Abstracts tagged "Scleroderma, Systemic"

Abstract Number: 1523 • ACR Convergence 2020
Pulmonary Cytokine, Chemokine and Growth Factor Profiles of Distinct Radiographic Patterns of Interstitial Lung Disease in Systemic Sclerosis
Elizabeth Volkmann¹, Donald Tashkin², Ning Li², Mei Leng², Grace Kim², Jonathan Goldin², Airi Harui² and Michael Roth², ¹University of California, Los Angeles, Los Angeles, CA, ²University of California, Los Angeles, Los Angeles
Background/Purpose: The radiological hallmarks of systemic sclerosis-related interstitial lung disease (SSc-ILD) include interstitial inflammation (ground glass opacity) with reticular changes (fibrosis). The precise pathobiology of…
Abstract Number: 0931 • ACR Convergence 2020
Oropharyngeal Dysfunction in Patients with Systemic Sclerosis – Results of a Monocentric Clinical Study
Philipp Klemm¹, Mirjam Wirths², Ole Hudowenz², Ulrike Hoffmann³, Ulf Müller-Ladner² and Uwe Lange², ¹Department of Rheumatology, Immunology, Osteology and Physical Medicine, Justus Liebig University Gießen, Campus Kerckhoff, Bad Nauheim, Germany, Bad Nauheim, Hessen, Germany, ²Department of Rheumatology, Immunology, Osteology and Physical Medicine, Justus Liebig University Gießen, Campus Kerckhoff, Bad Nauheim, Germany, Bad Nauheim, Germany, ³Practice for Speech Therapy Ulrike Hoffmann, Friedberg, Germany
Background/Purpose: Nearly 98% of patients with systemic sclerosis (SSc) are suffering from gastrointestinal involvement (Schmeiser et al. 2012). While in everyday clinical practice screening for…
Abstract Number: 1909 • ACR Convergence 2020
High-density Proteomic Analysis of Skin Blister Fluid and Plasma in Systemic Sclerosis Identifies Local and Systemic Differences for Key Proteins
Kristina Clark¹, Corrado Campochiaro², Eszter Csomor³, Adam Taylor³, Katherine Nevin³, Mary Morse³, Voon Ong⁴, Emma Derrett-Smith⁵, Nicolas Wisniacki³, Shaun Flint³ and Christopher Denton¹, ¹University College London, London, United Kingdom, ²Royal Free Hospital NHS trust, London, United Kingdom, ³GlaxoSmithKline, Stevenage, United Kingdom, ⁴UCL, London, United Kingdom, ⁵University College London Division of Medicine, London, United Kingdom
Background/Purpose: Simultaneous analysis of multiple proteins in biological fluids offers insight into the pathogenesis of SSc. Here, we report a proteomic analysis of plasma and…
Abstract Number: 0933 • ACR Convergence 2020
Advanced Multiparametric Magnetic Resonance Imaging of Muscle for Detection and Quantification of Muscle Disease in Systemic Sclerosis
Julie Paik¹, Fredrick Wigley², Laura Fayad³, Laura Hummers⁴ and Michael Jacobs¹, ¹Johns Hopkins University, Baltimore, MD, ²Johns Hopkins University School of Medicine, Baltimore, MD, ³Joh, Baltimore, MD, ⁴Johns Hopkins Univerisity, Ellicott City, MD
Background/Purpose: Skeletal myopathy in systemic sclerosis (SSc) is underappreciated yet an important manifestation of SSc. While it has been reported that there are distinct histopathologic…
Abstract Number: 1910 • ACR Convergence 2020
Integrated Molecular Analysis of Systemic Sclerosis Skin and Blood Highlights Significant Differences Between Major Autoantibody Subgroups
Kristina Clark¹, Corrado Campochiaro², Eszter Csomor³, Adam Taylor³, Katherine Nevin³, Nicholas Galwey³, Mary Morse³, Voon Ong⁴, Emma Derrett-Smith⁵, Shaun Flint³ and Christopher Denton¹, ¹University College London, London, United Kingdom, ²Royal Free Hospital NHS trust, London, United Kingdom, ³GlaxoSmithKline, Stevenage, United Kingdom, ⁴UCL, London, United Kingdom, ⁵University College London Division of Medicine, London, United Kingdom
Background/Purpose: The major antinuclear autoantibodies of systemic sclerosis (SSc) associate with different skin score trajectories and risk of internal organ manifestations. To elucidate molecular differences…
Abstract Number: 1052 • ACR Convergence 2020
Connective Tissue Disease-Related Interstitial Lung Disease in American Indian/Alaska Native People in Alaska
Joanna Marco¹ and Elizabeth Ferucci², ¹University of Washington, Seattle, WA, ²Alaska Native Tribal Health Consortium, Anchorage, AK
Background/Purpose: Many connective tissue diseases are known to cause interstitial lung disease (ILD). American Indian/Alaska Native (AI/AN) populations have higher prevalence and severity of a…
Abstract Number: 2034 • ACR Convergence 2020
Resolving Phenotypic and Prognostic Differences in Interstitial Lung Disease Related to Systemic Sclerosis by Computed Tomography-based Radiomics
Janine Schniering¹, Malgorzata Maciukiewicz¹, Hubert Gabrys², Matthias Brunner¹, Christian Blütghen³, Chantal Meier¹, Sophie Braga-Lagache⁴, Anne-Christine Ulgry⁴, Manfred Heller⁴, Oliver Distler¹, Matthias Guckenberger², Havard Fretheim⁵, Anna-Maria Hoffmann-Vold⁵, Christos Nakas⁶, Thomas Frauenfelder³, Stephanie Tanadini-Lang² and Britta Maurer⁷, ¹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, Switzerland, Zurich, Switzerland, ²Department of Radiation Oncology, University Hospital Zurich, Switzerland, Zurich, Switzerland, ³Institute of Diagnostic and Interventional Radiology, University Hospital Zurich, Switzerland, Zurich, Switzerland, ⁴Proteomics and Mass Spectrometry Core Facility, Department for BioMedical Research (DBMR), University of Bern, Switzerland, Bern, Switzerland, ⁵Department of Rheumatology, Oslo University Hospital, and Institute of Clinical Medicine, University of Oslo, Norway, Oslo, Norway, ⁶Laboratory of Biometry, University of Thessaly, Volos, Greece, Volos, Greece, ⁷Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, Switzerland, Zurich
Background/Purpose: Radiomics describes the in-depth analysis of tissue phenotypes by computational retrieval of high-dimensional quantitative imaging features including tissue intensity, texture, and wavelet characteristics. Here,…
Abstract Number: 1069 • ACR Convergence 2020
Scleromyositis Is Associated with Nailfold Capillary Abnormalities Compared to Immune-Mediated Necrotizing Myopathy
Océane Landon-Cardinal¹, Valérie Leclair², Yves Troyanov³, Martial Koenig⁴, Alain Meyer⁵, Sabrina Hoa¹, Josiane Bourré-Tessier¹, Isabelle Ferdinand⁶, Jason Karamchandani⁷, Erin O'Ferrall⁸, Minoru Satoh⁹, Marvin Fritzler¹⁰, Benjamin Ellezam¹¹, Jean-Luc Senécal¹, Marie Hudson² and Geneviève Gyger², ¹Division of Rheumatology, Centre hospitalier de l'Université de Montréal, Montréal, QC, Canada, ²Division of Rheumatology, Jewish General Hospital, Montréal, QC, Canada, ³Division of Rheumatology, Hôpital du Sacré-Coeur, Montréal, QC, Canada, ⁴Division of Internal Medicine, Centre hospitalier de l'Université de Montréal, Montréal, QC, Canada, ⁵Service de rhumatologie et Centre de références des maladies autoimmunes rares, Hôpitaux universitaires de Strasbourg, Strasbourg, Alsace, France, ⁶Institut de Rhumatologie de Montréal, Montréal, QC, Canada, ⁷Department of Pathology, Montreal Neurological Institute and Hospital, Montreal, QC, Canada, ⁸Department of Neurology and Neurosurgery, Montreal Neurological Institute and Hospital, Montreal, QC, Canada, ⁹Department of Clinical Nursing, University of Occupational and Environmental Health, Kitakyushu, Japan, ¹⁰Department of Medicine, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada, ¹¹Division of Pathology, Centre hospitalier universitaire Sainte-Justine, Montréal, QC, Canada
Background/Purpose: Scleromyositis (SM) is an emerging subgroup of autoimmune myositis associated with features of systemic sclerosis (SSc) and characterized by prominent vasculopathic features on ultrastructural…
Abstract Number: 2037 • ACR Convergence 2020
Geographic Distribution and Environmental Triggers of Systemic Sclerosis in Massachusetts
Anastasiya Muntyanu¹, Bina Kassamali², Ruth Ann Vleugels³ and Avery LaChance³, ¹McGill University, Montreal, QC, Canada, ²Harvard Medical School, Brigham and Women's Hospital, Boston, MA, ³Brigham and Women's Hospital, Boston, MA
Background/Purpose: Systemic sclerosis (SSc) is a chronic autoimmune sclerosing disease with a 10-year survival rate of less than 65%. This rate has remained unchanged for…
Abstract Number: 1171 • ACR Convergence 2020
Under Detection of Interstitial Lung Disease in Juvenile Systemic Sclerosis (jSSc) Utilizing Pulmonary Function Tests. Results from the Juvenile Scleroderma Inception Cohort
Ivan Foeldvari¹, Bernd Hinrichs², Kathryn Torok³, Maria Jose Santos⁴, Ozgur Kasapcopur⁵, Amra Adrovic⁶, Valda Stanevicha⁷, Flavio Sztajnbok⁸, Maria Teresa Terreri⁹, Ana Paula Sakamoto¹⁰, Ekaterina Alexeeva¹¹, Jordi Anton¹², Maria Katsicas¹³, Vanessa Smith¹⁴, Tadej Avcin¹⁵, Edoardo Marrani¹⁶, Mikhail Kostik¹⁷, Thomas Lehman¹⁸, Walter Alberto Sifuentes-Giraldo¹⁹, Simone Appenzeller²⁰, Mahesh Janarthanan²¹, Monika Moll²², Dana Nemcova²³, Dieneke Schonenberg-Meinema²⁴, Cristina Battagliotti²⁵, Lillemor Berntson²⁶, Blanca Bica²⁷, Jürgen Brunner²⁸, Patricia Costa Reis²⁹, Despina Eleftheriou³⁰, Liora Harel³¹, Gerd Horneff³², Tilmann Kallinich³³, Dragana Lazarevic³⁴, Kirsten Minden³⁵, Susan Nielsen³⁶, Farzana Nuruzzaman³⁷, Anjali Patwardhan³⁸, Yosef Uziel³⁹ and Nicola Helmus⁴⁰, ¹Head of the Hamburg Centre for Pediatric and Adolescence Rheumatology, Budapest, Hungary, ²Kinderklinik Heidberg Pulmologie, Hamburg, Germany, ³University of Pittsburgh, Pittsburgh, PA, ⁴Hospital Garcia de Orta, Almada, Portugal, ⁵PRINTO, Istituto Giannina Gaslini, Genova, Italy, ⁶Cerrahpaşa Tıp Fakültesi, Istanbul, Turkey, ⁷Riga Stradins University, Riga, Latvia, ⁸Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil, ⁹Federal University of São Paulo, São Paulo, Brazil, ¹⁰UNIFESP, Sao Paulo, Brazil, ¹¹Scientific Center of Children’s Health of RAMS, Moscow, Russia, ¹²Sant Joan de Déu Hospital, Madrid, Spain, ¹³Hospital de Pediatria, Buenos Aires, Argentina, ¹⁴Department of Rheumatology, Ghent University Hospital, Department of Internal Medicine, VIB Inflammation Research Centre Ghent University, Ghent, Belgium, ¹⁵Ljubljana University Medical Centre, Ljubljana, Slovenia, ¹⁶University of Florence, Firenze, Italy, ¹⁷Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ¹⁸Hospital for Special Surgery, New York, ¹⁹Hospital Universitario Ramon y Cajal, Madrid, Spain, ²⁰Unicamp, Campinas, Brazil, ²¹Sri Ramachandra University, Chennai, India, ²²University of Tübingen, Tübingen, Germany, ²³General University Hospital, Prague, Czech Republic, ²⁴Amsterdam UMC, Amsterdam, Netherlands, ²⁵Children's Hospital Dr. Orlando Alassia, Santa Fee, Argentina, ²⁶Uppsala University Hospital, Uppsala, Sweden, ²⁷Hospital Universitário Clementino Fraga Filho (HUCFF/UFRJ), Rio de Janeiro, Brazil, ²⁸Tirol Kliniken, Innsbruck, Innsbruck, Austria, ²⁹Hospital de Santa Maria, Lisbon, Portugal, ³⁰Great Ormond Street Hospital, London, United Kingdom, ³¹Clalit Health Services, Nettnja, Israel, ³²Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany, ³³Charite, Berlin, Germany, ³⁴KC Niš, Nis, Serbia, ³⁵Deutsches Rheuma-Forschungszentrum Berlin, Berlin, Germany, ³⁶Rigshospitalet, Copenhagen, Denmark, ³⁷Stony Brook Children's Hospital, Stony Brook, NY, ³⁸University of Missouri, Columbia, ³⁹Meir Medical Center, Kfar Saba, Israel, ⁴⁰Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany
Background/Purpose: Juvenile systemic sclerosis (jSSc) has a prevalence in around 3 in a million children. Pulmonary involvement occurs in approximately 40 % in the international…
Abstract Number: 2042 • ACR Convergence 2020
A Low FODMAP Diet Is Not Associated with Decreased GI Symptoms or Changes in GI Microbial Composition in Patients with Systemic Sclerosis
Natalie Howlett¹, Sungeun Lee¹, Venu Lagishetty¹, Zsuzsanna McMahan², Meifang Wu¹, Jonathan Jacobs¹ and Elizabeth Volkmann³, ¹University of California, Los Angeles, Los Angeles, ²Johns Hopkins University, Baltimore, ³University of California, Los Angeles, Los Angeles, CA
Background/Purpose: Dietary restriction of short-chain fermentable oligosaccharides, disaccharides, monosaccharides and polyols (low FODMAP) has been found to reduce GI symptoms in patients with IBS and…
Abstract Number: 0383 • ACR Convergence 2020
Sex Differences in Severity and Progression of Interstitial Lung Disease in Systemic Sclerosis: What We Have Learned from Clinical Trials
Elizabeth Volkmann¹, Ning Li², Michael Roth², Carol Feghali-Bostwick³, Richard Silver⁴, DeAnna Baker Frost⁵, Shervin Assassi⁶, Grace Kim², Jonathan Goldin² and Donald Tashkin², ¹University of California, Los Angeles, Los Angeles, CA, ²University of California, Los Angeles, Los Angeles, ³The Medical University of South Carolina, Charleston, SC, ⁴Medical University of South Carolina, Charleston, ⁵The Medical University of South Carolina, Charleston, ⁶University of Texas Houston McGovern Medical School, Division of Rheumatology and Clinical Immunogenetics, Houston, TX
Background/Purpose: Although systemic sclerosis (SSc) disproportionately affects females compared with males [1], observational studies have demonstrated higher mortality rates in males with SSc [2]. This…
Abstract Number: 1172 • ACR Convergence 2020
Cardiovascular involvement as a clue for diagnosis of Juvenile Systemic Sclerosis sine scleroderma
Gloria Lanzoni¹, Giorgia Martini¹, Alessandra Meneghel¹, Fabio Vittadello², Biagio Castaldi¹, Elisabetta Zanatta³ and Francesco Zulian¹, ¹Department of Woman's and Child's Health, University of Padova, Padua, Italy, ²Centro Studi Statistici Explora, Padua, Italy, ³Department of Medicine-DIMED, University of Padova, Padua, Italy
Background/Purpose: Juvenile Systemic Sclerosis (JSSc) is a rare condition in childhood and its variety with no skin involvement, systemic sclerosis sine scleroderma (JSSSS) is anecdotal…
Abstract Number: 0384 • ACR Convergence 2020
Serum Neutrophil Count Predicts Progression of Interstitial Lung Disease and Mortality in Patients with Systemic Sclerosis Related Interstitial Lung Disease
Nancy Wareing¹, Ning Li², Elizabeth Volkmann³, Marka Lyons⁴, Michael Roth², Donald Tashkin² and Shervin Assassi¹, ¹University of Texas Houston McGovern Medical School, Division of Rheumatology and Clinical Immunogenetics, Houston, TX, ²University of California, Los Angeles, Los Angeles, ³University of California, Los Angeles, Los Angeles, CA, ⁴University of Texas Houston, McGovern Medical School, Division of Rheumatology and Clinical Immunogenetics, Houston, TX
Background/Purpose: Systemic sclerosis (SSc) patients have a prominent neutrophil gene expression signature. However, investigations into the pathophysiologic role of neutrophils in SSc are lacking. This…
Abstract Number: 1173 • ACR Convergence 2020
No Disease Progression After 36 Months Follow up in the Juvenile Systemic Scleroderma Inception Cohort
Ivan Foeldvari¹, Jens Klotsche², Ozgur Kasapcopur³, Amra Adrovic⁴, Maria Teresa Terreri⁵, Edoardo Marrani⁶, Tadej Avcin⁷, Maria Katsicas⁸, Dana Nemcova⁹, Maria Jose Santos¹⁰, Jürgen Brunner¹¹, Tilmann Kallinich¹², Mikhail Kostik¹³, Kirsten Minden¹⁴, Anjali Patwardhan¹⁵, Kathryn Torok¹⁶ and Nicola Helmus¹⁷, ¹Head of the Hamburg Centre for Pediatric and Adolescence Rheumatology, Budapest, Hungary, ²German Rheumatism Research Center, Berlin, Germany, ³PRINTO, Istituto Giannina Gaslini, Genova, Italy, ⁴Cerrahpaşa Tıp Fakültesi, Istanbul, Turkey, ⁵Federal University of São Paulo, São Paulo, Brazil, ⁶University of Florence, Firenze, Italy, ⁷Ljubljana University Medical Centre, Ljubljana, Slovenia, ⁸Hospital de Pediatria, Buenos Aires, Argentina, ⁹General University Hospital, Prague, Czech Republic, ¹⁰Hospital Garcia de Orta, Almada, Portugal, ¹¹Tirol Kliniken, Innsbruck, Innsbruck, Austria, ¹²Charite, Berlin, Germany, ¹³Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ¹⁴Deutsches Rheuma-Forschungszentrum Berlin, Berlin, Germany, ¹⁵University of Missouri, Columbia, ¹⁶University of Pittsburgh, Pittsburgh, PA, ¹⁷Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany
Background/Purpose: Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. Longitudinal prospective follow up data of…

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