Abstracts tagged "pulmonary complications and systemic sclerosis"

Abstract Number: 764 • 2014 ACR/ARHP Annual Meeting
ERG and FLI1 in Systemic Sclerosis-Associated Pulmonary Complications
Rong Han and Maria Trojanowska, Arthritis Center, Boston University, Boston, MA
Background/Purpose: Pulmonary arterial hypertension (PAH) and pulmonary fibrosis (PF) are the two major lung complications associated with the autoimmune disease systemic sclerosis (SSc), and constitute…
Abstract Number: 2578 • 2013 ACR/ARHP Annual Meeting
Pulmonary Hypertension In Systemic Sclerosis: Clinical Classification and Pulmonary Hypertension Subtypes
Monica Mohile¹, Mary Lucas², Virginia D. Steen³, Thomas A. Medsger Jr.⁴ and Robyn T. Domsic⁵, ¹University of Pittsburgh Medical Center, Pittsburgh, PA, ²Division of Rheumatology, University of Pittsburgh, Pittsburgh, PA, ³Department of Rheumatology, Georgetown University Medical Center, Washington, DC, ⁴Medicine/Rheumatology, University of Pittsburgh, Pittsburgh, PA, ⁵Medicine - Rheumatology, University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Pumonary hypertension (PH) is a significant complication of systemic sclerosis (SSc), with prevalence reports of 10-25%. Predictors of PH remain somewhat elusive. Our…
Abstract Number: 2581 • 2013 ACR/ARHP Annual Meeting
Can Changes In NT-Probnp Predict Early Response To Therapy and Prognosis In Systemic Sclerosis Associated Pre-Capillary Pulmonary Hypertension?
Vincent Sobanski¹, Bernadette Lynch², Benjamin E. Schreiber³, Clive Handler⁴, Christopher P. Denton⁵ and John G. Coghlan⁶, ¹Department of Rheumatology, Royal Free Hospital, London, United Kingdom, ²Department of Rheumatology, The Royal Free Hospital, London, United Kingdom, ³Royal Free Hospital NHS Foundation Trust, National Pulmonary Hypertension Service, London, United Kingdom, ⁴Department of Pulmonary Hypertension, The Royal Free Hospital, London, United Kingdom, ⁵Centre for Rheumatology, Royal Free and University College Medical School, London, United Kingdom, ⁶National Pulmonary Hypertension Service, The Royal Free Hospital NHS Foundation Trust, London, United Kingdom
Background/Purpose: Pulmonary hypertension (PH) is a severe complication of systemic sclerosis (SSc), affecting 5-12% of patients. Despite recent progress in treatment, prognosis remains poor. Early…
Abstract Number: 2584 • 2013 ACR/ARHP Annual Meeting
Screening and Diagnostic Modalities For Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: A Systematic Review
Heather Gladue¹, Nezam I. Altorok², Whitney Townsend¹, Vallerie McLaughlin³ and Dinesh Khanna⁴, ¹University of Michigan, Ann Arbor, MI, ²Internal Medicine and Rheumatology, University of Michigan, Ann Arbor, MI, ³Internal Medicine, Division of Cardiology, University of Michigan, Ann Arbor, MI, ⁴Division of Rheumatology, University of Michigan Medical Center, Ann Arbor, MI
Background/Purpose: Pulmonary arterial hypertension (PAH) affects patients with connective tissue diseases (CTD), especially systemic sclerosis (SSc) and MCTD. Despite this, there continues to be delay…
Abstract Number: 695 • 2013 ACR/ARHP Annual Meeting
Combined Pulmonary Fibrosis and Emphysema (CPFE) In Systemic Sclerosis
Nicolas Champtiaux¹, Vincent Cottin², Eric Hachulla³, Dominique Valeyre⁴, Hilario Nunes⁵, David Launay⁶, Alice Berezne⁷, Bruno Crestani⁸, Loic Guillevin⁹, Jean-Francois Cordier² and Luc Mouthon¹⁰, ¹Service de Médecine Interne, hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, ²Division of Pneumology, Hôpital Louis-Pradel, Hospices Civils de Lyon, Lyon 1, Lyon, France, ³Internal Medicine, Lille CEDEX, France, ⁴Department of Pneumology, Avicenne hospital (APHP), Bobigny, France, ⁵Department of Pneumology, Avicenne Hospital (AP-HP), Bobigny, France, ⁶Internal Medicine, Claude Huriez University Hospital, Lille, France, ⁷Paris Descartes University, Internal Medicine department, Cochin Hospital, Paris, France, ⁸Pneumology A, Hôpital Bichat, Paris, France, ⁹Internal Medicine, Cochin University Hospital, Paris, France, ¹⁰Department of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, Paris, France
Combined pulmonary fibrosis and emphysema (CPFE) in systemic sclerosis Background/Purpose: Combined pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome, in which centrilobular and/or…
Abstract Number: 1465 • 2012 ACR/ARHP Annual Meeting
World Health Organization Classification of Pulmonary Hypertension and Survival in Systemic Sclerosis Patients in the Pharos Cohort
Jessica K. Gordon¹, Lorinda Chung², Robyn T. Domsic³, Wei-Ti Huang⁴, Stephen L. Lyman⁵, Evelyn M. Horn⁶, Virginia D. Steen⁷ and PHAROS Investigators⁸, ¹Rheumatology, Hospital for Special Surgery, New York, NY, ²Rheumatology, Stanford Univ Medical Center, Palo Alto, CA, ³Medicine - Rheumatology, University of Pittsburgh, Pittsburgh, PA, ⁴Epidemiology and Statistics, Hospital for Special Surgery, New York, NY, ⁵Research, Hospital for Special Surgery, New York, NY, ⁶Cardiology, New York Presbyterian Hospital/Weill Cornell Medical College, New York, NY, ⁷Department of Rheumatology, Georgetown University Medical Center, Washington, DC, ⁸Washington, DC, DC
Background/Purpose: Pulmonary hypertension (PH) is a leading cause of death in patients (pts) with Systemic Sclerosis (SSc). The World Health Organization (WHO) classifies PH…
Abstract Number: 1466 • 2012 ACR/ARHP Annual Meeting
Patients with Systemic Sclerosis Are Dying of Non-Systemic Sclerosis Related Causes, However Interstitial Lung Disease Remains the Predominant Systemic Sclerosis Related Cause of Death
Rebecca L. Batten and Bridget Griffiths, Rheumatology, Freeman Hospital, Newcastle Upon Tyne, United Kingdom
Interstitial Lung Disease Remains the leading Cause of Death in Patients with Systemic Sclerosis, Despite Aggressive Treatment with CyclophosphamideBackground/Purpose: Studies suggest that despite aggressive treatment…
Abstract Number: 719 • 2012 ACR/ARHP Annual Meeting
Biomarkers of Pulmonary Hypertension in Patients with Scleroderma: A Case-Control Study
Zsuzsanna H. McMahan¹, Florian Schoenhoff², Jennifer van Eyk³, Fredrick M. Wigley⁴ and Laura K. Hummers⁴, ¹Rheumatology, Johns Hopkins University, Baltimore, MD, ²Department of Cardiovascular Surgery, Berne, Switzerland, ³Johns Hopkins University and Cedars Sinai Medical Center, Los Angeles, CA, ⁴Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD
Background/Purpose: The objective of this study was to evaluate for an association between various biomarkers and the presence or absence of pulmonary hypertension (PH) in…
Abstract Number: 724 • 2012 ACR/ARHP Annual Meeting
Survival, Hospitalization or Need for Combination Therapy At One Year in Patients with Scleroderma-Associated Pulmonary Arterial Hypertension
Robyn T. Domsic¹, Lorinda Chung², Jessica K. Gordon³, Yona Cloonan⁴, Virginia D. Steen⁵ and PHAROS Investigators⁶, ¹Medicine - Rheumatology, University of Pittsburgh, Pittsburgh, PA, ²Rheumatology, Stanford Univ Medical Center, Palo Alto, CA, ³Rheumatology, Hospital for Special Surgery, New York, NY, ⁴Graduate School of Public Health, University of Pittsburgh, Pittsburgh, PA, ⁵Department of Rheumatology, Georgetown University Medical Center, Washington, DC, ⁶Washington, DC, DC
Background/Purpose: Pulmonary arterial hypertension (PAH) is a leading cause of death in patients with systemic sclerosis (SSc). Although survival has improved with PAH-specific medications in…
Abstract Number: 2304 • 2012 ACR/ARHP Annual Meeting
Differential Association of IRAK1 and MECP2 with Specific Systemic Sclerosis Phenotypes
F. David Carmona¹, M.C. Cénit¹, L.M. Díaz-Gallo¹, Carmen P. Simeón², Patricia Carreira³, the Spanish Scleroderma Group⁴, Nicolas Hunzelmann⁵, Gabriela Riemekasten⁶, Torsten Witte⁷, Alexander Kreuter⁸, Jörg HW Distler⁹, Paul Shiels¹⁰, Jacob M. van Laar¹¹, Annemie Schuerwegh¹², Madelon C. Vonk¹³, Alexandre Voskuyl¹⁴, Carmen Fonseca¹⁵, Christopher Denton¹⁶, Ariane Herrick¹⁷, Frank C. Arnett¹⁸, Filemon K. Tan¹⁸, Shervin Assassi¹⁸, T.R.D.J. Radstake¹⁹, Maureen D. Mayes¹⁸ and Javier Martin¹, ¹Instituto de Parasitología y Biomedicina López-Neyra, Consejo Superior de Investigaciones Científicas, Armilla (Granada), Spain, ²Department of Internal Medicine, Hospital Valle de Hebron, Barcelona, Spain, ³Rheumatology, Hospital Universitario 12 de Octubre, Madrid, Spain, ⁴Granada, Spain, ⁵Department of Dermatology, University of Cologne, Cologne, Germany, ⁶Charité University Hospital and German Rheumatism Research Centre, a Leibniz Institute, Berlin, Germany, ⁷Clinical Immunology and Rheumatology, Medical University Hannover, Hanover, Germany, ⁸Department of Dermatology, Venereology, and Allergologie, HELIOS St. Elisabeth Hospital, Oberhausen, Germany, ⁹Department of Internal Medicine, Institute for Clinical Immunology, University of Erlangen-Nuremberg, Erlangen, Germany, ¹⁰University of Glasgow, Glasgow, United Kingdom, ¹¹Newcastle University, Musculoskeletal Research Group, Newcastle, United Kingdom, ¹²Leids Univ Medisch Centrum, Leiden, Netherlands, ¹³Department of Rheumatology, Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands, ¹⁴Department of Rheumatology, VU University Medical Center, Amsterdam, Netherlands, ¹⁵Department of Rheumatology, Royal Free Hospital, London, United Kingdom, ¹⁶Department of Rheumatology, Royal Free and University College Medical School, London, United Kingdom, ¹⁷Musculoskeletal Research Group, University of Manchester, Salford, United Kingdom, ¹⁸Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, ¹⁹Department of Rheumatology & Clinical Immunology, University Medical Center Utrecht, Utrecht, Netherlands
Background/Purpose: Systemic sclerosis (SSc) is a fibrotic autoimmune disease that represents a clear example of a sex biased immune disorder. The X-chromosome gene IRAK1 has…
Abstract Number: 1458 • 2012 ACR/ARHP Annual Meeting
Left Ventricular Diastolic Dysfunction May Play a Role in Pathophysiology and Poor Prognosis of Pulmonary Arterial Hypertension Associated with Systemic Sclerosis
Sumiaki Tanaka¹, Eisuke Ogawa¹, Tatsuhiko Wada¹, Tatsuo Nagai¹, Jun Okada² and Shunsei Hirohata¹, ¹Int Med/Rheumatol & Infec Dis, Kitasato University School of Medicine, Sagamihara, Japan, ²Nutritional management, Kitasato Junior Collage of health and Hygienic Sciences, Minami-Uonuma, Japan
Background/Purpose: Cardio-pulmonary involvements of systemic sclerosis (SSc), including cardiomyopathy, interstitial lung disease, and pulmonary arterial hypertension (PAH) are leading causes of SSc-related deaths. Several potent…
Abstract Number: 1462 • 2012 ACR/ARHP Annual Meeting
Expert Consensus for Performing Right Heart Catheterization in Suspicion of Pulmonary Arterial Hypertension Associated with Systemic Sclerosis: A Delphi Consensus Study with Cluster Analysis From the Eposs Group
Jerome Avouac¹, Dörte Huscher², Daniel Furst³, Oliver Distler⁴ and Yannick Allanore⁵, ¹Paris Descartes University, Rheumatology A department, Cochin Hospital, Paris, France, ²German Rheumatism Research Centre and Charité University Medicine, Berlin, Germany, ³Div of Rheumatology, UCLA Medical School, Los Angeles, CA, ⁴Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland, ⁵Rheumatology, Paris Descartes University, Rheumatology A department, Cochin Hospital, Paris, France
Background/Purpose: Pulmonary hypertension (PH) has emerged as a critical cause of death in systemic sclerosis (SSc). Recent data have highlighted the poorer outcomes of SSc…
Abstract Number: 1463 • 2012 ACR/ARHP Annual Meeting
Pulmonary Hypertension and Interstitial Lung Disease within Pharos: Impact of Extent of Fibrosis and Pulmonary Physiology On Cardiac Hemodynamic Parameters
Aryeh Fischer¹, Stephen C. Mathai², Marcy B. Bolster³, Lorinda Chung⁴, Mary Ellen Csuka⁵, Robyn T. Domsic⁶, Tracy M. Frech⁷, Monique E. Hinchcliff⁸, Vivien M. Hsu⁹, Laura K. Hummers¹⁰, Jason R. Kolfenbach¹¹, Mardi Gomberg-Maitland¹², Aida Manu¹³, Robert W. Simms¹⁴ and Virginia D. Steen¹⁵, ¹Rheumatology / ILD Program, National Jewish Health, Denver, CO, ²Medicine, Johns Hopkins University, Baltimore, MD, ³Medicine, Medical Univ of South Carolina, Charleston, SC, ⁴Rheumatology, Stanford Univ Medical Center, Palo Alto, CA, ⁵Rheumatology, Medical College of Wisconsin, Milwaukee, WI, ⁶Medicine - Rheumatology, University of Pittsburgh, Pittsburgh, PA, ⁷Internal Medicine-Division of Rheumatology, University of Utah School of Medicine, SLC, UT, ⁸Division of Rheumatology, Northwestern Univ Med School, Chicago, IL, ⁹Rheumatology, RWJ Med Schl Scleroderma Prog, New Brunswick, NJ, ¹⁰Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, ¹¹Division of Rheumatology, University of Colorado School of Medicine, Aurora, CO, ¹²University of Chicago, Chicago, ¹³Rheumatology, Georgetown University Medical Center, Disrict of Columbia, ¹⁴Rheumatology, Boston University School of Medicine, Boston, MA, ¹⁵Department of Rheumatology, Georgetown University Medical Center, Washington, DC
Background/Purpose: Pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) are the leading causes of mortality in systemic sclerosis (SSc). Many SSc patients have both…

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