Reflect Disease Duration or Vasculopathy

    Anna Gill1, Svetlana I. Nihtyanova2, Pratima Chowdary3 and Christopher Denton1, 1Division of Medicine, Centre for Rheumatology and Connective Tissue Disease, University College London, London, United Kingdom, 2Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, 3Department of Haematology, Royal Free Hospital, London, United Kingdom

    Background/Purpose: D-dimers are degradation products of cross linked fibrin and are biomarkers of activation of the coagulation system. Plasma D-dimer levels can be raised in…
  • Abstract Number: 2691 • 2017 ACR/ARHP Annual Meeting

    Increased Circulating Cadherin-11 Levels in Patients with Systemic Sclerosis

    Bochra Jandali1, Grace H. Lo2, Robert L. Welschhans3, Julio Charles4, Ramona Mihu3,5, Maureen D. Mayes6, Shervin Assassi7 and Sandeep K. Agarwal3, 1Medicine, Section of Immmunology, Allergy and Rheumatology, Baylor College of Medicine, Houston, TX, 2Immunology, Allergy, Rheumatology, Baylor College of Medicine, Houston, TX, 3Medicine, Section of Immunology, Allergy and Rheumatology, Baylor College of Medicine, Houston, TX, 4Internal Medicine/Rheumatology, University of Texas - Houston Medical School, Houston, TX, 5Medicine, Division of Rheumatology, Houston Methodist Hospital, Houston, TX, 6Department of Internal Medicine - Rheumatology, University of Texas-McGovern Medical School, Houston, TX, 7University of Texas McGovern Medical School, Houston, TX

    Background/Purpose: Systemic sclerosis (SSc) is a chronic systemic disease characterized by skin and internal organs fibrosis along with vasculopathy. Previous studies have reported increased cadherin-11…
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