Abstracts tagged "Scleroderma"

Abstract Number: 1539 • ACR Convergence 2022
Gene Expression Meta-Analysis Reveals Aging and Cellular Senescence Signatures in Scleroderma-associated Interstitial Lung Disease
Monica Yang¹, Seoyeon Lee², Jessica Neely³, Marina Sirota² and Paul Wolters², ¹UCSF, SF, CA, ²UCSF, SF, ³UCSF Benioff Children's Hospital, San Francisco, CA
Background/Purpose: Systemic Sclerosis (SSc) is an autoimmune disease characterized by diffuse fibrosis and vasculopathy that has potential to affect nearly every organ system. Interstitial lung…
Abstract Number: 2170 • ACR Convergence 2022
Validation of Ranked Composite Important Difference (RCID) Score in Early Diffuse Cutaneous Systemic Sclerosis
Francesco Del Galdo¹, Suiyuan Huang², Lesley-Anne Bissell³, sindhu johnson⁴, Daniel Furst⁵ and Dinesh Khanna⁶, ¹Leeds Institute of Rheumatic and Musculoskeletal Medicine, Faculty of Medicine and Health and NIHR Biomedical Research Centre, University of Leeds, Leeds, United Kingdom, ²University of Michigan, Ann Arbor, MI, ³University of Leeds, Leeds, United Kingdom, ⁴Rheumatology, Department of Medicine, Schroeder Arthritis Institute, Toronto Western Hospital, Mount Sinai Hospital; Institute of Health Policy, Management, and Evaluation, University of Toronto, Toronto, ON, Canada, ⁵University of California Los Angeles, Los Angeles, CA, ⁶Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI
Background/Purpose: The Ranked Composite Important Difference (RCID) in diffuse cutaneous Systemic Sclerosis ( dcSSc) is a clinically and patient meaningful composite score for outcome of…
Abstract Number: 1052 • ACR Convergence 2022
Short-term Safety of COVID-19 Vaccination in Systemic Sclerosis Patients: Report from a Global Patient-Reported E-survey
Darpan Thakare¹, Naveen R¹, John Pauling², Sakir Ahmed³, Chris Wincup⁴, Nicolletta Del Papa⁵, Gianluca Sambataro⁶, Fabiola Atzeni⁷, Marcello Govoni⁸, SIMONE PARISI⁹, Elena Bartoloni Bocci¹⁰, Gian Domenico Sebastiani¹¹, Enrico Fusaro¹², Marco Sebastiani¹³, Luca Quartuccio¹⁴, Franco Franceschini¹⁵, Pier Paolo Sainaghi¹⁶, Giovanni Orsolini¹⁷, Rossella De Angelis¹⁸, Maria Giovanna Danielli¹⁹, Vincenzo Venerito²⁰, Parikshit Sen²¹, Minchul Kim²², Abraham Edgar Gracia-Ramos²³, Akira Yoshida²⁴, James B. Lilleker²⁵, Vishwesh Agarwal²⁶, Sinan Kardes²⁷, Jessica Day²⁸, Marcin Milchert²⁹, Mrudula Joshi³⁰, Tamer A Gheita³¹, Babur Salim³², Ioannis Parodis³³, Albert Selva O’Callaghan³⁴, Elena Nikiphorou³⁵, Tulika Chatterjee²², Ai Lyn Tan³⁶, Arvind Nune³⁷, Lorenzo Cavagna³⁸, Samuel Shinjo³⁹, Nelly Ziade⁴⁰, Johannes Knitza⁴¹, Hector Chinoy⁴², Oliver Distler⁴³, Masataka Kuwana⁴⁴, Rohit Aggarwal⁴⁵, Latika Gupta⁴⁶, Vikas Agarwal¹ and Ashima Makol⁴⁷, ¹Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India, ²North Bristol NHS Trust, Bristol, United Kingdom, ³Kalinga Institute of Medical Sciences, Bhubaneswar, India, ⁴Rayne Institute, University College London, London, United Kingdom, ⁵Unità operativa complessa (UOC) Day Hospital Reumatologia via Gaetano Pini 9, Centro Specialistico Ortopedico Traumatologico, Gaetano Pini-CTO,, Milano, ⁶Medico Immunologia e reumatologia presso, Artoreuma S.R.L., Cors S, Mascalucia, ⁷Rheumatology Unit, University of Messina, Messina, Italy, ⁸S. Anna Hospital and University of Ferrara, Ferrara, Italy, ⁹Italian Society for Rheumatology, Turin, Italy, ¹⁰Rheumatology Unit. Department of Medicine, Perugia, Perugia, Italy, ¹¹U.O.C. Reumatologia, Ospedale San Camillo-Forlanini,, Roma, ¹²Azienda Ospedaliero-Universitaria Città della Salute e della Scienza di Torino, Turin, Italy, ¹³Azienda Policlinico di Modena, Modena, Italy, ¹⁴Department of Medicine (DAME), ASUFC, University of Udine, Udine, ¹⁵Department of Clinical and Experimental Sciences, ASST Spedali Civili and University of Brescia, Brescia, Italy, ¹⁶IRCAD, Interdisciplinary Research Center of Autoimmune Diseases, Novara, Italy, ¹⁷Department of Medicine, Rheumatology Unit, University of Verona, Verona, Verona, ¹⁸Rheumatology Unit, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Marche, Italy, ¹⁹Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle, Marche, Italy, ²⁰Department of Emergency and Organ Transplantations-Rheumatology Unit, University of Bari "Aldo Moro", Bari, Italy, ²¹Maulana Azad Medical College, New Delhi, India, ²²University of Illinois College of Medicine Peoria, Peoria, IL, ²³Instituto Mexicano del Seguro Social, Ciudad de México, Mexico, ²⁴Nippon Medical School Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan, ²⁵The University of Manchester, Manchester, United Kingdom, ²⁶Mahatma Gandhi Missions Medical College, Lucknow, India, ²⁷Istanbul University, Istanbul, Turkey, ²⁸Walter and Eliza Hall Institute, Melbourne, Australia, ²⁹Pomeranian Medical University in Szczecin, Szczecin, Poland, ³⁰Byramjee Jeejeebhoy Government Medical College and Sassoon General Hospitals, Pune, India, ³¹Rheumatology Department, Faculty of Medicine, Cairo University, Cairo, Egypt, ³²Fauji foundation hospital Rawalpindi, Rawalpindi, Pakistan, ³³Karolinska Institutet, Stockholm, Sweden, ³⁴Hospital Universitari Vall d'Hebron, Barcelona, Spain, ³⁵Leiden University Medical Center & King's College London, London, United Kingdom, ³⁶University of Leeds, Leeds, United Kingdom, ³⁷Southport and Ormskirk Hospital NHS Trust, Southport, United Kingdom, ³⁸Università di Pavia, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy, Pavia, Italy, ³⁹Faculdade de Medicina FMUSP, Universidade de São Paulo, São Paulo, Brazil, ⁴⁰Saint-Joseph University, Beirut, Lebanon, ⁴¹Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg and Universitätsklinikum Erlangen, 91054 Erlangen, Germany; Deutsches Zentrum Immuntherapie, Friedrich-Alexander-UniversityErlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, ⁴²The University of Manchester, Sale, United Kingdom, ⁴³Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ⁴⁴Nippon Medical School Graduate School of Medicine, Tokyo, Japan, ⁴⁵Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, ⁴⁶Royal Wolverhampton Trust, Wolverhampton/University of Manchester, United Kingdom, ⁴⁷Mayo Clinic, Rochester, MN, Rochester, MN
Background/Purpose: Dedicated data on COVID-19 vaccine-related adverse events (ADEs) in patients with systemic sclerosis (SSc) is limited. Therefore, we aimed to compare all patient-reported ADEs…
Abstract Number: 1181 • ACR Convergence 2022
Hippo Pathway Effectors Promote and Maintain Myofibroblast Differentiation and Endothelial-to-mesenchymal Transition in Systemic Sclerosis
Feiyang Ma¹, Pei-Suen Tsou¹, Grace Hile¹, John Varga¹, J. Michelle Kahlenberg¹, Allison Billi¹, Johann Gudjonsson¹ and Dinesh Khanna², ¹University of Michigan, Ann Arbor, MI, ²Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI
Background/Purpose: Systemic sclerosis (SSc) is a devastating autoimmune disease characterized by excessive production and accumulation of extracellular matrix molecules leading to fibrosis of skin and…
Abstract Number: 1622 • ACR Convergence 2022
TNF Receptor 1 Drives Murine Pulmonary Arterial Hypertension and Is Characterized by Loss of Capillary Endothelial Cells and Pericytes, Smooth Muscle Cell Proliferation, and Alterations in Fibroblast Phenotype
Stacey Duemmel¹, Marc Nuzzo¹, Soumyaroop Bhattacharya¹, Qingfu Xu¹, Amy Mohan¹ and Benjamin Korman², ¹URMC, Rochester, NY, ²University of Rochester, Rochester, NY
Background/Purpose: We previously demonstrated that TNF-transgenic (TNF-Tg) mice have findings consistent with connective-tissue disease associated pulmonary arterial hypertension (CTD-PAH), and that this pathology is mediated…
Abstract Number: 2171 • ACR Convergence 2022
Serum IFN Score Predicts Long Term Outcome in Limited Cutaneous Systemic Sclerosis
Ranjitha Karanth¹, Giuseppina Abignano², Vishal Kakkar¹, Rebecca Ross¹, Chris Denton³ and Francesco Del Galdo⁴, ¹University of Leeds, Leeds, United Kingdom, ²Istituto Reumatologico Lucano (IReL), Azienda Ospedaliera Regionale San Carlo, Potenza, Italy, ³University College London, London, United Kingdom, ⁴Leeds Institute of Rheumatic and Musculoskeletal Medicine, Faculty of Medicine and Health and NIHR Biomedical Research Centre, University of Leeds, Leeds, United Kingdom
Background/Purpose: Type-I Interferon pathway activation has been associated with severity and progression of diffuse cutaneous SSc (dcSSc). The role of type-I Interferons (IFNs) in limited…
Abstract Number: 1054 • ACR Convergence 2022
Distinct Differences in Complement Perturbation Between Scleroderma-related Interstitial Lung Disease with and Without Emphysema
Claire Beesley¹, Alice Cole², Nina Goldman¹, Odewale Oluwakorede¹, Joseph Barnett², David Abraham¹, Chris Denton¹, Rizgar Mageed³ and Voon Ong⁴, ¹University College London, London, United Kingdom, ²Royal Free Hospital, London, United Kingdom, ³Experimental Medicine and Rheumatology, William Harvey Research Institute, Queen Mary University, London, United Kingdom, ⁴UCL Medical School Royal Free Campus, London, United Kingdom
Background/Purpose: Aberrant complement activation is associated with autoimmune diseases including systemic sclerosis (SSc). As a leading cause of death in SSc, interstitial lung disease (ILD)…
Abstract Number: 1183 • ACR Convergence 2022
Functionally Distinct Fibroblast Populations in Systemic Sclerosis Skin Revealed by Explant Tissue Characterization and Single-cell RNAseq
Kristina Clark¹, Alice Cole², Shiwen Xu¹, Voon Ong³, Christopher Buckley⁴ and Chris Denton¹, ¹University College London, London, United Kingdom, ²Royal Free Hospital, London, United Kingdom, ³UCL Medical School Royal Free Campus, London, United Kingdom, ⁴University of Oxford, Oxford, United Kingdom
Background/Purpose: Traditional studies of skin fibroblasts in SSc focus on the early "migratory" fibroblast population derived from explant culture of skin biopsies. Single-cell RNAseq has…
Abstract Number: 1657 • ACR Convergence 2022
Extreme Phenotype Approach Identifies Rare Variants in Systemic Sclerosis and Dermatomyositis Patients with Severe Calcinosis
Srijana Davuluri¹, Urvashi Kaundal², Christian Lood³, Puneet Kapoor¹, Yumeko Kawano⁴, Stefania Dell'Orso⁵, Zuoming Deng⁶, Zsuzsanna McMahan⁷, Ami Shah⁷, Laura Hummers⁸, Daniel Kastner⁹, Fredrick Wigley¹⁰, David Fiorentino¹¹, Pravitt Gourh¹² and Lorinda Chung¹¹, ¹Stanford University School of Medicine, Palo Alto, CA, ²National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ³Division of Rheumatology, University of Washington, Seattle, WA, ⁴Brigham and Women's Hospital, Boston, MA, ⁵NIH, Bethesda, MD, ⁶National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ⁷Johns Hopkins Rheumatology, Baltimore, MD, ⁸Johns Hopkins Univerisity, Baltimore, MD, ⁹National Human Genome Research Institute, Bethesda, MD, ¹⁰Johns Hopkins University, Baltimore, MD, ¹¹Stanford University, Stanford, CA, ¹²National Institutes of Health, Bethesda, MD
Background/Purpose: Calcinosis, deposition of insoluble calcium salts in skin and subcutaneous tissues, affects up to 40% of systemic sclerosis (SSc) patients, and up to 20%…
Abstract Number: 2173 • ACR Convergence 2022
Rheumatology Telemedicine Consults: Impact of Demographic Variability on Patient Perception in Systemic Sclerosis versus Rheumatoid Arthritis Pts
Yossra Suliman¹, Julia mumper², Gary Feldman³, M. Cameron Hay⁴ and Daniel Furst⁵, ¹Assiut University Hospital, Assiut, Egypt, ²Miami University, Miami, OH, ³Pacific Arthritis, Los Angeles, CA, ⁴Miami University (Ohio), Oxford, OH, ⁵University of California Los Angeles, Los Angeles, CA
Background/Purpose: In the era of emerging need to utilize remote technology-based health care services due to the COVID-19 pandemic, we wanted to evaluate patient satisfaction…
Abstract Number: 1055 • ACR Convergence 2022
Associations of Potential Risk Factors with Severe Outcomes in Scleroderma Renal Crisis: Results from a Single Center Study
Alex Luta¹, Saloni Godbole¹ and Virginia Steen², ¹Medstar Georgetown University Hospital, Washington, DC, ²Georgetown University School of Medicine, Washington, DC
Background/Purpose: Scleroderma Renal Crisis (SRC) is a severe and life-threatening complication of Systemic Sclerosis (SSc). Given the rare nature of SSc, it is challenging to…
Abstract Number: 1184 • ACR Convergence 2022
Intracellular Calcium Signals Regulate the Pro-fibrotic Phenotype of Scleroderma Fibroblasts via Calcium/calmodulin-dependent Kinase II
Sirapa Vichaikul¹, William Brodie², Megan Mattichak², Qi Wu², Dinesh Khanna² and Eliza Pei-Suen Tsou², ¹Michigan Medicine, Howell, MI, ²University of Michigan, Ann Arbor, MI
Background/Purpose: We performed an in-depth analysis of the involvement of histone reader bromodomain extra-terminal proteins (BETs) in scleroderma (SSc) fibrosis and showed that JQ1, a…
Abstract Number: 1658 • ACR Convergence 2022
Association Between Anti-Sjögren Syndrome/Scleroderma Autoantigen 1 (SSSCA1) Antibodies and Cancer in Systemic Sclerosis
Rachel Wallwork¹, Livia Casciola-Rosen¹ and Ami Shah², ¹Johns Hopkins University, Baltimore, MD, ²Johns Hopkins Rheumatology, Baltimore, MD
Background/Purpose: We sought to examine the association between anti-Sjögren syndrome/scleroderma autoantigen 1 (SSSCA1) antibody and cancer in systemic sclerosis (SSc). We also describe the frequency…
Abstract Number: 2174 • ACR Convergence 2022
Influence of Immunosuppressive Therapy on Gastrointestinal Symptoms in Patients with Systemic Sclerosis
Lea Stamm¹, Alexandru Garaiman¹, Norina Zampatti¹, Mike Becker¹, Cosimo Bruni¹, Rucsandra Dobrota¹, Muriel Elhai¹, Sherif Ismail², Suzana Jordan¹, Aurora Tatu³, Oliver Distler¹ and Carina Mihai¹, ¹Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ²Department of Internal Medicine, National Research Center, Cairo, Egypt, ³Department of Gastroenterology, University Hospital Zurich, University of Zurich, Zürich, Switzerland
Background/Purpose: The gastrointestinal (GI) tract is frequently affected in systemic sclerosis (SSc), leading to considerable morbidity and even mortality. While important progress has been made…
Abstract Number: 1056 • ACR Convergence 2022
Clinical Characterization of a Cohort of Patient with Sclerotic-type Chronic Graft-versus-host Disease with Fascial/joint Involvement
Cristina Hidalgo¹, Concepción Roman², Olga Compan³, Sonia Pastor Navarro⁴, Marta Ibañez⁵, Belen Miguel⁶, Maria Dolores Sanchez⁷, Lucía Pantoja⁸, Carlos Montilla⁵ and Lucía López-Corral², ¹Hospital Universitario Salamanca, Salamanca, Spain, ²Universitary Hospital, Salamanca, Spain, ³Complejo asistencial Universitario de Palencia, Carbajosa De La Sagrada, Spain, ⁴Hospital Universitario de Salamanca, Salamanca, Spain, ⁵Complejo asistencial Universitario de Salamanca, Salamanca, Spain, ⁶University Hospital, Salamanca, Spain, ⁷Universitary Hospital, Valladolid, Spain, ⁸Complejo Asistencial Hospital General, Segovia, Spain
Background/Purpose: Graft-versus-host disease (GVHD) is the most frequent complication after allogeneic hematopoietic cell transplantation (allo-HCT). Its chronic form usually involves a multisystemic syndrome that reflects…

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