ACR Meeting Abstracts

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Abstracts tagged "Systemic sclerosis"

  • Abstract Number: 0716 • ACR Convergence 2021

    Marked Capillary Basement Membrane Reduplication Is the Hallmark Histopathological Feature of Scleromyositis

    Benjamin Ellezam1, Yves Troyanov2, Valérie Leclair3, Imane Bersali4, Margherita Giannini4, Sabrina Hoa5, Josiane Bourré-Tessier5, Minoru Satoh6, Marvin Fritzler7, Béatrice Lannes8, Jean-Luc Senécal5, Marie Hudson9, Alain Meyer4 and Océane Landon-Cardinal5, 1Division of Pathology, CHU Sainte-Justine; Department of Pathology and Cell Biology, Université de Montréal, Montréal, QC, Canada, 2Division of Rheumatology, Hôpital du Sacré-Coeur; Department of Medicine, Université de Montréal, Montréal, QC, Canada, 3Division of Rheumatology, Jewish General Hospital; Department of Medicine, McGill University, Montréal, QC, Canada, 4Service de physiologie- explorations fonctionnelles musculaire, service de rhumatologie et Centre de références des maladies autoimmunes rares, Hôpitaux universitaires de Strasbourg., Strasbourg, France, 5Division of Rheumatology, Centre hospitalier de l'Université de Montréal; Department of Medicine, Université de Montréal, Montréal, QC, Canada, 6Department of Clinical Nursing, University of Occupational and Environmental Health, Kitakyushu, Japan, 7University of Calgary, Calgary, AB, Canada, 8Service de Pathologie, Hôpitaux Universitaires de Strasbourg, Strasbourg, France, 9McGill University, Montréal, QC, Canada

    Background/Purpose: Scleromyositis (SM) is an emerging subset of autoimmune myositis (AIM) in patients with features of systemic sclerosis (SSc). SM patients may present without characteristic…
  • Abstract Number: 1366 • ACR Convergence 2021

    The Use of Lipid-Lowering Agents in Systemic Sclerosis: Is There a Relationship with Prevalence of Digital Ulcers and Overall Survival?

    Olivia Sallis1, Dylan Hansen2, Kathleen Morrisroe3, Wendy Stevens3, Mandana Nikpour4 and Susanna Proudman5, 1Royal Adelaide Hospital, Adelaide, Australia, 2St. Vincent’s Hospital Melbourne, Victoria, Australia, Adelaide, Australia, 3St Vincent's Hospital, Melbourne, Australia, 4University of Melbourne at St Vincent's Hospital, Melbourne, Australia, 5University of Adelaide, Medindie, Australia

    Background/Purpose: Digital ulcers (DU) are common in systemic sclerosis (SSc). Vasodilator therapies have limited benefit in prevention and promoting healing. Lipid-lowering agents (LLAs) such as…
  • Abstract Number: 1435 • ACR Convergence 2021

    Identification of Distinct Fibroblast Populations in Systemic Sclerosis 3D Skin Tissues with Single Cell Omics

    Noelle Kosarek1, Heetaek Yang2, Fred W. Kolling3, Tamar Abel4, Mengqi Huang5, Avi Smith6, Jonathan Garlick6, Patricia A. Pioli2 and Michael L. Whitfield7, 1Dartmouth Geisel School of Medicine, Hartford, VT, 2Geisel School of Medicine, Hanover, NH, 3Centers for Quantitative Biology, Hanover, 4Geisel School of Medicine, West Lebanon, NH, 5University of Pittsburgh, Pittsburgh, PA, 6Tufts University School of Dental Medicine, Boston, MA, 7Geisel School of Medicine, Lebanon, NH

    Background/Purpose: Systemic sclerosis (SSc) is a rare autoimmune disease characterized by skin and internal organ fibrosis, vascular abnormalities, and autoantibody formation. Single cell genomics studies…
  • Abstract Number: 1848 • ACR Convergence 2021

    Untangling the Gut: A Phenome-Wide Association Study of Drugs and Diseases with Gastrointestinal Dysfunction in Systemic Sclerosis

    Rory Maclean1, Fiza Ahmed2, Voon Ong3, Charles Murray4 and Christopher Denton5, 1Royal Free Hospital & University College London, London, United Kingdom, 2Royal Free Hospital, Ilford, United Kingdom, 3University College London Medical School Royal Free Campus, London, United Kingdom, 4Royal Free Hospital, London, United Kingdom, 5University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, United Kingdom

    Background/Purpose: Gastrointestinal dysfunction (SSc-GI) is a significant burden to patients with systemic sclerosis (SSc), particularly in those with longstanding disease. The management of SSc-GI is…
  • Abstract Number: 0393 • ACR Convergence 2021

    Severity and Impact of Gastrointestinal Symptoms in Patients with SSc-ILD Treated with Nintedanib: Data from SENSCIS-ON

    Dinesh Khanna1, Elizabeth Volkmann2, Kristin B Highland3, Yannick Allanore4, Stéphane Jouneau5, James R Seibold6, Alexandra James7, Margarida Alves8 and Oliver Distler9, 1University of Michigan, Ann Arbor, MI, 2Department of Medicine, Division of Rheumatology, University of California, David Geffen School of Medicine, Los Angeles, CA, 3Cleveland Clinic, Cleveland, OH, 4Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, 5Department of Respiratory Medicine, Competences Centre for Rare Pulmonary Diseases, Univ Rennes, CHU Rennes, Inserm, EHESP, Irset (Institut de recherche en santé, environnement et travail) - UMR_S 1085, F-35000 Rennes, France, Rennes, France, 6Scleroderma Research Consultants LLC, Aiken, SC, 7Elderbrook solutions GmbH, Bietigheim-Bissingen, Germany, Bietigheim-Bissingen, Germany, 8Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, Ingelheim, Germany, 9Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich/University of Zurich, Zurich, Switzerland

    Background/Purpose: Gastrointestinal (GI) involvement is a common manifestation of systemic sclerosis (SSc) and a frequent side-effect of drugs used to treat SSc. In the SENSCIS…
  • Abstract Number: 0498 • ACR Convergence 2021

    Background Mycophenolate (MMF) Treatment Is Associated with Improved Outcomes in a Phase 3 Trial of Lenabasum in Diffuse Cutaneous Systemic Sclerosis (dcSSc)

    Robert Spiera1, Dan Furst2, Tracy Frech3, Masataka Kuwana4, Laura Hummers5, Wendy Stevens6, Suzanne Kafaja7, Eun bong Lee8, Scott Constantine9, Nancy Dgetluck9, Barbara White10 and Christopher Denton11, 1Hospital for Special Surgery, New York, NY, 2University of California Los Angeles, Los Angeles, CA, 3University of Utah, Salt Lake City, UT, 4Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, 5Johns Hopkins Univerisity, Baltimore, MD, 6St Vincent's Hospital, Melbourne, Australia, 7University of California Los Angeles Division of Rheumatology, Los Angeles, CA, 8Seoul National University Hospital, Seoul, Republic of Korea, 9Corbus Pharmaceuticals, Inc., Norwood, MA, 10Corbus Pharmaceuticals, Norwood, MA, 11University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, United Kingdom

    Background/Purpose: Treatment of dcSSc is evolving, with limited information on relative efficacies of different immunosuppressive therapies (IST). Our hypothesis was that patients on MMF have…
  • Abstract Number: 0764 • ACR Convergence 2021

    Patients with Juvenile Systemic Sclerosis Have a Distinct Pattern of Organ Involvement: Results from the Juvenile Systemic Sclerosis 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: 1367 • ACR Convergence 2021

    Associations of Esophageal Dysmotility Patterns with Extra-intestinal Features in Patients with Systemic Sclerosis

    Ana Tucker1, Jamie Perin2, Elizabeth Volkmann3, Ami Shah4, John Pandolfino5 and Zsuzsanna McMahan4, 1Medical University of South Carolina, Charleston, SC, 2Johns Hopkins University, Baltimore, MD, 3Department of Medicine, Division of Rheumatology, University of California, David Geffen School of Medicine, Los Angeles, CA, 4Johns Hopkins Rheumatology, Baltimore, MD, 5Northwestern University, Chicago, IL

    Background/Purpose: The gastrointestinal (GI) tract is the most commonly affected internal organ in systemic sclerosis (SSc). SSc GI disease is heterogeneous, with some patients experiencing…
  • 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…
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