Abstracts tagged "Systemic sclerosis"

Abstract Number: 1436 • ACR Convergence 2021
Expanded PD-1hi CXCR5- HLA-DR+ T Cells Is Associated with Interstitial Lung Disease in Systemic Sclerosis
Mehreen Elahee¹, Alisa Mueller², Runci Wang³, Ye Cao², Andrea Fava⁴, Paul Dellaripa², Francesco Boin⁵ and Deepak Rao², ¹Brigham and Women's Hospital, Natick, MA, ²Brigham and Women's Hospital, Boston, MA, ³Brigham and Women’s Hospital, Boston, MA, ⁴Johns Hopkins University, Baltimore, MD, ⁵Cedars-Sinai Medical Center, West Hollywood, CA
Background/Purpose: Interstitial lung disease (ILD) is the major cause of morbidity and mortality in systemic sclerosis (SSc), yet there are few biomarkers to identify pathologic…
Abstract Number: 1849 • ACR Convergence 2021
False Positive Anti-Topoisomerase I (Scl-70) Antibody Results: A Case Series from a Scleroderma Referral Center
Brian Lam¹, Rana Taherian¹, Julio Charles¹, Maureen Mayes², Shervin Assassi³ and Brian Skaug⁴, ¹Division of Rheumatology, University of Texas McGovern Medical School at Houston, Houston, TX, ²Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, TX, ³University of Texas McGovern Medical School at Houston, Houston, TX, ⁴University of Texas McGovern Medical School Houston, Houston, TX
Background/Purpose: Systemic sclerosis (SSc) is a progressive autoimmune disease with high morbidity and mortality, making early diagnosis and management critical. Anti-Topoisomerase I antibody (anti-Topo I,…
Abstract Number: 0394 • ACR Convergence 2021
Hyperspectral Imaging in Systemic Sclerosis-Raynaud Phenomenon
Akash Gupta¹, Shannon Teaw², Alyssa Williams², F. Perry Wilson³, Brandon Sumpio⁴, Bauer Sumpio⁴ and Monique Hinchcliff⁵, ¹Department of Medicine, Yale University School of Medicine, New Haven, CT, ²Section of Rheumatology, Allergy & Immunology, Department of Medicine, Yale School of Medicine, New Haven, CT, ³Section of Nephrology, Department of Medicine, Yale School of Medicine, New Haven, CT; Department of Medicine Clinical and Translational Research Accelerator Yale School of Medicine, New Haven, CT, ⁴Division of Vascular Surgery, Department of Surgery, Yale School of Medicine, New Haven, CT, ⁵Yale School of Medicine, Westport, CT
Background/Purpose: Raynaud phenomenon (RP), a microcirculatory, vasospastic disorder, may be primary or secondary to an autoimmune disease [e.g., an early indicator of systemic sclerosis (SSc)].…
Abstract Number: 0499 • ACR Convergence 2021
Preliminary Assessment of Internal Reliability and Construct Validity of Long and Short-form Assessment of Systemic Sclerosis-associated RAynaud’s Phenomenon (ASRAP) Questionnaires
John Pauling¹, Lan Yu², Christopher Denton³, Tracy Frech⁴, Ariane Herrick⁵, Laura Hummers⁶, Lesley Ann Saketkoo⁷, Ami Shah⁸, Dinesh Khanna⁹ and Robyn Domsic², ¹Royal National Hospital for Rheumatic Diseases, Bath, United Kingdom, ²University of Pittsburgh, Pittsburgh, PA, ³University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, United Kingdom, ⁴University of Utah, Salt Lake City, UT, ⁵University of Manchester, Salford, United Kingdom, ⁶Johns Hopkins Univerisity, Baltimore, MD, ⁷University Medical Center - Comprehensive Pulmonary Hypertension Center, New Orleans, LA, ⁸Johns Hopkins Rheumatology, Baltimore, MD, ⁹University of Michigan, Ann Arbor, MI
Background/Purpose: The Assessment of Systemic sclerosis-associated RAynaud’s Phenomenon (ASRAP) questionnaire is a novel patient-reported outcome instrument devised to assess the severity and impact of SSc-RP.…
Abstract Number: 0765 • ACR Convergence 2021
Male Juvenile Systemic Sclerosis Patients Have More Severe Disease: Results from the International Juvenile Scleroderma Inception Cohort
Ivan Foeldvari¹, Jens Klotsche², Ozgur Kasapcopur³, Amra Adrovic⁴, Kathryn Torok⁵, Maria Terreri⁶, Ana Paula Sakamoto⁷, Flavio Sztajnbok⁸, Brian Feldman⁹, Valda Stanevicha¹⁰, Jordi Anton¹¹, Raju Khubchandani¹², Ekaterina Alexeeva¹³, Sindhu Johnson¹⁴, Maria Martha Katsicas¹⁵, Sujata Sawhney¹⁶, Vanessa Smith¹⁷, Simone Appenzeller¹⁸, Tadej Avcin¹⁹, Mikhail Kostik²⁰, Thomas Lehman²¹, Edoardo Marrani²², Dieneke Schonenberg-Meinema²³, Walter Alberto Sifuentes-Giraldo²⁴, Natalia Vasquez-Canizares²⁵, Mahesh Janarthanan²⁶, Hana Malcova²⁷, Monika Moll²⁸, Dana Nemcova²⁹, Anjali Patwardhan³⁰, Maria José Santos³¹, cristina battagliotti³², Lillemor Berntson³³, Blanca Elena Rios Gomes Bica³⁴, Jürgen Brunner³⁵, Rolando Cimaz³⁶, Patricia Costa Reis³⁷, Despina Eleftheriou³⁸, Liora Harel³⁹, Gerd Horneff⁴⁰, Daniela Kaiser⁴¹, Tilmann Kallinich⁴², Dragana Lazarevic⁴³, Kirsten Minden², Susan Nielsen⁴⁴, Farzana Nuruzzaman⁴⁵, Siri Opsahl Hetlevik⁴⁶, Yosef Uziel⁴⁷ and Nicola Helmus⁴⁸, ¹Hamburger Zentrum fuer Kinder- und Jugendrheumatologie, Hamburg, Germany, ²German Rheumatism Research Center, Berlin, Germany, ³Istanbul University-Cerrahpasa, Cerrahpasa Medical School, İstanbul, Turkey, ⁴Cerrahpasa Medical School, Istanbul, Turkey, ⁵University of Pittsburgh, Pittsburgh, PA, ⁶UNIFESP, São Paulo, Brazil, ⁷Federal University of So Paulo (UNIFESP), São Paulo, Brazil, ⁸UFRJ/UERJ, Rio de Janeiro, Brazil, ⁹The Hospital for Sick Children, Toronto, ON, Canada, ¹⁰Paediatric Rheumatology International Trials Organisation (PRINTO), Riga, Latvia, ¹¹Hospital Sant Joan de Deu, University of Barcelona, Barcelona, Spain, ¹²Jaslok Hospital and Research Center, Mumbai, India, ¹³Scientific Center of Children Health of RAMS, Moscow, Russia, ¹⁴University of Toronto, Toronto, ON, Canada, ¹⁵Hospital de Pediatria J.P Garrahan, Buenos Aires, Argentina, ¹⁶Pediatric Rheumatology Department, Institute of Child Health, Sir Gangarm Hospital, New Delhi, India, ¹⁷Department of Rheumatology and Internal Medicine, Ghent University Hospital, Ghent, Belgium, ¹⁸Hospital das Clínicas da Universidade Estadual de Campinas, Campinas, Brazil, ¹⁹University Medical Center Ljubljana, Ljubljana, Slovenia, ²⁰Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ²¹Hospital for Special Surgery, New York, NY, ²²University of Florence, Florence, Italy, ²³Emma Children’s Hospital, Amsterdam, Netherlands, ²⁴Hospital Universitario Ramón y Cajal, Madrid, Spain, ²⁵Children’s Hospital at Montefiore, Bronx, NY, ²⁶Sri Ramachandra University, Chennai, India, ²⁷Motol University Hospital, Prague, Czech Republic, ²⁸University Tuebingen, Tübingen, Germany, ²⁹Charles University, Prague, Czech Republic, ³⁰University of Missouri-Columbia, Columbia, MO, ³¹Rheumatology Department, Hospital Garcia de Orta, Almada, Portugal, ³²Hospital de Niños Dr Orlando Alassia, Santa Fe, Argentina, ³³Uppsala University, Uppsala, Sweden, ³⁴Faculdade de Medicina, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, ³⁵Medical University Innsbruck, Innsbruck, Austria, ³⁶ASST Gaetano Pini-CTO, Università degli Studi di Milano, Milan, Italy, ³⁷Hospital de Santa Maria, Lisbon, Portugal, ³⁸Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, ³⁹Scheiders Children Medical Center of Israel, Petah-Tiqva, Israel, ⁴⁰Paediatric Rheumatology International Trials Organisation (PRINTO), Sankt Augustin, Germany, ⁴¹Luzerner Kantonsspital, Kinderspital, Luzern, Switzerland, ⁴²Charité University Medicine, Nuremberg, Germany, ⁴³Dept of Pediatric Rheumatology and Immunology Clinical Center Nis, Nis, Serbia, ⁴⁴Rigshospitalet, Copenhagen, Denmark, ⁴⁵Stony Brook Children's Hospital, Stony Brook, NY, ⁴⁶Oslo University Hospital, Oslo, Norway, ⁴⁷Meir Medical Center, Kfar Saba, Israel, ⁴⁸Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany
Background/Purpose: Juvenile systemic sclerosis (jSSc) is a rare disease with a prevalence of around 3 in 1,000,000 children. To better capture the clinical manifestations of…
Abstract Number: 1368 • ACR Convergence 2021
Characterising Exercise Capacity in Systemic Sclerosis Using Cardiac Magnetic Resonance Imaging, Skeletal Muscle Imaging and Cardiopulmonary Exercise Testing
Laura Ross¹, Benedict Costello², Dylan Hansen³, Anniina Lindqvist², Zoe Brown⁴, Andrew Burns⁴, David Prior⁴, Wendy Stevens⁴, Marcus Pianta⁴, Warren Perera⁴, Andre La Gerche² and Mandana Nikpour¹, ¹University of Melbourne at St Vincent's Hospital, Melbourne, Australia, ²Baker Heart and Diabetes Institute, Melbourne, Australia, ³St. Vincent’s Hospital Melbourne, Victoria, Australia, Adelaide, Australia, ⁴St Vincent's Hospital, Melbourne, Australia
Background/Purpose: Impaired exercise capacity contributes to functional impairment, negatively impacting individuals’ quality of life. Functional impairment is notable from early in the disease course of…
Abstract Number: 1437 • ACR Convergence 2021
Fat and Fibrosis: A Novel Developmental Gene in Systemic Sclerosis
Nancy Wareing¹, Brian Skaug², Minghua Wu³, Scott Collum¹, Cory Wilson¹, Lucy Revercomb⁴, Marka Lyons⁵, Weizhen Bi⁶, Tingting Mills¹, Julio Charles⁵, Shervin Assassi¹ and Harry Karmouty-Quintana¹, ¹University of Texas McGovern Medical School at Houston, Houston, TX, ²University of Texas McGovern Medical School Houston, Houston, TX, ³University of Texas Health Science Center at Houston, Houston, TX, ⁴Rice University, Houston, TX, ⁵Division of Rheumatology, University of Texas McGovern Medical School at Houston, Houston, TX, ⁶McGovern Medical School at UTHealth, Houston, TX
Background/Purpose: Early loss of skin-associated adipose tissue and concomitant replacement by extracellular matrix is a hallmark of systemic sclerosis (SSc). However, the contribution of adipose…
Abstract Number: 1850 • ACR Convergence 2021
Continued Treatment with Nintedanib in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD): Two-Year Data from SENSCIS-ON
Yannick Allanore¹, Madelon C Vonk², Oliver Distler³, Arata Azuma⁴, Maureen Mayes⁵, Martina Gahlemann⁶, Alexandra James⁷, Veronika Kohlbrenner⁸, Margarida Alves⁹, Dinesh Khanna¹⁰ and Kristin B Highland¹¹, ¹Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, ²Department of Rheumatology, Radboud University Medical Center, Nijmegen, Netherlands, ³Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich/University of Zurich, Zurich, Switzerland, ⁴Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan, ⁵Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, TX, ⁶Boehringer Ingelheim (Schweiz) GmbH, Basel, Switzerland, Basel, Switzerland, ⁷Elderbrook solutions GmbH, Bietigheim-Bissingen, Germany, Bietigheim-Bissingen, Germany, ⁸Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, CT, ⁹Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, Ingelheim, Germany, ¹⁰University of Michigan, Ann Arbor, MI, ¹¹Cleveland Clinic, Cleveland, OH
Background/Purpose: In the SENSCIS trial in patients with SSc-ILD, nintedanib reduced the rate of decline in forced vital capacity (FVC) (mL/year) over 52 weeks by…
Abstract Number: 0396 • ACR Convergence 2021
Esophageal Involvement and Gastroesophageal Reflux Disease in Patients with SSc-ILD: Data from a Sub-Study of the SENSCIS Trial
Michael Kreuter¹, Dinesh Khanna², Christopher Ryerson³, Stephen Humphries⁴, Tohru Takeuchi⁵, Mark Hamblin⁶, Dag Wormanns⁷, Carina Ittrich⁸, Frank Risse⁸, Margarida Alves⁹ and David Lynch⁴, ¹Center for Interstitial and Rare Lung Diseases, Pneumology and Respiratory Care Medicine, Thoraxklinik, University of Heidelberg, Member of the German Center for Lung Research, Heidelberg, Germany, ²University of Michigan, Ann Arbor, MI, ³Department of Medicine & Centre for Heart Lung Innovation, University of British Columbia, Vancouver, BC, Canada, Vancouver, BC, Canada, ⁴Department of Radiology, National Jewish Health, Denver, CO, ⁵Osaka Medical and Pharmaceutical University, Osaka, Japan, ⁶University of Kansas Hospital, Kansas City, KS, ⁷Evangelische Lungenklinik, Berlin, Germany, ⁸Boehringer Ingelheim Pharma GmbH & Co. KG, Biberach an der Riss, Germany, ⁹Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, Ingelheim, Germany
Background/Purpose: Upper gastrointestinal involvement such as esophageal dilation is frequently observed in patients with SSc-ILD and may be associated with the presence or severity of…
Abstract Number: 0501 • ACR Convergence 2021
Immunogenicity of a Single Dose of Covid-19 Vaccination in Patients with Systemic Sclerosis with or Without Immunosupression
Vishal Kakkar¹, Rebecca Ross², Ranjitha Karanth³, Sumit Lahiri⁴, Panji Mulipa¹, Pamela Hughes⁵, Brendan Clarke⁵, Clive Carter⁵, Mark Lobb⁵, Sinisa Savic⁶ and Francesco Del Galdo¹, ¹University of Leeds, Leeds, United Kingdom, ²LIRMM University of Leeds, Leeds, United Kingdom, ³Leeds Institute of Rheumatic and Musculoskeletal Medicine, LTHT, Leeds, United Kingdom, ⁴Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, ⁵Transplant and Cellular Immunology, Leeds, United Kingdom, ⁶Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, United Kingdom
Background/Purpose: Systemic Sclerosis (SSc) is a rare connective tissue disease with multi-systemic involvement, which at times requires the use of immunosuppressive medication. None of the…
Abstract Number: 0778 • ACR Convergence 2021
Autologous Stem Cell Transplantation with CD34-Selected Peripheral Blood Stem Cells in Patients with Treatment-Resistant Juvenile-Onset Systemic Sclerosis
Kathryn Torok¹, Franziska Rosser², Kirsten Rose-Felker¹, Vibha Sood¹, Geoffrey Kurland¹, Adam Olson¹, Meghan Frost¹, Vickie Vandergrift¹, Shawna McIntyre¹, Alisha Maslanka³, Kaila Schollaert-Fitch¹, Jessie Barnum¹ and Paul Szabolcs¹, ¹University of Pittsburgh, Pittsburgh, PA, ²UPMC Children's Hospital of Pittbsurgh, Pittsburgh, PA, ³University of Pittsburgh, Pittsburgh
Background/Purpose: Juvenile-onset systemic sclerosis (jSSc) is a rare autoimmune disease associated with life-threatening multi-organ inflammation and fibrosis. As in adults, jSSc organ involvement includes vascular,…
Abstract Number: 1370 • ACR Convergence 2021
Complement Factor D and Factor H Represent Disease and Severity Biomarkers for Systemic Sclerosis Associated Pulmonary Arterial Hypertension (SSc-PAH)
Roberta G Marangoni¹, Changyong Feng¹, Ashley Frazer-Abel², Stephen Tomlinson³, Amy Wielgosz¹, Katie Lutz⁴, Michael Pauciulo⁴, William Nichols⁴, V. Michael Holers⁵, Christopher Ritchlin⁶, R James White III¹ and Benjamin Korman¹, ¹University of Rochester, Rochester, NY, ²University of Colorado Denver, Colorado, CO, ³Medical University of South Carolina, Charleston, SC, ⁴Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁵University of Colorado, Denver, CO, ⁶Division of Allergy, Immunology, and Rheumatology, School of Medicine and Dentistry, University of Rochester Medical Center, Rochester, NY
Background/Purpose: Pulmonary arterial hypertension (PAH) is a severe vascular complication of systemic sclerosis (SSc) and a major cause of mortality. Despite significant advances in the…
Abstract Number: 1465 • ACR Convergence 2021
Trends in Adverse Pregnancy Outcomes Among Women with Systemic Sclerosis in the United States
Yumeko Kawano¹, Kathleen Kolstad², Shufeng Li¹, Julia Simard³ and Lorinda Chung¹, ¹Stanford University, Palo Alto, CA, ²University of California Los Angeles, Santa Barbara, CA, ³Stanford School of Medicine, Stanford, CA
Background/Purpose: Autoimmune connective tissue diseases disproportionately affect women of childbearing age. Systemic sclerosis (SSc) is associated with increased risk of adverse pregnancy outcomes (APO). However,…
Abstract Number: 1851 • ACR Convergence 2021
Early Intervention with Immunomodulators Leads to Better Outcomes in Patients with Systemic Sclerosis
Keina Yomono¹ and Masataka Kuwana², ¹Nippon Medical School Graduate School of Medicine, Tokyo, Japan, ²Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan
Background/Purpose: Systemic sclerosis (SSc) is a complex disease characterized by microvascular abnormalities, immune dysregulation and chronic inflammation, and subsequent excessive fibrosis of the skin and…
Abstract Number: 0397 • ACR Convergence 2021
Safety and Persistence of Monthly Intravenous Iloprost in Systemic Sclerosis
Patrícia Martins¹, Eduardo Dourado¹, João Eurico Fonseca¹, Vasco C Romão¹ and Catarina Resende², ¹Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisbon Academic Medical Centre and European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ERN-ReCONNET); Rheumatology Research Unit, Instituto de Medicina Molecular João Lobo Antunes, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal, ²Centro Hospitalar Universitário Lisboa Norte, Rheumatology Department, Lisbon, Portugal
Background/Purpose: Vasculopathy is a crucial feature of systemic sclerosis (SSc). Raynaud’s phenomenon (RP) and digital ulcers (DU) greatly impact the patients’ quality of life. Intravenous…

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