Abstracts tagged "Scleroderma, Systemic"

Abstract Number: 0682 • ACR Convergence 2024
Results from Outcomes Selection and Development of a Patient-reported Outcome Measure for a Combined Response Index for Limited Cutaneous SSc: The CRISTAL Project
Alain Lescoat¹, Yen Chen², Susan Murphy³, Nadia Vann², Neda Kortam², Rosemary Gedert², Sue Farrington⁴, Yannick Allanore⁵, David Cella⁶, Lorinda Chung⁷, Philip Clements⁸, Christopher Denton⁹, Francesco Del Galdo¹⁰, Oliver Distler¹¹, Monique Hinchcliff¹², Michael Hughes¹³, Laura Hummers¹⁴, John Pauling¹⁵, Janet Pope¹⁶, Virginia Steen¹⁷, John Varga², Peter Merkel¹⁸, Maya H. Buch¹⁹ and Dinesh Khanna², and all CRISTAL collaborators, ¹CHU Rennes - University Rennes 1, Rennes, France, ²University of Michigan, Ann Arbor, MI, ³University of Michigan, Plymouth, MI, ⁴Scleroderma & Raynaud UK, London, United Kingdom, ⁵Université Paris Cité, Paris, France, ⁶Northwestern University, Chicago, IL, ⁷Stanford University, Woodside, CA, ⁸United States, Los Angeles, CA, ⁹University College London, Northwood, United Kingdom, ¹⁰University of Leeds, Leeds, United Kingdom, ¹¹Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland, ¹²Yale School of Medicine, Westport, CT, ¹³Tameside and Glossop Integrated NHS Foundation Trust & The University of Manchester, Manchester, United Kingdom, ¹⁴Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Ellicott City, MD, ¹⁵North Bristol NHS Trust, Bristol, United Kingdom, ¹⁶University of Western Ontario, London, ON, Canada, ¹⁷Georgetown University School of Medicine, Washington, DC, ¹⁸University of Pennsylvania, Philadelphia, PA, ¹⁹Division of Musculoskeletal & Dermatological Sciences, University of Manchester, and NIHR Manchester Biomedical Research Centre, Manchester, United Kingdom
Background/Purpose: Limited cutaneous systemic sclerosis (lcSSc) is the most frequent subset of scleroderma, yet there is a paucity of outcome measures available to assess symptoms…
Abstract Number: 1580 • ACR Convergence 2024
Overt Polyautoimmunity and Its Risk Factors in a Multicenter Cohort of Systemic Sclerosis Patients
Irene Carrión-Barberà¹, Laura Tío², Laura Triginer², María Lee², Alfredo Guillén-del Castillo³, Anna Ribes², Lidia Valencia Muntalà⁴, Jordi Monfort¹, Tarek Carlos Salman Monte:⁵, Carmen PIlar Simeón-Aznar³, Anna Pros¹ and Javier Narvaez-García⁶, ¹Hospital del Mar, Barcelona, Spain, ²Hospital del Mar Research Institute, Barcelona, Spain, ³Hospital Vall d'Hebron, Barcelona, Spain, ⁴Hospital de Bellvitge, Barcelona, Spain, ⁵Hospital del Mar/Parc de Salut Mar-IMIM, Barcelona, Spain, ⁶Hospital Universitario de Bellvitge, Barcelona, Spain
Background/Purpose: Overt PolyA (the clinical coexistence of ≥2 autoimmune diseases (AID) that fulfill classification criteria), is a frequent phenomenon in systemic sclerosis (SSc). However, the…
Abstract Number: 2463 • ACR Convergence 2024
Interstitial Lung Disease in Very Early Systemic Sclerosis – a Clinical and Radiological Characterization
Sinziana Muraru¹, Christian Blüthgen², Cosimo Bruni¹, Carina Mihai¹, Anna-Maria Hoffmann-Vold³, Muriel Elhai⁴, Mike Becker⁵, Thomas Frauenfelder⁶, Oliver Distler⁷ and Rucsandra Dobrota¹, ¹University Hospital Zurich, University of Zurich, Zurich, Switzerland, ²Institute for Diagnostic and Interventional Radiology, University Hospital Zurich, Zurich, Switzerland, ³Department of Rheumatology, Oslo University Hospital, Oslo, Norway, Oslo, Norway, ⁴University Hospital zurich, Zürich, Switzerland, ⁵Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ⁶University Hospital Zurich, Zurich, Zurich, Switzerland, ⁷Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland
Background/Purpose: Interstitial lung disease (ILD) occurs in about 50% of patients with systemic sclerosis (SSc). The aims of this study were to assess the prevalence…
Abstract Number: 0683 • ACR Convergence 2024
Prognostic Value of Systemic Sclerosis-associated Primary Heart Involvement
Caya Gharibian¹, Vanessa Lupi¹, Alexander Gotschy¹, Mike Becker², Rucsandra Dobrota¹, Muriel Elhai³, Sinziana Muraru¹, Suzana Jordan¹, Anna-Maria Hoffmann-Vold⁴, Oliver Distler⁵, Robert Manka¹, Cosimo Bruni¹ and Carina Mihai¹, ¹University Hospital Zurich, University of Zurich, Zurich, Switzerland, ²Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ³University Hospital zurich, Zürich, Switzerland, ⁴Oslo University Hospital, Oslo, Norway, ⁵Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland
Background/Purpose: A definition of primary heart involvement (pHI) in systemic sclerosis (SSc) was recently developed (Bruni C et al. J Scleroderma Relat Disord. 2022;7:24-32). Cardiac…
Abstract Number: 1584 • ACR Convergence 2024
Efficacy of Tofacitinib in the Treatment of Early Diffuse Cutaneous Systemic Sclerosis: A Clinical Study in Bangladesh
Md Zahid Amin¹, Nabil Amin khan², Prof. Md. Abu Shahin², Dr. Md. Ariful Islam², Prof. Md. Nazrul Islam², Prof. Syed Atiqul Haq³, Prof. Minhaj Rahim Choudhury³ and Prof. Mohammad Mostafa Zaman⁴, ¹Central Police Hospital, Dhaka, Dhaka, Bangladesh, ²BSMMU, Dhaka, Dhaka, Bangladesh, ³Department of Rheumatology, BSMMU, Dhaka, Dhaka, Bangladesh, ⁴Department of Public Health and Informatics, BSMMU, Dhaka, Dhaka, Bangladesh
Background/Purpose: Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis, vascular abnormalities, and immune system dysregulation. Current treatment options are limited, and there…
Abstract Number: 2472 • ACR Convergence 2024
Are Anti-centromere Antibodies (aCENP) Predictive of Systemic Sclerosis Development in Patients Without Raynaud´s Phenomenon?
Andrea Alvear-Torres¹, María José Martínez-Becerra², María Carmen Vegas-Sánchez³, Sheila Recuero-Díaz¹, María Pérez-ferro⁴ and Olga Sánchez-Pernaute⁵, ¹Department of Rheumatology and Joint and Bone Research Unit. Hospital Universitario Fundación Jiménez Díaz and IIS-FJD, Madrid, Spain, ²Department of Immunology. Hospital Universitario Fundación Jiménez Díaz and IIS-FJD, Madrid, Spain, ³Hospital Universitario Fundación Jiménez Díaz and IIS-FJD, Madrid, Spain, ⁴Department of Rheumatology. Hospital Universitario Rey Juan Carlos, Madrid, Spain, ⁵Department of Rheumatology and Joint and Bone Research Unit. Hospital Universitario Fundación Jiménez Díaz and IIS-FJD. Autonomous University of Madrid, Madrid, Spain
Background/Purpose: Anti-centromere (aCENP) antibodies are considered highly specific of systemic sclerosis (SSc), typically defining a limited cutaneous phenotype, usually associated to Raynaud´s phenomenon (RP). It…
Abstract Number: L06 • ACR Convergence 2023
Improvement in Clinical and Patient-Reported Outcomes for Refractory Juvenile-Onset Systemic Sclerosis (jSSc) 6 Months to 2 Years After Autologous Stem Cell Transplantation (ASCT)
Kathryn Torok¹, Paulina Horvei¹, Franziska Rosser¹, Kirsten Rose-felker², Vibha Sood², Adam Olsen², Nicole Hogue², Vickie Vandergrift², Lauren Farver², Devin Mcguire², Jonathan Li³, Haley Havrilla², Jessie Alexander⁴, Shawna McIntyre² and Paul Szabolcs¹, ¹University of Pittsburgh; UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, ²UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, ³University of Pittsburgh Medical Center, Pittsburgh, PA, ⁴Stanford Children's Hospital, Palo Alto, CA
Background/Purpose: Juvenile-onset systemic sclerosis (jSSc) is an inflammatory, fibrotic, and vasculopathic disease that causes severe multi-organ dysfunction leading to significant morbidity and early mortality.When patients…
Abstract Number: 0638 • ACR Convergence 2023
Application of the 2022 European Society of Cardiology (ESC) Risk Assessment Model in Australian and Singaporean Systemic Sclerosis Patients with Newly Diagnosed Pulmonary Arterial Hypertension (PAH)
Zoe Brown¹, Dylan Hansen², Wendy Stevens³, Nava Ferdowsi⁴, laura ross⁵, Alannah Quinlivan⁶, Joanne Sahhar⁷, Gene-Siew Ngian⁷, Diane Apostolopoulos⁷, Jennifer G Walker⁸, Susanna Proudman⁹, Gim Gee Teng¹⁰, Andrea Hsiu Ling Low¹¹, Kathleen Morrisroe¹² and Mandana Nikpour¹³, ¹The University of Melbourne, Armadale, Australia, ²Department of Rheumatology, St Vincent’s Hospital Melbourne, Fitzroy, Australia, ³Department of Rheumatology, St Vincent's Hospital Melbourne, Fitzroy, Australia, ⁴St Vincents Hospital, Kew, Australia, ⁵St. Vincent's Hospital Melbourne, Fitzroy, Australia, ⁶St Vincent's Hospital Melbourne, Highett, Australia, ⁷Monash Health and Monash University, Clayton, Australia, ⁸Royal Adelaide Hospital, Adelaide, Australia, ⁹Royal Adelaide Hospital and University of Adelaide, Adelaide, Australia, ¹⁰Alexandra Hospital, Department of Medicine, Division of Rheumatology, National University Health System, Singapore, Singapore, ¹¹Department of Rheumatology and Immunology, Singapore General Hospital, Singapore, Singapore, ¹²The University of Melbourne at St Vincent’s Hospital Melbourne, Fitzroy, Australia, ¹³The University of Melbourne at St. Vincent’s Hospital Melbourne, Departments of Medicine and Rheumatology, Melbourne, Australia
Background/Purpose: Pulmonary arterial hypertension (PAH) patients can be stratified as low, intermediate, or high risk of 1-year mortality based on clinical, biochemical and haemodynamic prognostic…
Abstract Number: 1702 • ACR Convergence 2023
Outcomes in Systemic Sclerosis Patients Treated with Rituximab and Mycophenolate Mofetil Combination Therapy Compared to Autologous Hematological Stem Cell Transplantation
shiri keret¹, Lisa Kaly², Aniela Shouval², Tsila Zuckerman³, Israel Henig⁴, Abid Awisat⁵, Itzhak Rosner⁶, Michel Rozenbaum², Nina Boulman², Yair Molad⁷, ariela Dortort Lazar⁸, Gleb Slobodin² and Doron Rimar², ¹Bnai Zion, Atlit, Israel, ²Bnai Zion Medical Center, Haifa, Israel, ³Hematology department, Rambam Medical Center, Haifa, Israel, ⁴Stem cell transplantation unit, Hematology department, Rambam Medical Center, Haifa, Israel, ⁵Bney-Zion Medical Center, Baqa Elgharbiya, Israel, ⁶Bnai Zion Medical Center/Technion, Haifa, Israel, ⁷Rabin Medical Center, Beilinson Hospital, and Tel Aviv University, Petah-Tikva, Israel, ⁸Rabin Medical Center and Tel Aviv university, Tel Aviv, Israel
Background/Purpose: Autologous hematological stem cell transplantation (AHSCT) is a grade A therapy for early diffuse progressive systemic sclerosis (SSc), that has been validated in three…
Abstract Number: 0639 • ACR Convergence 2023
Evaluating Esophageal Dysmotility by Scintigraphy in Systemic Sclerosis: Subsets and Phenotypes
Antonio Salas¹, Lisa Yanek² and Zsuzsanna McMahan³, ¹Johns Hopkins University School of Medicine, Baltimore, MD, ²Johns Hopkins University School of Medicine Department of Medicine, Baltimore, MD, ³Johns Hopkins Rheumatology, Lutherville, MD
Background/Purpose: Gastrointestinal (GI) dysmotility affects most patients with systemic sclerosis (SSc), and the esophagus is the most commonly affected region. While most SSc patients are…
Abstract Number: 1703 • ACR Convergence 2023
Increased Mortality and Cardiovascular Events in Male Patients with Systemic Sclerosis: Left Ventricular Global Longitudinal Strain as Possible Screening Tool
Jeska de Vries-Bouwstra¹, Tea Gegenava², Federico Fortuni³, Nina Van Leeuwen⁴, Anders Tennoe⁵, Anna Maria Hoffmann-Vold⁶, Ruxandra Jurcut⁷, Adrian Giuca⁷, Laura Groseanu⁸, Felix Tanner⁹, Oliver Distler¹⁰, Jeroen Bax¹¹ and Nina Ajmone Marsan¹¹, ¹Leiden University Medical Center, Leiden, Netherlands, ²Department of Cardiology, Heart Lung Centre, Leiden University Medical Centre; Department of Internal Medicine,Tbilisi State Medical University, Tbilisi, Georgia, ³Department of Cardiology, San Giovanni Battista Hospital, Foligno, Italy, ⁴Department of Rheumatology, Leiden University Medical Centre, Leiden, Netherlands, ⁵Department of Rheumatology, Oslo University Hospital, Oslo, Norway, ⁶Oslo University Hospital, Department of Rheumatology, Oslo, Norway, ⁷Department of Cardiology, Emergency Institute for Cardiovascular Diseases “Prof .Dr. C. C. Iliescu”, University of Medicine and Pharmacy "Carol Davila", Bucharest, Romania, ⁸University of Medicine and Pharmacy "Carol Davila", Department of Internal Medicine -Rheumatology; Santa Maria Clinical Hospital, Internal Medicine and Rheumatology, Bucharest, Romania, ⁹Department of Cardiology, University Heart Centre, University Hospital Zurich, Zurich, Switzerland, ¹⁰Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ¹¹Department of Cardiology, Heart Lung Centre, Leiden University Medical Centre, Leiden, Netherlands
Background/Purpose: Systemic sclerosis (SSc) is less frequent in males, but the risk of severe outcomes is higher in males than in females(1). Seven to 30%…
Abstract Number: 0646 • ACR Convergence 2023
Racial Variability in Immune Responses Only Partially Explains Differential Systemic Sclerosis Disease Severity
Kamini Kuchinad¹, Ji Soo Kim¹, Adrianne Woods¹, Gwen Leatherman¹, Laura Gutierrez², Maureen Mayes³, Robyn Domsic⁴, Paula Ramos⁵, Richard Silver⁵, John Varga⁶, Lesley Ann Saketkoo⁷, Suzanne Kafaja⁸, Victoria Shanmugam⁹, Jessica Gordon¹⁰, Lorinda Chung¹¹, Elana Bernstein¹², Pravitt Gourh¹³, Francesco Boin¹⁴, Daniel Kastner¹⁵, Scott Zeger¹⁶, Livia Casciola-Rosen¹, Fred Wigley¹ and Ami Shah¹⁷, ¹Department of Rheumatology, Johns Hopkins Medicine, Baltimore, MD, ²Johns Hopkins Medicine, Baltimore, MD, ³Division of Rheumatology, University of Texas McGovern Medical School, Houston, TX, ⁴University of Pittsburgh, Pittsburgh, PA, ⁵Medical University of South Carolina, Charleston, SC, ⁶University of Michigan, Ann Arbor, MI, ⁷University Medical Center - Comprehensive Pulmonary Hypertension Center and ILD Clinic Programs // New Orleans Scleroderma and Sarcoidosis Patient Care & Research Centeris, New Orleans, LA, ⁸UCLA Department of Medicine, Division of Rheumatology, Los Angeles, CA, ⁹Victoria Shanmugam, MD, Great Falls, VA, ¹⁰Hospital for Special Surgery, New York, NY, ¹¹Department of Medicine, Division of Immunology and Rheumatology, Stanford University School of Medicine, Woodside, CA, ¹²Columbia University, New York, NY, ¹³National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ¹⁴Cedars-Sinai Medical Center, Los Angeles, CA, ¹⁵National Human Genome Research Institute, Bethesda, MD, ¹⁶Johns Hopkins University, Baltimore, MD, ¹⁷Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, Ellicott City, MD
Background/Purpose: Individuals with self-identified Black race have a higher incidence of systemic sclerosis (SSc), develop SSc at a younger age, and have a more severe…
Abstract Number: 1714 • ACR Convergence 2023
Aberrant Myeloid Populations in the TNF-Transgenic Model of Pulmonary Hypertension Overexpress Interferon Pathways and Are Driven by TNFR1 Signaling
Gaochan Wang¹, Qingfu Xu², Stacey Duemmel¹ and Benjamin Korman¹, ¹University of Rochester, Rochester, NY, ²University of Rochester Medical Center, Rochester, NY
Background/Purpose: Pulmonary hypertension (PH) is a severe, progressive disorder characterized by elevated pulmonary artery pressures, right ventricular hypertrophy, and increased mortality. We previously demonstrated that…
Abstract Number: 0654 • ACR Convergence 2023
Comparison of Cardiovascular Risk in Patients with Systemic Sclerosis and the General Population
Sabina Oreska¹, Hana Storkanova¹, Jaroslav Kudlicka², Vladimir Tuka², Ondrej Mikes², Zdislava Krupickova², Martin Satny², Eva Chytilova², Jan Kvasnicka², Maja Spiritovic³, Barbora Hermankova⁴, Petr Cesak⁵, Marian Rybar⁶, Karel Pavelka⁷, Ladislav Senolt⁸, Radim Becvar⁹, Jiri Vencovsky¹, Michal Vrablik² and Michal Tomcik¹, ¹Institute of Rheumatology, Prague, Czech Republic, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic, ²3rd Department of Internal Medicine, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic, ³Institute of Rheumatology, Prague, Czech Republic, Department of Physiotherapy, Faculty of Physical Education and Sport, Charles University, Prague, Czech Republic, ⁴Department of Physiotherapy, Faculty of Physical Education and Sport, Charles University, Prague, Czech Republic, ⁵Department of Human Movement Laboratory, Faculty of Physical Education and Sport, Charles University, Prague, Czech Republic, ⁶Department of Biomedical Technology, Faculty of Biomedical Engineering, Czech Technical University in Prague, Kladno, Czech Republic, ⁷Institut of Rheumatology and Department of Rheumatology, First Faculty of Medicine, Charles University, Praha, Czech Republic, ⁸Institute of Rheumatology and Department of Rheumatology, First Faculty of Medicine, Praha, Czech Republic, ⁹Institute of Rheumatology, Prague, Czech Republic, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Praha, Czech Republic
Background/Purpose: Patients with systemic sclerosis (SSc) may be burdened by increased cardiovascular (CV) risk due to accelerated atherosclerosis (ATS) due to systemic inflammation, and vascular…
Abstract Number: 2037 • ACR Convergence 2023
Assessing Risk of Depression in Common Rheumatologic Disorders Using Diagnostic Codes, Survey Scores, and Propensity Score Matching Methodology
COMFORT ANIM-KORANTENG¹, YONG EUN², Okeoghene Akpoigbe² and AMANDA SAMMUT², ¹Harlem Hospital Center, New York, NY, ²NYCHH- HARLEM, New York, NY
Background/Purpose: Many studies have shown that rheumatologic conditions are associated with a higher risk of depression. The two most common methods to detect depression in…

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