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: 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: 735 • 2017 ACR/ARHP Annual Meeting
Cardiopulmonary Exercise Testing to “Detect” Pulmonary Arterial Hypertension in Systemic Sclerosis
Rosa Casella¹, Alessandro Santaniello^2,3, 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: 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 Richardson^3,4, 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: 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-Vold^1,2, 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: 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: 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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