Abstracts tagged "Scleroderma"

Abstract Number: 712 • 2019 ACR/ARP Annual Meeting
Safety and Tolerability of Nintedanib in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease in the SENSCIS Trial: Subgroup Analysis Based on Demographic Characteristics
Oliver Distler¹, Kristin Highland ², Maureen Mayes ³, Masataka Kuwana ⁴, Lesley Saketkoo ⁵, Madelon Vonk ⁶, Michael Kreuter ⁷, Laura Hummers ⁸, Ute von Wangenheim ⁹, Martina Gahlemann ¹⁰, Veronika Kohlbrenner ¹¹, Margarida Alves ¹², Emmanuelle Clerisme-Beaty ¹² and Arata Azuma ¹³, ¹Dept. of Rheumatology, University Hospital Zürich, Zürich, Switzerland, Zürich, Switzerland, ²Cleveland Clinic, Cleveland, Ohio, USA, Cleveland, OH, ³Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, Texas, USA, Houston, TX, ⁴Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, Bunkyo-ku, Tokyo, Japan, ⁵New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans; Tulane University School of Medicine, University Medical Center – Comprehensive Pulmonary Hypertension Center, USA, New Orleans, ⁶Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands, Nijmegen, Gelderland, Netherlands, ⁷Center for Interstitial and Rare Lung Diseases, Pneumology, Thoraxklinik, University of Heidelberg, Member of the German Center for Lung Research, Germany, Germany, Germany, ⁸Johns Hopkins University School of Medicine, Division of Rheumatology, Baltimore, MD, USA, Baltimore, MD, ⁹Boehringer Ingelheim Pharma GmbH & Co. KG, Biberach an der Riss, Germany, Biberach an der Riss, ¹⁰Boehringer Ingelheim (Schweiz) GmbH, Basel, Switzerland, Basel, Switzerland, ¹¹Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, CT, USA, Ridgefield, ¹²Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, Ingelheim am Rhein, Germany, ¹³Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan, Tokyo, Japan
Background/Purpose: In the SENSCIS trial in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD), the adverse events associated with nintedanib were manageable for most patients…
Abstract Number: 1059 • 2019 ACR/ARP Annual Meeting
BDCA2 Targeting of Human Plasmacytoid Dendritic Cells via CBS004 Reverts Dependent IFN Activation and Tissue Fibrosis in vitro and in vivo
Rebecca Ross¹, Clarissa Corinaldesi ², Gemma Migneco ³, Yasser El-Sherbiny ⁴, Steve Holmes ⁵, Jörg Distler ⁶, Clive McKimmie ⁷ and Francesco Del Galdo ⁸, ¹Univesristy of Leeds, Leeds, England, United Kingdom, ²University of Leeds, Leeds, England, United Kingdom, ³University of Leeds, Leeds, United Arab Emirates, ⁴University of Nottingham, Nottingham, United Kingdom, ⁵Capella Bioscience, london, United Kingdom, ⁶Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, Erlangen, Germany, ⁷University of Leeds, Leeds, United Kingdom, ⁸University of Leeds and LTHT NIHR Biomedical Research Centre, Leeds, United Kingdom
Background/Purpose: Human plasmacytoid dendritic cells (pDCs) have been implicated in the pathogenesis of Systemic Sclerosis (SSc) through their ability to infiltrate the skin and secrete…
Abstract Number: 1834 • 2019 ACR/ARP Annual Meeting
Serum Interferon Chemokine Score Predicts Better Response to Immunosuppression in Systemic Sclerosis Related Interstitial Lung Disease
Shervin Assassi¹, Ning Li ², Elizabeth Volkmann ³, Maureen Mayes ¹, Jun Ying ⁴, Michael Roth ⁵, Philip Clements ², Daniel Furst ⁶, Dinesh Khanna ⁷, Jonathan Goldin ², Robert Elashoff ² and Donald Tashkin ², ¹Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, Texas, USA, Houston, TX, ²University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, ³University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, ⁴University of Texas McGovern Medical School at Houston, Houston, TX, ⁵University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, ⁶University of California, Los Angeles, CA, ⁷Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor
Background/Purpose: Response to immunosuppression is highly variable in systemic sclerosis (SSc) related interstitial lung disease (ILD), and there are no widely accepted clinical or biological…
Abstract Number: 721 • 2019 ACR/ARP Annual Meeting
Ultrasound Measurement of the Nail Bed Matrix Thickness as a Useful Marker for Scleroderma-Related Interstitial Lung Disease
Marian Sidor¹, Roman Zuckerman ¹, Naomi Schlesinger ², Ross Sussman ³, Huma Jaleel Sabahath ⁴ and Vivien Hsu ⁵, ¹Rutgers-RWJ Medical School, New Brunswick, NJ, ²Rutgers Health- RWJ Medical School, New Brunswick, NJ, ³Rutgers-RWJ Medical School, East Brunswick, NJ 08816, NJ, ⁴AtlantiCare Regional Medical Center, Atlantic City, NJ, ⁵Rutgers- RWJ Medical School, SOUTH PLAINFIELD, NJ
Background/Purpose: Lung involvement is the leading cause of death in systemic sclerosis (SSc) (1). Pulmonary function tests (PFTs) and chest high-resolution CT scan (HRCT) are…
Abstract Number: 1062 • 2019 ACR/ARP Annual Meeting
Lymphocyte Subset Abnormalities in Early Diffuse Cutaneous Systemic Sclerosis
David Fox¹, Steven Lundy ², Michael Whitfield ³, Veronica Berrocal ⁴, Phillip Campbell ⁵, Stephanie Rasmussen ⁵, Ray Ohara ⁶, Alexander Stinson ⁶, Evan Wiewiora ⁷, Cathie Spino ⁷, Erica Bush ⁸, Daniel Furst ⁹, Shiv Pillai ¹⁰ and Dinesh Khanna ¹¹, ¹Division of Rheumatology, Department of Internal Medicine, Autoimmunity Center of Excellence,University of Michigan, Ann Arbor, MI, ²SystImmune, Inc., Redmond, WA, ³Department of Biomedical Data Science, Geisel School of Medicine at Dartmouth, Hannover, NH, ⁴Department of Biostatistics, School of Publich Health, University of Michigan, Ann Arbor, MI, ⁵Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, ⁶Washington University, St. Louis, MO, ⁷Department of Biostatistics, School of Public Health, University of Michigan, Ann Arbor, MI, ⁸Scleroderma Program, Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, ⁹University of California, Los Angeles, CA, ¹⁰Ragon Institude of MGH, MIT and Harvard, Charlestown, MA, ¹¹Scleroderma Program, Division of Rheumatology, Department of Internal Medicine, Autoimmunity Center of Excellence, University of Michigan, Ann Arbor
Background/Purpose: A variety of abnormalities in lymphocyte surface markers and functional subsets have been described in patients with systemic sclerosis (SSc), but conflicting results abound,…
Abstract Number: 1835 • 2019 ACR/ARP Annual Meeting
Frequency and Predictors of Meaningful Decline in Forced Vital Capacity During Follow up оf a Large Cohort of Systemic Sclerosis Associated Pulmonary Fibrosis Patients
Svetlana Nihtyanova¹, Emma Derrett-Smith ¹, Carmen Fonseca ¹, Voon Ong ² and Christopher Denton ³, ¹UCL Medical School, Royal Free Campus, London, United Kingdom, ²UCL Medical School, Royal Free Campus, London, ³University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, UK, London, United Kingdom
Background/Purpose: Pulmonary fibrosis (PF) is common in systemic sclerosis (SSc) and serial pulmonary function tests (PFTs) are used for routine PF monitoring. Forced vital capacity…
Abstract Number: 724 • 2019 ACR/ARP Annual Meeting
Biomechanical Properties of Skin for Assessment of Scleroderma: A Systemic Review
Peiyun Ni¹, Lilit Garibyan ² and Rox Anderson ², ¹Harvard-MIT Health Sciences and Technology, Harvard Medical School, Boston, MA, ²Wellman Center for Photomedicine, Massachusetts General Hospital, Boston, MA
Background/Purpose: Skin involvement is one of the early features of scleroderma and an important predictor of internal organ involvement and mortality. Skin fibrosis alters the…
Abstract Number: 1064 • 2019 ACR/ARP Annual Meeting
CD123+ Plasmacytoid Dendritic Cells from Systemic Sclerosis Patients Are Susceptible to the Cytotoxic Activity of Tagraxofusp, a CD123-Targeted Therapy
Ross Lindsay¹, Janice Chen ¹, Robert Spiera ², Jessica Gordon ², Marie-Dominique Ah Kioon ³, Franck Barrat ³ and Christopher Brooks ¹, ¹Stemline Therapeutics, New York, ²Hospital for Special Surgery, New York, NY, ³HSS Research Institute, Hospital for Special Surgery, New York
Background/Purpose: Tagraxofusp is a novel targeted therapy directed to the interleukin-3 receptor (CD123). Tagraxofusp is comprised of human IL-3 recombinantly fused to a truncated diphtheria…
Abstract Number: 1957 • 2019 ACR/ARP Annual Meeting
Aggregation of Functional Variants in NOTCH4 Gene Increases SSc Risk
Pravitt Gourh¹, Sarah Safran ², Theresa Alexander ³, Steven Boyden ⁴, Ami Shah ⁵, Maureen Mayes ⁶, Ayo Doumatey ⁷, Amy Bentley ⁷, Daniel Shriner ⁷, Robyn Domsic ⁸, Thomas Medsger Jr ⁹, Paula Ramos ¹⁰, Richard Silver ¹¹, Virginia Steen ¹², John Varga ¹³, Vivien Hsu ¹⁴, Lesley Saketkoo ¹⁵, Elena Schiopu ¹⁶, Dinesh Khanna ¹⁷, Jessica Gordon ¹⁸, Brynn Kron ¹⁹, Lindsey Criswell ²⁰, Heather Gladue ²¹, Chris Derk ²², Elana Bernstein ²³, S Louis Bridges ²⁴, Victoria Shanmugam ²⁵, Kathleen Kolstad ²⁶, Lorinda Chung ²⁷, Suzanne Kafaja ²⁸, Reem Jan ²⁹, Marcin Trojanowski ³⁰, Avram Goldberg ³¹, Benjamin Korman ³², Peter Steinbach ³³, Settara Chandrasekharappa ⁷, James Mullikin ⁷, Adebowale Adeyemo ⁷, Charles Rotimi ⁷, Frederick Wigley ³⁴, Daniel Kastner ³⁵, Francesco Boin ³⁶ and Elaine Remmers ⁷, ¹National Institutes of Rheumatology, Bethesda, ²National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), Bethesda, MD, ³University of Maryland, College Park, MD, ⁴University of Utah, SALT LAKE CITY, UT, ⁵Johns Hopkins Hospital, Baltimore, MD, ⁶Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, Texas, USA, Houston, TX, ⁷National Human Genome Research Institute (NHGRI), Bethesda, MD, ⁸University of Pittsburgh, Pittsburgh, PA, ⁹University of Pittsburg School of Medicine, Pittsburg, PA, ¹⁰Medical University of South Carolina, Charleston, ¹¹Division of Rheumatology, Medical University of South Carolina, Charleston, SC, ¹²Georgetown University, Washington, D.C., USA, Georgetown, ¹³Northwestern University, Chicago,, IL, ¹⁴Rutgers- RWJ Medical School, SOUTH PLAINFIELD, NJ, ¹⁵New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans; Tulane University School of Medicine, University Medical Center – Comprehensive Pulmonary Hypertension Center, USA, New Orleans, ¹⁶Department of Rheumatology, University of Michigan, Ann Arbor, ¹⁷Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor, ¹⁸Hospital for Special Surgery, New York, NY, ¹⁹University of California San Francisco, San Francisco, CA, ²⁰University of California, San Francisco, San Francisco, ²¹Arthritis & Osteoporosis Consultants Of The Carolinas, Charlotte, NC, ²²University of Pennsylvania, Philadelphia, PA, ²³Division of Rheumatology, Columbia University, New York, NY, ²⁴University of Alabama at Birmingham, Birmingham, ²⁵George Washington University, Georgetown, DC, ²⁶Division of Immunology and Rheumatology, Department of Medicine, Stanford University, Stanford, CA, ²⁷Stanford University, Palo Alto, CA, ²⁸Department of Medicine. Rheumatology Division. UCLA, Los Angeles, CA, ²⁹University of Chicago Pritzker School of Medicine, Chicago, IL, ³⁰Boston University Medical Center, Boston, MA, ³¹New York University Langone Medical Center, New York, NY, ³²University of Rochester Medical Center, Rochester, NY, ³³Center for Molecular Modeling, Center for Information Technology, National Institutes of Health, Bethesda, MD, ³⁴Johns Hopkins University, Division of Rheumatology, Baltimore, ³⁵National Institutes of Health, Bethesda, MD, ³⁶UCSF, San Francisco, CA
Background/Purpose: Genetic studies of common variants in scleroderma (systemic sclerosis, SSc) have identified several susceptibility loci increasing SSc risk. Most of these common variants are…
Abstract Number: 725 • 2019 ACR/ARP Annual Meeting
Amniotic Membrane Dressings Provide an Effective Treatment for Systemic Sclerosis Digital Ulcers
Tracy Frech¹, Jan Pierce ², Greg Stoddard ³, Constance McNeill ⁴, Mislav Radic ⁵ and Jo -Anna Reems ⁶, ¹Division of Rheumatology, University of Utah and Salt Lake VAMC, Salt Lake City, UT, ²University of Utah Regenerative Medicine, Salt Lake City, United Kingdom, ³University of Utah Study Design and Biostatics Core, Salt Lake City, UT, ⁴University of Utah Hospitals and Clinics, Salt Lake City, UT, ⁵University Hospital Split, Split, Croatia, ⁶University of Utah Regenerative Medicine, Salt Lake City, UT
Background/Purpose: Systemic sclerosis (SSc)-related digital ulcers are a major burden for patients. The purpose of this study was to assess the effectiveness of amnion membrane…
Abstract Number: 1066 • 2019 ACR/ARP Annual Meeting
Dysregulated IL-6 Dependent Dermal Adenosine Signaling via Adenosine A2A Receptor May Drive Fibrosis in Systemic Sclerosis
Christopher Denton ¹, Shiwen Xu ², Sue Verma ³ and Voon Ong⁴, ¹University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, UK, London, United Kingdom, ²University College London, London, United Kingdom, ³Leadiant Biosciences, London, United Kingdom, ⁴UCL Medical School, Royal Free Campus, London
Background/Purpose: Altered generation of adenosine from extracellular nucleotides by ectonucleotidases may orchestrate chronic injury responses and promote fibrosis via the adenosine A2A receptor (ADORA2A). We…
Abstract Number: 2590 • 2019 ACR/ARP Annual Meeting
Computer Vision Applied to Dual Energy Computed Tomography Images for Precise Calcinosis Cutis Quantification in Patients with Systemic Sclerosis
Anita Chandrasekaran¹, Imran Omar ², Zhicheng Fu ³, Shangping Ren ³ and Monique Hinchcliff ¹, ¹Yale University, Section of Rheumatology, Allergy and Immunology, New Haven, CT, ²Northwestern University Feinberg School of Medicine, Chicago, IL, ³San Diego State University, Department of Computer Science, San Diego, CA
Background/Purpose: Calcinosis cutis, found in both systemic sclerosis (SSc) and juvenile dermatomyositis patients, can be extensive and debilitating. Potential treatments have been identified, but a…
Abstract Number: 728 • 2019 ACR/ARP Annual Meeting
Nailfold Videocapillaroscopy Patterns and Digital Occlusive Arterial Disease on Laser Doppler Flowmetry Strongly Predicts the Diagnosis of Systemic Sclerosis and Other Connective Tissue Diseases
Yasser Radwan¹, Tina Gunderson ², Cynthia Crowson ³, David Liedl ⁴, Alicia Hinze ¹, Kenneth Warrington ⁴, Paul Wennberg ⁴ and Ashima Makol ¹, ¹Mayo Clinic Minnesota, rochester, MN, ²Mayo Clinic, Rochester, MN, ³Mayo Clinic Rochester, Rochester, ⁴Mayo Clinic Rochester, rochester, MN
Background/Purpose: Vasculopathy is a key feature of systemic sclerosis (SSc), a chronic autoimmune disease associated with widespread fibrosis and internal organ dysfunction. While structural abnormalities…
Abstract Number: 1069 • 2019 ACR/ARP Annual Meeting
CD4+ T Helper Cell Populations with High PD-1 Expression Are Expanded in Systemic Sclerosis
Alisa Mueller¹, Andrea Fava ², Deepak Rao ¹ and Francesco Boin ³, ¹Brigham and Women's Hospital, Boston, MA, ²Johns Hopkins University School of Medicine, Baltimore, MD, ³UCSF, San Francisco, CA
Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disease characterized by tissue fibrosis, vascular injury, and autoantibody production. CXCR5+ PD-1+ T follicular helper (Tfh) cells, which…
Abstract Number: 2591 • 2019 ACR/ARP Annual Meeting
Hospitalization Among Incident Cases of Systemic Sclerosis: Results from a Population-based Cohort (1980-2016)
Caitrin Coffey¹, Avneek Singh Sandhu ², Cynthia Crowson ³, Sara Achenbach ¹, Eric Matteson ⁴, Thomas Osborn ⁵, Kenneth Warrington ⁶ and Ashima Makol ⁵, ¹Mayo Clinic, Rochester, MN, ²kettering health, dayton, OH, ³Mayo Clinic Rochester, Rochester, ⁴Mayo Clinic College of Medicine and Science, Rochester, Minnesota, USA, Rochester, ⁵Mayo Clinic Minnesota, rochester, MN, ⁶Mayo Clinic Rochester, Rochester, MN
Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disorder associated with multi-organ dysfunction, requiring long-term and multi-disciplinary care. Few studies have estimated the healthcare resource usage…

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