Abstracts tagged "Scleroderma"

Abstract Number: 1915 • ACR Convergence 2020
Leptin Plays a Critical Role in Modulating Dermal Adipose Tissue, Inflammation and Skin Fibrosis
Roberta Goncalves Marangoni¹, Stacey Duemmel², Marc Nuzzo², Christopher Ritchlin³ and Benjamin Korman³, ¹University of Rochester Medical Center, Rochester, NY, ²Department of Medicine, University of Rochester Medical Center, Rochester, ³Department of Medicine, University of Rochester Medical Center, Rochester, NY
Background/Purpose: We previously demonstrated that systemic sclerosis (SSc) patients have substantial reduction in dermal white adipose tissue (dWAT) which correlates with skin fibrosis. In animal…
Abstract Number: 0913 • ACR Convergence 2020
Systemic Sclerosis Portends a Higher Risk of Conduction and Rhythm Disorders at Diagnosis and During Disease Course: Results from a US Population Based Study
Yasser Radwan¹, Reto Kurmann², Avneek Singh Sandhu³, Edward El-Am⁴, Cynthia Crowson², Eric Matteson⁵, Thomas Osborn², Kenneth Warrington⁶, Rekha Mankad² and Ashima Makol², ¹Mayo Clinic, Rochester, Minnesota, USA, Lansing, MI, ²Mayo Clinic, Rochester, Minnesota, USA, Rochester, MN, ³Mayo Clinic, Rochester, Minnesota, USA, Dayton, OH, ⁴Indiana University School of Medicine, Indianapolis, IN, ⁵Mayo Clinic College of Medicine and Science, Rochester, Minnesota, USA, Rochester, MN, ⁶Mayo Clinic, Rochester, MN
Background/Purpose: Cardiac involvement, including electrocardiogram (ECG) abnormalities, is associated with worse prognosis in systemic sclerosis (SSc). We studied the incidence, risk factors and outcomes of…
Abstract Number: 1170 • ACR Convergence 2020
Exploring the Use of Von Willebrand Factor as a Disease Biomarker in a Cohort of Patients with Juvenile Scleroderma: A Pilot Study
Natalia Vasquez-Canizares¹, Beamon Agarwal², Tamar Rubinstein³, Dawn Wahezi¹ and Morayma Reyes-Gil¹, ¹Albert Einstein College of Medicine, Bronx, NY, ²GenomeRxUS LLC, Clifton Heights, PA, ³Albert Einstein College of Medicine / Montefiore Medical Center, White Plains, NY
Background/Purpose: Juvenile Scleroderma (JScl) is a heterogenous disease characterized by autoimmunity, vasculopathy, and fibrosis. Morbidity and mortality remain high in part due to the continuing…
Abstract Number: 1991 • ACR Convergence 2020
Abatacept Treatment Reduces Cutaneous and Joint Activity in Juvenile Localized Scleroderma
Suzanne Li¹, Sarah Ishaq², Mary Buckley³, Kathryn Torok⁴, Barbara Edelheit⁵, Kaleo Ede⁶, Christopher Liu⁷ and C. Egla Rabinovich⁸, ¹Hackensack University Medical Center, Hackensack, ²Montclair State University, Montclair, ³Duke University, Durham, NC, ⁴University of Pittsburgh, Pittsburgh, PA, ⁵Conneticut Children's Medical Center, Hartford, ⁶Phoenix Children's Hospital, Phoenix, AZ, ⁷U Pittsburgh, Pittsburgh, ⁸Duke University Hospital, Durham, NC
Background/Purpose: Juvenile localized scleroderma (jLS) is an autoimmune disease commonly associated with damage. Damage includes dyspigmentation, tissue atrophy, arthropathy, hemiatrophy, vision loss, and seizures. To…
Abstract Number: 0175 • ACR Convergence 2020
Identifying Immuno-phenotypes in Juvenile Localized Scleroderma with RNA Sequencing
Christina Schutt¹, Emily Mirizio², Kaila Schollaert-Fitch², Claudia Salgado³, Miguel Reyes-Mugica³ and Kathryn Torok², ¹University of Pittsburgh Medical Center, Pittsburgh, PA, ²University of Pittsburgh, Pittsburgh, PA, ³University of Pittsburgh Medical Center, Pittsburgh
Background/Purpose: Juvenile localized scleroderma (jLS) is an autoimmune disease of the skin and underlying tissue characterized by an early inflammatory infiltrate followed by fibrosis and…
Abstract Number: 0915 • ACR Convergence 2020
Clinical Bedside Tools to Assess Systemic Sclerosis Vasculopathy: Can Digital Thermal Monitoring and Sublingual Microscopy Identify Patients with Current or past Digital Ulcers?
Tracy Frech¹, Zhining Ou², Julie Thomas² and Angela Presson², ¹University of Utah and Salt Lake Veterans Affair Medical Center, Salt Lake City, UT, ²University of Utah, Salt Lake City, UT
Background/Purpose: Sublingual microscopy is reported as a useful tool for vasculopathy assessment in SSc. Digital thermal monitoring (DTM) correlates with flow-mediated dilatation, and may identify…
Abstract Number: 1378 • ACR Convergence 2020
Understanding Diagnostic Pathways in Systemic Sclerosis and Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD)
Shervin Assassi¹, Nan Shao², Ziwei Yin², Elizabeth Volkmann³, Donald Zoz² and Thomas Leonard², ¹University of Texas Houston McGovern Medical School, Division of Rheumatology and Clinical Immunogenetics, Houston, TX, ²Boehringer Ingelheim Pharmaceuticals, Ridgefield, CT, ³University of California, Los Angeles, Los Angeles, CA
Background/Purpose: Interstitial lung disease (ILD) is a common manifestation of SSc. SSc-ILD is usually detected in a patient known to have SSc, but ILD may…
Abstract Number: 2018 • ACR Convergence 2020
Development and Validation of an Artificial Intelligence Approach for the Detection of Radiographic Sacroiliitis
Keno-Kyrill Bressem¹, Janis Vahldiek¹, Lisa Adams¹, Stefan Niehues¹, Hildrun Haibel¹, Valeria Rios Rodriguez¹, Murat Torgutalp¹, Mikhail Protopopov¹, Fabian Proft¹, Judith Rademacher¹, Joachim Sieper¹, Martin Rudwaleit², Bernd Hamm¹, Markus Makowski¹, Kay-Geert Hermann¹ and Denis Poddubnyy³, ¹Charité Universitätsmedizin Berlin, Berlin, Germany, ²Department of Internal Medicine and Rheumatology, Klinikum Bielefeld, Germany, ³Charité – Universitätsmedizin Berlin, Berlin, Germany
Background/Purpose: Conventional radiography of the sacroiliac joints is still recommended as the first imaging method if axial spondyloarthritis (axSpA) is suspected. Furthermore, radiographic sacroiliitis is…
Abstract Number: 711 • 2019 ACR/ARP Annual Meeting
Ultrasound Evaluation of the Hands in Patients with Systemic Sclerosis: Osteophytosis Is a Major Contributor to Tender Joints
Robert Fairchild¹, Melody Chung ¹, Laurel Sharpless ¹, Shufeng Li ², Jison Hong ¹, Khushboo Sheth ¹ and Lorinda Chung ¹, ¹Stanford University, Palo Alto, CA, ²Division of Immunology and Rheumatology, Department of Medicine, Stanford, Palo Alto, CA
Background/Purpose: Systemic Sclerosis (SSc) is a progressive fibrotic and vascular disease with peripheral manifestations including arthritis, tendinopathy, sclerodactyly, contractures, calcinosis, acroosteolysis, and vascular disease which…
Abstract Number: 1057 • 2019 ACR/ARP Annual Meeting
Dissecting the Cellular Mechanism of Prostacyclin Analog Iloprost in Reversing Vascular Dysfunction in Scleroderma
Pei-Suen Tsou¹, Nicholas Flavahan ² and Dinesh Khanna ³, ¹University of Michigan, Ann Arbor, MI, ²The Johns Hopkins University School of Medicine, Baltimore, MD, ³Scleroderma Program, Division of Rheumatology, Department of Internal Medicine, Autoimmunity Center of Excellence, University of Michigan, Ann Arbor
Background/Purpose: Iloprost improves Raynaud‘s phenomenon and digital ulcers in scleroderma (SSc) patients. This is hypothesized to reflect anti-platelet and vasodilatory effects. Different trials and cohorts…
Abstract Number: 1833 • 2019 ACR/ARP Annual Meeting
Efficacy and Safety of Nintedanib in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease by Use of Mycophenolate at Baseline: Subgroup Analysis of the SENSCIS Trial
Kristin Highland¹, Oliver Distler ², Masataka Kuwana ³, Yannick Allanore ⁴, Shervin Assassi ⁵, Arata Azuma ⁶, Arnaud Bourdin ⁷, Christopher Denton ⁸, Jörg Distler ⁹, Anna Maria Hoffmann-Vold ¹⁰, Dinesh Khanna ¹¹, Maureen Mayes ⁵, Ganesh Raghu ¹², Madelon Vonk ¹³, Martina Gahlemann ¹⁴, Mannaig Girard ¹⁵, Susanne Stowasser ¹⁶, Donald Zoz ¹⁷, Aryeh Fischer ¹⁸ and Toby Maher ¹⁹, ¹Cleveland Clinic, Cleveland, Ohio, USA, Cleveland, OH, ²Dept. of Rheumatology, University Hospital Zürich, Zürich, Switzerland, Zürich, Switzerland, ³Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, Bunkyo-ku, Tokyo, Japan, ⁴Dept. of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France, ⁵Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, Texas, USA, Houston, TX, ⁶Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan, Tokyo, Japan, ⁷PhyMedExp, University of Montpellier, INSERM U1046, CNRS UMR 9214 and Department of Respiratory Diseases, University of Montpellier, CHU Montpellier, Montpellier, France, Montpellier, ⁸University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, UK, London, United Kingdom, ⁹Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, Erlangen, Germany, ¹⁰Department of Rheumatology, Oslo University Hospital, Oslo, Norway, Oslo, Norway, ¹¹Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor, ¹²University of Washington, Seattle, USA, Seattle, ¹³Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands, Nijmegen, Gelderland, Netherlands, ¹⁴Boehringer Ingelheim (Schweiz) GmbH, Basel, Switzerland, Basel, Switzerland, ¹⁵Boehringer Ingelheim France S.A.S., Reims, France, Reims, France, ¹⁶Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, Ingelheim am Rhein, Germany, ¹⁷Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, Connecticut, USA, Ridgefield, CT, ¹⁸University of Colorado School of Medicine, Denver, Colorado, USA, Denver, CO, ¹⁹National Heart and Lung Institute, Imperial College London, UK and National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London, UK, London, United Kingdom
Background/Purpose: In the SENSCIS trial in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD), nintedanib reduced the annual rate of decline in forced vital capacity…
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…

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