Abstracts tagged "scleroderma and systemic sclerosis"

Abstract Number: 733 • 2017 ACR/ARHP Annual Meeting
Ethnic Variation in Systemic Sclerosis Morbidity and Mortality
Sindhu Johnson¹, Zareen Ahmad² and Haifa Al Sheikh³, ¹Division of Rheumatology, Toronto Western Hospital, Mount Sinai Hospital, Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, ON, Canada, ²Toronto Scleroderma Program, Division of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, ³Division of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada
Background/Purpose: Systemic sclerosis (SSc) is an uncommon connective tissue disease characterized by pathological skin thickening and can involve multiple internal organs. Ethnic variations in SSc…
Abstract Number: 2696 • 2017 ACR/ARHP Annual Meeting
Things Left Unsaid: Important Topics That Are Not Discussed between Patients with Systemic Sclerosis, Their Carers and Their Healthcare Professionals
Christopher Denton¹, Bee Laird², Lizette Moros³ and José Luis Luna Flores⁴, ¹Department of Rheumatology, University College London, Royal Free Hospital, London, United Kingdom, ²Reframe Research, East Sussex, United Kingdom, ³Boehringer-Ingelheim, Ingelheim, Germany, ⁴Boehringer Ingelheim, Ingelheim, Germany
Background/Purpose: Systemic sclerosis (SSc) is a serious rare condition that can be complicated by internal organ damage including interstitial lung fibrosis (SSc-ILD), which is a…
Abstract Number: 737 • 2017 ACR/ARHP Annual Meeting
Dynamic Prediction of Pulmonary Hypertension Development in Systemic Sclerosis Patients Using Landmark Analysis – Comparison of Two Models
Svetlana I. Nihtyanova¹, Voon H. Ong², Emma C. Derrett-Smith³, Benjamin Schreiber⁴, J. Gerry Coghlan⁴, Bianca DeStavola⁵ and Christopher Denton⁶, ¹Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, ²Rheumatology, UCL Division of Medicine, London, United Kingdom, ³Centre for Rheumatology and Connective Tissue Diseases, UCL Division of Medicine, London, United Kingdom, ⁴National Pulmonary Hypertension Service, Royal Free Hospital, London, United Kingdom, ⁵Faculty of Population Health Sciences, UCL Institute of Child Health, London, United Kingdom, ⁶Department of Rheumatology, University College London, Royal Free Hospital, London, United Kingdom
Background/Purpose: Pulmonary hypertension (PH) contributes substantially to systemic sclerosis (SSc)-related morbidity and mortality. It tends to develop later in the disease, creating an opportunity for…
Abstract Number: 2982 • 2017 ACR/ARHP Annual Meeting
Morbidity and Mortality of Scleroderma in African Americans
Duncan F. Moore¹, Elisabeth Kramer¹, Rami Eltaraboulsi² and Virginia D. Steen¹, ¹Division of Rheumatology, Department of Medicine, MedStar Georgetown University Hospital, Washington, DC, ²MedStar Georgetown University Hospital, Washington, DC
Background/Purpose: Retrospective cohorts have demonstrated that African Americans (AAs) with scleroderma are more likely to have severe disease and higher mortality than non-AAs. A prior…
Abstract Number: 761 • 2017 ACR/ARHP Annual Meeting
Increased Expression of the TNF Superfamily Member LIGHT/TNFSF14 and Its Receptor (TNFRSF14) in Patients with Systemic Sclerosis
Otylia Kowal-Bielecka¹, Ewa Gindzienska-Sieskiewicz¹, Oliver Distler², Joanna Reszec³, Suzana Jordan², Pawel Bielecki⁴, Andzrzej Sieskiewicz⁴, Agnieszka Sulik¹ and Krzysztof Kowal^5,6, ¹Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland, ²Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, ³Department of Medical Pathomorphology, Department of Medical Pathomorphology, Medical University of Bialystok, Bialystok, Poland, ⁴Department of Otolaryngology, Medical University of Bialystok, Bialystok, Poland, ⁵Department of Allergology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland, ⁶Department of Experimental Allergology and Immunology, Medical University of Bialystok, Bialystok, Poland
Background/Purpose: The TNF Superfamily member LIGHT (TNFSF14) regulates immune response and angiogenesis. Moreover, recent studies indicate that interactions of LIGHT with its receptor, TNFRSF14, might…
Abstract Number: 775 • 2017 ACR/ARHP Annual Meeting
Transcriptome Sequencing Reveals Genetic Polymorphisms Associated with Ssc Gene Expression Subtypes
Guoshuai Cai¹, Bhaven K. Mehta², Mengqi Huang², Jennifer Franks¹, Tammara A. Wood¹, Kathleen D. Kolstad³, Marianna Stark⁴, Antonia Valenzuela⁵, David Fiorentino⁶, Robert W. Simms⁷, Nicole Orzechowski⁸, Lorinda Chung⁹ and Michael L. Whitfield², ¹Department of Molecular and Systems Biology, Geisel School of Medicine at Dartmouth, Hanover, NH, ²Molecular and Systems Biology, Geisel School of Medicine at Dartmouth, Hanover, NH, ³Rheumatology, Stanford University Medical Center, Stanford, CA, ⁴Stanford University, Stanford, CA, ⁵Immunology and Rheumatology, Stanford University, Palo Alto, CA, ⁶Department of Dermatology, Stanford University School of Medicine, Palo Alto, CA, ⁷Rheumatology, Boston University School of Medicine, Boston, MA, ⁸Dartmouth-Hitchcock Medical Center, Lebanon, NH, ⁹Rheumatology, Stanford University Medical Center, Palo Alto, CA
Background/Purpose: Systemic sclerosis (SSc) is a complex disease characterized by substantial genotypic and phenotypic heterogeneity. Four molecular gene expression subsets have been identified from SSc…
Abstract Number: 919 • 2017 ACR/ARHP Annual Meeting
HLA Type Imputation in the Genome Research in African American Scleroderma Patients (GRASP) Cohort Reveals Strong Associations of African Ancestry MHC Class II Types with Scleroderma and Lack of Class I HLA Type Associations
Elaine F. Remmers¹, Pravitt Gourh², Steven Boyden³, Nadia D. Morgan⁴, Ami A. Shah⁴, Adebowale Adeyemo¹, Amy Bentley¹, Mary A. Carns⁵, Settara C. Chandrasekharappa¹, Lorinda Chung⁶, Lindsey A. Criswell⁷, Chris T. Derk⁸, Robyn T. Domsic⁹, Ayo Doumatey¹, Heather Gladue¹⁰, Avram Goldberg¹¹, Jessica K. Gordon¹², Vivien M Hsu¹³, Reem Jan¹⁴, Dinesh Khanna¹⁵, Maureen D. Mayes¹⁶, Thomas A. Medsger Jr.¹⁷, Paula S. Ramos¹⁸, Marcin A. Trojanowski¹⁹, Lesley A. Saketkoo²⁰, Elena Schiopu¹⁵, Victoria K. Shanmugam²¹, Daniel Shriner¹, Richard M. Silver²², Virginia D. Steen²³, Antonia Valenzuela²⁴, John Varga²⁵, Charles Rotimi¹, Fredrick M. Wigley²⁶, Francesco Boin²⁷ and Daniel L. Kastner²⁸, ¹National Institutes of Health (NIH), National Human Genome Research Institute, Bethesda, MD, ²NIAMS-Rheumatology, National Institutes of Health (NIH), National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ³National Institutes of Health (NIH), National Institute on Deafness and Other Communication Disorders, Bethesda, MD, ⁴Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, ⁵Northwestern University, Feinberg School of Medicine Scleroderma Program, Chicago, IL, ⁶Rheumatology, Stanford University Medical Center, Palo Alto, CA, ⁷Medicine/Rheumatology, University of California, San Francisco, San Francisco, CA, ⁸Rheumatology, University of Pennsylvania, Philadelphia, PA, ⁹Rheumatology, University of Pittsburgh, Pittsburgh, PA, ¹⁰Rheumatology, Arthritis and Osteoporosis Consultants of the Carolinas, Charlotte, NC, ¹¹NYU Langone Medical Center, New York, NY, ¹²Rheumatology, Hospital for Special Surgery, New York, NY, ¹³University of Medicine and Dentistry of New Jersey--Robert Wood Johnson Medical School, New Brunswick, NJ, ¹⁴Medicine, Rheumatology, University of Chicago, Chicago, IL, ¹⁵University of Michigan, Ann Arbor, MI, ¹⁶University of Texas McGovern Medical School, Houston, TX, ¹⁷Department of Medicine/Rheumatology, University of Pittsburgh, Pittsburgh, PA, ¹⁸Department of Public Health Sciences, Medical University of South Carolina, Charleston, SC, ¹⁹Boston University School of Medicine, Boston, MA, ²⁰Rheumatology, Tulane University School of Medicine, New Orleans, LA, ²¹Rheumatology, The George Washington University, Washington, DC, ²²Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, Charleston, SC, ²³Rheumatology, MedStar Georgetown University Hospital, Washington, DC, ²⁴Stanford University School of Medicine, Stanford, CA, ²⁵Rheumatology and Dermatology, Northwestern University, Feinberg School of Medicine Scleroderma Program, Chicago, IL, ²⁶Rheum Div/Mason F Lord, Johns Hopkins University, Baltimore, MD, ²⁷Rheumatology, University California, San Francisco, San Francisco, CA, ²⁸Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD
Background/Purpose: The Genome Research in African American Scleroderma Patients (GRASP) consortium was created to obtain a collection of African American (AA) scleroderma patients to facilitate…
Abstract Number: 1248 • 2017 ACR/ARHP Annual Meeting
Evaluation of Validated Patient Reported Outcome Measures to Assess Sensitivity to Change in Patients with Systemic Sclerosis and Gastroesophageal Reflux Disease —a Scleroderma Clinical Trials Consortium Collaborative Project
Zsuzsanna McMahan¹, Tracy M. Frech², David Lim³, Veronica J. Berrocal⁴, Cosimo Bruni⁵, Marco Matucci-Cerinic⁶, Vanessa Smith⁷, Karin Melsons⁸, Susanna Proudman⁹, Jinyu Zhang¹⁰, Fabian A Mendoza¹¹, Melanie Woods³ and Dinesh Khanna³, ¹Department of Internal Medicine, Johns Hopkins University, Baltimore, MD, ²Division of Rheumatology, University of Utah, Salt Lake City, UT, ³University of Michigan, Ann Arbor, MI, ⁴Div of Rheumatology, University of Michigan, Ann Arbor, MI, ⁵Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Firenze, Italy, ⁶Dept of Medicine/Div of Rheum, University of Florence, Firenze, Italy, ⁷Faculty of Internal Medicine, Ghent University, Ghent, Belgium, ⁸Ghent University, Gent, Belgium, ⁹Discipline of Medicine, University of Adelaide, Adelaide, Australia, ¹⁰Thomas Jefferson University, Philadelphia, PA, ¹¹Jefferson Institute of Molecular Medicine and Scleroderma Center, Rheumatology Division, Department of Medicine, Thomas Jefferson University, Philadelphia, PA
Background/Purpose: The UCLA SCTC GIT 2.0 and NIH PROMIS GI Symptoms Scales are validated in scleroderma to assess patient-reported gastrointestinal (GI) involvement. We sought to…
Abstract Number: 1676 • 2017 ACR/ARHP Annual Meeting
Improving Sensitivity to Change of the Modified Rodnan Skin Score over Time
Annel M. Fernandez¹, Robert F. Spiera², Jackie Szymonifka² and Jessica K. Gordon², ¹Medicine- Rheumatology/ Research, Hospital For Special Surgery, New York, NY, ²Rheumatology, Hospital for Special Surgery, New York, NY
Background/Purpose: The Modified Rodnan Skin Score (MRSS) assesses global dermal thickness through the examination of 17 body areas scored by clinical palpation using a 4…
Abstract Number: 1695 • 2017 ACR/ARHP Annual Meeting
Baseline Characteristics and Outcomes in a Retrospective Cohort of Patients with Systemic Sclerosis Related Interstitial Lung Disease
Robert L. Mango¹, Eric L. Matteson², Cynthia S. Crowson³, Jay H. Ryu⁴ and Ashima Makol², ¹Internal Medicine, Division of Rheumatology, Mayo Clinic College of Medicine and Science, Rochester, MN, MN, ²Rheumatology, Mayo Clinic College of Medicine and Science, Rochester, MN, ³Health Sciences Research, Mayo Clinic College of Medicine and Science, Rochester, MN, ⁴Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine and Science, Rochester, MN
Background/Purpose: Interstitial lung disease (ILD) is a major contributor to morbidity and mortality in systemic sclerosis (SSc). We sought to identify the distribution of clinical…
Abstract Number: 1713 • 2017 ACR/ARHP Annual Meeting
Defining Genetic Risk for Scleroderma Renal Crisis in RNA-Polymerase III Antibody Positive Patients
Edward Stern¹, Sandra Guerra¹, Harry Chinque¹, David Gonzalez Serna², Markella Ponticos¹, Javier Martin², Maureen D. Mayes³, Shervin Assassi⁴, Carmen Fonseca¹ and Christopher Denton⁵, ¹UCL Centre for Rheumatology and Connective Tissue Diseases, London, United Kingdom, ²Instituto de Parasitología y Biomedicina López-Neyra, Granada, Spain, ³Internal Medicine/Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, ⁴University of Texas McGovern Medical School, Houston, TX, ⁵Department of Rheumatology, University College London, Royal Free Hospital, London, United Kingdom
Background/Purpose: Scleroderma renal crisis (SRC), characterised by accelerated hypertension and acute kidney injury, is a life-threatening complication of systemic sclerosis (SSc). Most SSc cases have…
Abstract Number: 1922 • 2017 ACR/ARHP Annual Meeting
Cell Type Specific Gene Expression Analysis of Early Systemic Sclerosis Skin Shows a Prominent Activation Pattern of Innate and Adaptive Immune System in the Prospective Registry for Early Systemic Sclerosis (PRESS) Cohort
Shervin Assassi¹, Dinesh Khanna², Monique Hinchcliff³, Virginia D. Steen⁴, Faye Hant⁵, Jessica K. Gordon⁶, Ami A. Shah⁷, Jun Ying⁸, William Swindell⁹, Wenjin Zheng¹⁰, Lisha Zhu¹⁰, Victoria K. Shanmugam¹¹, Robyn T. Domsic¹², Flavia V. Castelino¹³, Elana J. Bernstein¹⁴ and Tracy M. Frech¹⁵, ¹University of Texas McGovern Medical School, Houston, TX, ²University of Michigan, Ann Arbor, MI, ³Rheumatology, Northwestern Medicine, Chicago, IL, ⁴Rheumatology, MedStar Georgetown University Hospital, Washington, DC, ⁵Medicine/Rheumatology & Immunology, Medical University of South Carolina, Charleston, SC, ⁶Rheumatology, Hospital for Special Surgery, New York, NY, ⁷Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, ⁸Department of Internal Medicine - Rheumatology, University of Texas McGovern Medical School, Houston, TX, ⁹Dermatology, University of Michigan - Ann Arbor, Ann Arbor, MI, ¹⁰University of Texas - School of Biomedical Informatics, Houston, TX, ¹¹Rheumatology, The George Washington University, Washington, DC, ¹²Rheumatology, University of Pittsburgh, Pittsburgh, PA, ¹³Rheumatology, Harvard Medical School, Boston, MA, ¹⁴Rheumatology, Columbia University, New York, NY, ¹⁵Division of Rheumatology, University of Utah, Salt Lake City, UT
Background/Purpose: To examine the global gene expression profile in patients with very early diffuse systemic sclerosis (SSc). Methods: Skin biopsies were obtained from patients enrolled…
Abstract Number: 1923 • 2017 ACR/ARHP Annual Meeting
Single Cell RNA Sequencing Reveals a Signature of Endothelial Injury in Scleroderma Skin
Sokratis Apostolidis¹, Giuseppina Stifano², Tracy Tabib³, Lisa Rice², Christina Morse³, Bashar Kahaleh⁴ and Robert A. Lafyatis³, ¹Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA, ²Boston University School of Medicine, Boston, MA, ³Medicine, Rheumatology, University of Pittsburgh Medical Center, Pittsburgh, PA, ⁴Medicine/Rheumatology, University of Toledo, Toledo, OH
Background/Purpose: Vascular injury is a hallmark event in the pathogenesis of Systemic Sclerosis (SSc). Endothelial dysfunction happens early in the course of the disease and…
Abstract Number: 6L • 2016 ACR/ARHP Annual Meeting
Myeloablative Autologous Transplantation of CD34+ -Selected Hematopoietic Stem Cells (HSCT) Vs Monthly Intravenous Cyclophosphamide (CYC) for Severe Scleroderma with Internal Organ Involvement: Outcomes of a Randomized North American Clinical Trial
Keith Sullivan¹, Lynette Keyes-Elstein², Peter McSweeney³, Ashley Pinckney², Beverly Welch⁴, Maureen D Mayes⁵, Richard Nash³, Leslie J. Crofford⁶, Sharon Castina², Linda Griffith⁷, Ellen Goldmuntz⁸ and Daniel E. Furst⁹, ¹Duke University, Durham, NC, ²Rho, Inc, Chapel Hill, NC, ³Colorado Blood Cancer Institute, Denver, CO, ⁴6610 Rockledge Dr., NIAID/NIH, Bethesda, MD, ⁵Department of Internal Medicine - Rheumatology, University of Texas-McGovern Medical School, Houston, TX, ⁶Medicine, Vanderbilt University Medical Center, Nasville, TN, ⁷NIAID, NIH, Bethesda, MD, ⁸NIAID, National Institutes of Health, Bethesda, MD, ⁹David Geffen School of Medicine at UCLA, Los Angeles, CA
Background/Purpose: Therapeutic options for diffuse cutaneous systemic sclerosis (dcSSc) are limited. The Scleroderma: Cyclophosphamide or Transplantation (SCOT) trial was a multicenter study designed to…
Abstract Number: 825 • 2016 ACR/ARHP Annual Meeting
KL-6 and Not CCL-18 Is a Predictor of Early Progression in Systemic Sclerosis Related Interstitial Lung Disease
Gloria Salazar¹, Masataka Kuwana², Minghua Wu¹, Jun Ying¹, Julio Charles³, Maureen D Mayes¹ and Shervin Assassi¹, ¹Department of Internal Medicine - Rheumatology, University of Texas-McGovern Medical School, Houston, TX, ²Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan, ³Internal Medicine-Rheumatology, University of Texas-McGovern Medical School, Houston, TX
Background/Purpose: Pneumoproteins are attractive biomarker candidates in systemic sclerosis (SSc) related interstitial lung disease (ILD) because they are easily obtainable and lung-specific. KL-6 and CCL-18…

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