ACR Meeting Abstracts

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Abstracts tagged "pulmonary complications and systemic sclerosis"

  • Abstract Number: 809 • 2018 ACR/ARHP Annual Meeting

    The Lymphangiogenetic Factors VEGF-C, CCL21 and Ang-2 Are Associated with Pulmonary Arterial Hypertension in Systemic Sclerosis

    Henriette Didriksen1, Håvard Fretheim1, Vyacheslav Palchevskiy2, Arne K Andreassen1, Torhild Garen1, Oyvind Midtvedt1, Einar Gude1, John A Belperio3, Øyvind Molberg1 and Anna-Maria Hoffmann-Vold1, 1Oslo University Hospital, Oslo, Norway, 2University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, 3University of California, Los Angeles, Los Angeles, CA

    Background/Purpose: Systemic sclerosis (SSc) is characterized by abnormalities in vascular pathways and lymphatic vessels with pulmonary hypertension as a major complication. Vascular endothelial growth factor…
  • Abstract Number: 863 • 2018 ACR/ARHP Annual Meeting

    Combining Cardiac Magnetic Resonance and Right Heart Catheterization to Evaluate Right Ventricular Function for the Prognosis Prediction in Patients with Connective Tissue Diseases and Pulmonary Hypertension

    Nobuya Abe1, Masaru Kato2, Hiroyuki Nakamura2, Atsushi Noguchi2, Yuichiro Fujieda2, Kenji Oku2, Toshiyuki Bohgaki2, Olga Amengual2, Shinsuke Yasuda2 and Tatsuya Atsumi2, 1Department of Rheumatology, Endocrinology and Nephrology, Graduate School of Medicine, Hokkaido University, Sapporo, Japan, 2Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan

    Background/Purpose: Pulmonary arterial hypertension associated with connective tissue diseases (CTD-PAH), particularly PAH associated with systemic sclerosis (SSc-PAH), has a poor prognosis compared with other PAH.…
  • 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: 735 • 2017 ACR/ARHP Annual Meeting

    Cardiopulmonary Exercise Testing to “Detect” Pulmonary Arterial Hypertension in Systemic Sclerosis

    Rosa Casella1, Alessandro Santaniello2,3, Marco Vicenzi1 and Lorenzo Beretta2, 1Cardiovascular Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy, 2Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy, 3VIA FRANCESCO SFORZA 28, OSPEDALE MAGGIORE POLICLINICO, MILANO, Italy

    Background/Purpose: Pulmonary arterial hypertension (PAH) is one of the leading death causes in SSc patients. To gain a better prognosis, screening methods are needed to…
  • Abstract Number: 743 • 2017 ACR/ARHP Annual Meeting

    Impact of Detect on Right Heart Catheterization Referral and Results; Data from a Prospective, Unselected, Systemic Sclerosis Cohort

    Anna-Maria Hoffmann-Vold1, Håvard Fretheim2, Anders Heiervang Tennøe2, Oyvind Midtvedt2, Torhild Garen2, Einar Gude2, Arne K Andreassen2 and Øyvind Molberg2, 1Division of Rheumatology, Oslo University Hospital, Oslo, Norway, 2Oslo University Hospital, Oslo, Norway

    Background/Purpose: The DETECT calculator has been freely available as a tool for earlier detection and diagnosis of pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc)…
  • Abstract Number: 758 • 2017 ACR/ARHP Annual Meeting

    Identification of Biomarkers Predictive of Pulmonary Arterial Hypertension in Systemic Sclerosis

    Kathleen D. Kolstad1, Tyson Holmes2, Yael Rosenberg-Hasson2, Andrew Sweatt3, Roham T. Zamanian4, Shufeng Li5, Virginia D. Steen6, Paul J. Utz7 and Lorinda Chung8, 1Rheumatology, Stanford University Medical Center, Stanford, CA, 2Stanford University Medical Center, Stanford, CA, 3Medicine, Division of Pulmonary and Critical Care, Stanford University Medical Center, Stanford, CA, 4Stanford University Medical Center, Palo Alto, CA, 5Dermatology, Stanford University School of Medicine, Stanford, CA, 6Rheumatology, MedStar Georgetown University Hospital, Washington, DC, 7Medicine, Stanford University School of Medicine, Stanford, CA, 8Rheumatology, Stanford University Medical Center, Palo Alto, CA

    Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular abnormalities, immune system dysregulation, and fibrosis. Pulmonary arterial hypertension (PAH) affects approximately 10% of…
  • Abstract Number: 1700 • 2017 ACR/ARHP Annual Meeting

    Focusing on Pulmonary Vascular Disease at Early Stage of Systemic Sclerosis: Exercise-Induced Pulmonary Arterial Hypertension and Gene Co-Expression Networks Involved in Its Pathogenesis

    Yoshinobu Koyama1, Soichiro Fuke2, Yoshiharu Sato3 and Toshie Higuchi1, 1Center for Autoimmune Diseases, Division of Rheumatology, Japan Red Cross Okayama Hospital, Okayama, Japan, 2Department of Cardiology, Japan Red Cross Okayama Hospital, Okayama, Japan, 3DNA Chip Research Inc, Yokohama, Japan

    Background/Purpose: Pulmonary arterial hypertension (PAH) is prominent as a vascular involvement in systemic sclerosis (SSc), which remains a leading cause of death in spite of…
  • 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: 835 • 2016 ACR/ARHP Annual Meeting

    A Normal Pulmonary Diffusion Capacity Is Rare in Pulmonary Artery Hypertension in Systemic Sclerosis

    Rebecca Overbury1, Tracy M. Frech2, Maureen Murtaugh3, Virginia D. Steen4 and PHAROS investigators, 1Internal Medicine and Pediatrics, University of Utah, Salt Lake City, UT, 2Division of Rheumatology, University of Utah, Salt Lake City, UT, 3University of Utah, Salt Lake, UT, 4Rheumatology, Georgetown University Medical Center, Washington, DC

    Background/Purpose:   Methods:   Results:  > 70% (mean 78%) versus 191 patients (94.6%) who had a low DLCO <70% (mean 39%). There was no difference…
  • Abstract Number: 1884 • 2016 ACR/ARHP Annual Meeting

    Esophageal Dilation and Interstitial Lung Disease Incidence and Progression in Systemic Sclerosis

    Kimberly Showalter1, Aileen Hoffmann2, Carrie Richardson3,4, Julia (Jungwha) Lee5, David Aaby6, Rishi Agrawal7, Jane Dematte8, Rowland W. Chang9 and Monique Hinchcliff10, 1Internal Medicine, McGaw Medical Center of Northwestern University, Chicago, IL, 2Department of Medicine, Division of Rheumatology, Northwestern University Feinberg School of Medicine, Northwestern University, Chicago, IL, 3Department of Medicine, McGaw Medical Center of Northwestern University, Chicago, IL, 4Department of Rheumatology, Johns Hopkins University, Baltimore, MD, 5Preventive Medicine/Biostatistics, Northwestern University Feinberg School of Medicine, Chicago, IL, 6Northwestern University Feinberg School of Medicine, Chicago, IL, 7Radiology, Northwestern University Feinberg School of Medicine, Chicago, IL, 8Pulmonology, Northwestern University Feinberg School of Medicine, Chicago, IL, 9Institute for Public Health and Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, 10Northwestern University, Feinberg School of Medicine Scleroderma Program, Chicago, IL

    Background/Purpose: Interstitial lung disease (ILD) is the leading cause of death in systemic sclerosis (SSc). Esophageal dysfunction and aspiration may play a role in SSc-ILD.…
  • Abstract Number: 3246 • 2016 ACR/ARHP Annual Meeting

    Predictors of Long-Term Outcomes in Systemic Sclerosis Associated Pulmonary Arterial Hypertension from the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry

    Kathleen Kolstad1, Shufeng Li2, Virginia D. Steen3 and Lorinda Chung4, 1Rheumatology, Stanford Univeristy, Stanford, CA, 2Dermatology, Stanford University, Stanford, CA, 3Rheumatology, Georgetown University Medical Center, Washington, DC, 4Rheumatology, Stanford University Medical Center, Palo Alto, CA

    Background/Purpose:  Pulmonary arterial hypertension (PAH) is a leading cause of death in patients with systemic sclerosis (SSc). Predictors of short-term mortality include male sex, age>60…
  • Abstract Number: 3139 • 2015 ACR/ARHP Annual Meeting

    Screening Algorithm for Pulmonary Hypertension in Systemic Sclerosis – Comparison of Predictive Accuracy of Three Algorithms

    Vivek Nagaraja1, Scott H. Visovatti2, Heather Gladue3, Veronica J. Berrocal4, Jennifer Serrano5, Vallerie McLaughlin2 and Dinesh Khanna6, 1Department of Medicine [Division of Rheumatology], University of Toledo, Toledo, OH, 2Internal Medicine, Division of Cardiology, University of Michigan, Ann Arbor, MI, 3Rheumatology, Emory University, Atlanta, GA, 4Div of Rheumatology, University of Michigan, Ann Arbor, MI, 5University of Michigan, Ann Arbor, MI, 6Division of Rheumatology, University of Michigan, Ann Arbor, MI

    Background/Purpose: Pulmonary arterial hypertension (PAH) is the leading cause of mortality in systemic sclerosis (SSc), and is associated with a 3-year survival of approximately 50%.…
  • Abstract Number: 2990 • 2015 ACR/ARHP Annual Meeting

    Assessment of NT-Pro BNP As a Potential Marker for Pulmonary Hypertension in Systemic Sclerosis: Data from a Large, Prospective and Unselected Patient Cohort

    Anna-Maria Hoffmann-Vold1,2, Oyvind Midtvedt1, Torhild Garen3, May Brit Lund4, Arne Andreassen5 and Øyvind Molberg6, 1Rheumatology, Oslo University Hospital, Oslo, Norway, 2Institute of clinical medicine, University of Oslo, Oslo, Norway, 3Rheumatology, Department of Rheumatology, Oslo University Hospital, Oslo, Norway, 4Respiratory Diseases, Oslo University Hospital, Oslo, Norway, 5Cardiology, Oslo University Hospital, Oslo, Norway, 6Department of Rheumatology, Institute of Clinical Medicine, Oslo University Hospital, Oslo, Norway

    Background/Purpose: In Systemic Sclerosis (SSc), pulmonary arterial hypertension (PAH) is often diagnosed at an advanced stage. Serum levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) are…
  • Abstract Number: 1720 • 2014 ACR/ARHP Annual Meeting

    GATA6 Deficiency Activates UPR Pathways in Endothelial Cells during the Development of Pulmonary Arterial Hypertension

    Rong Han1, Rosanne Van Deuren2, Stefania Lenna1, Izabela Chrobak1, Timothy Radstake3, Carol Feghali-Bostwick4 and Maria Trojanowska1, 1Arthritis Center, Boston University, Boston, MA, 2Rheumatology Department, University Medical Center Nijmegen, Nijmegen, Netherlands, 3University Medical Center Utrecht, Utrecht, Netherlands, 4Medical University of South Carolina, Charleston, SC

    Background/Purpose: Pulmonary arterial hypertension (PAH) is a severe lung complication of systemic sclerosis (SSc), and accounts for a large proportion of SSc-related deaths. As a…
  • Abstract Number: 1261 • 2014 ACR/ARHP Annual Meeting

    Pulmonary Arterial Hypertension in Patients with Anti-PM-Scl Antibody

    Hiromichi Tamaki1, Colin O'Rourke2 and Soumya Chatterjee3, 1Rheumatology, Cleveland Clinic, Cleveland, OH, 2Quantitative Health Sciences, Cleveland Clinic, Cleveland, OH, 3Rheumatic and Immunologic Ds, Cleveland Clinic, Cleveland, OH

    Background/Purpose: Patients with anti-PM-Scl antibody (PM-Scl) can present with several different phenotypes: polymyositis (PM), dermatomyositis (DM), systemic sclerosis (SSc), scleromyositis, or sclero-dermatomyositis. Pulmonary arterial hypertension…
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