Abstracts tagged "Systemic sclerosis"

Abstract Number: 1826 • ACR Convergence 2024
Unveiling the Primary Cilia Signature Driving Systemic Sclerosis Pathogenesis
Le My Tu Nguyen¹, Carlos Córdova-Fletes², Poulami Dey³, Johann Gudjonsson³, Rebecca Wells⁴, Rebecca Ross⁵, Natalia Riobo-Del Galdo⁴, Francesco Del Galdo⁴, Dibyendu Bhattacharyya³, John Varga³ and Maria Teves¹, ¹Virginia Commonwealth University, Richmond, VA, ²Universidad Autónoma de Nuevo León, Monterrey, Mexico, ³University of Michigan, Ann Arbor, MI, ⁴University of Leeds, Leeds, United Kingdom, ⁵Medicine and Health, University of Leeds, Leeds, United Kingdom
Background/Purpose: Fibrosis in multiple organs is the defining hallmark that accounts for the high mortality associated with systemic sclerosis (SSc). Understanding how SSc patients develop…
Abstract Number: 2443 • ACR Convergence 2024
The Clinical Phenotype of Anti-Th/To+ Patients in Systemic Sclerosis: A Case-control Study Within the European Scleroderma Trials and Research (EUSTAR) Cohort
Liala Moschetti¹, Eleonora Pedretti², Francesco Bonomi³, María Martín López⁴, Fabio Cacciapaglia⁵, cristiana sieiro santos⁶, Gianluca Moroncini⁷, Yannick Allanore⁸, Joana Caetano⁹, Brigitte Granel¹⁰, Laura Groseanu¹¹, Maria De Santis¹², Masataka Kuwana¹³, Veronica Codullo¹⁴, Mariana Pereira Silva¹⁵, Pietro Bearzi¹⁶, Laura Belloli¹⁷, Alida Taberner-Cortés¹⁸, Cristina Maglio¹⁹, Francesco Del Galdo²⁰, Corrado Campochiaro²¹, Marie-Elise Truchetet²², Giovanna Cuomo²³, Magda Parvu²⁴, Florenzo Iannone²⁵, Patricia Carreira²⁶, Serena Guiducci²⁷, Franco Franceschini¹, Paolo Airò¹ and Maria-Grazia Lazzaroni¹, ¹Scleroderma Unit, Rheumatology and Clinical Immunology Unit, ERN ReCONNET, ASST Spedali Civili, Brescia, Italy, Brescia, Italy, ²Scleroderma Unit, Rheumatology Unit, ERN ReCONNET, ASST Spedali Civili and University of Brescia; Italy, Brescia, Italy, ³Division of Rheumatology, Scleroderma Unit, University of Florence, AOU Careggi Firenze; Italy, Firenze, Italy, ⁴General University Hospital of Ciudad Real, Ciudad de México, Spain, ⁵Rheumatology Unit � DiMePRe-J, University and AOU Policlinico of Bari, Italy, Bari, Italy, ⁶Rheumatology Department, Complejo Asistencial Universitario de León, León, Spain, Leon, Spain, ⁷Marche Polytechnic University, Ancona, Italy, ⁸Rheumatology department, Université Paris Cité, Cochin Hospital of Paris; France, Paris, France, ⁹Systemic Autoimmune Diseases Unit, Fernando Fonseca Hospital, Amadora; Portugal, Amadora, Portugal, ¹⁰Service de Médecine Interne Hôpital Nord de Marseille; France, Marseille, France, ¹¹Spitalul Sfanta Maria, Bucharest, Romania, ¹²Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital and Biomedical Sciences, Hu-manitas University, Milan; Italy, Milan, Italy, ¹³Department of Allergy and Rheumatology, Nippon Medical School, Tokyo, Japan, Tokyo, Japan, ¹⁴Unit of Rheumatology, San Matteo Hospital, Pavia, Italy, ¹⁵Rheumatology Department, Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa; Portugal, Lisboa, Portugal, ¹⁶Immunorheumatology Unit, Università Campus Bio-Medico University of Roma; Italy, Roma, Italy, ¹⁷Niguarda Hospital, Milan, Milan, Italy, ¹⁸Hospital Universitario Doctor Peset, Valencia, Spain, ¹⁹University of Gothenburg, Gothenburg, Sweden, ²⁰University of Leeds, Leeds, United Kingdom, ²¹IRCCS San Raffaele Hospital. Vita-Salute San Raffaele University, Milan, Milan, Italy, ²²Bordeaux University Hospital, Bordeaux, France, ²³Università degli studi della Campania Luigi Vanvitelli, Napoli, Italy, ²⁴Colentina Clinical Hospital, Rheumatology Department, Bucharest; Romania, Bucharest, Romania, ²⁵Rheumatology Unit- University of Bari "Aldo Moro", IT, Bari, Italy, ²⁶Hospital Universitario 12 de Octubre, Madrid, Madrid, Spain, ²⁷Division of Rheumatology, Scleroderma Unit, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy
Background/Purpose: To evaluate clinical associations of anti-Th/To antibodies in SSc patients in a multicentre international cohort, focusing on interstitial lung disease (ILD), pulmonary arterial hypertension…
Abstract Number: 2471 • ACR Convergence 2024
Association of Telangiectasias with Autoantibodies and Clinical Manifestations in Systemic Sclerosis
Namitha Nair¹, Thomas Medsger, Jr², Robert Lafyatis³, Maureen Laffoon⁴, Leigh Freno⁴ and Robyn Domsic⁵, ¹University of Pittsburgh Medical Center, Pittsburgh, PA, ²Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Verona, PA, ³University of Pittsburgh, Pittsburgh, PA, ⁴UPMC, Pittsburgh, PA, ⁵Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA
Background/Purpose: Telangiectasias (Tel) are visibly dilated cutaneous post-capillary venules reported to occur in about 75% of patients with systemic sclerosis (SSc), making them a common…
Abstract Number: 0350 • ACR Convergence 2024
Piloting an Adaptation of the Making It Work Program for Systemic Sclerosis: Promising Effects on Job Related Self-efficacy and Risk of Work Disability
Janet Poole¹, Kristine Carandang², Mary Thelander Hill¹, Jessica Salazar¹, Anna Koch³, Timothy Dionne¹ and Diane Lacaille⁴, ¹University of New Mexico, Albuquerque, NM, ²Young Patients' Autoimmune Research & Empowerment Alliance, San Diego, CA, ³Comfy Couch Counseling LLC, Albuquerque, NM, ⁴Arthritis Research Canada, University of British Columbia, Vancouver, BC, Canada
Background/Purpose: Although work disability presents a substantial burden for people with systemic sclerosis (SS), there are no tailored programs that aim to support their employment.…
Abstract Number: 0709 • ACR Convergence 2024
Linear Combination of Principal Components Achieves Top Performance in Identifying Rheumatologist-Diagnosed Systemic Sclerosis from Electronic Health Records
Yiming Luo, Gongbo Zhang, Chunhua Weng and Elana Bernstein, Columbia University Irving Medical Center, New York, NY
Background/Purpose: Systemic sclerosis (SSc) is a rare systemic autoimmune rheumatic disease. International Classification of Diseases (ICD) code counts (for example, using ≥ 2 ICD-10 codes…
Abstract Number: 0960 • ACR Convergence 2024
The Esophageal Epithelium in Systemic Sclerosis: Cellular and Molecular Dysregulation Revealed by Single-Cell RNA Sequencing
Matthew Dapas¹, Margarette Clevenger¹, Hadijat Makinde², Tyler Therron¹, Dustin Carlson¹, Mary Carns³, Kathleen Aren³, Carrie Richardson², Cenfu Wei², Lutfiyya Muhammad⁴, John Pandolfino¹, Harris Perlman², Deborah Winter⁵ and Marie-Pier Tetreault¹, ¹Northwestern University, Chicago, ²Northwestern University, Chicago, IL, ³Northwestern University Division of Rheumatology, Chicago, IL, ⁴Northwestern Feinberg School of Medicine, Chicago, IL, ⁵Northwestern University, Skokie, IL
Background/Purpose: Systemic sclerosis (SSc) is a rare autoimmune disease characterized by vasculopathy and progressive fibrosis of the skin and internal organs. Individuals with SSc often…
Abstract Number: 1567 • ACR Convergence 2024
Systemic Sclerosis Is Associated with Sub-Clinical Abnormalities in Myocardial Energetics, Perfusion and Increased Fibrosis
Chin Yit Soo¹, Nicholas Jex², Stefano Di Donato³, Lucy Elizabeth Thornton⁴, Vishal Kakkar⁵, Riccardo Bixio⁶, Raluca Tomoaia¹, Thomas Anderton², May Lwin², Christel Kamani², Amrit Chowdhary², Henry Procter¹, Sharmaine Thirunavukarasu¹, Sindhoora Kotha², Marilena Giannoudi², Eylem Levelt², Sven Plein¹, Francesco Delgado⁶ and Lesley-Anne Bissell⁷, ¹Multidisciplinary Cardiovascular Research Centre and Biomedical Imaging Science Department, University of Leeds, Leeds, United Kingdom., Leeds, England, United Kingdom, ²Multidisciplinary Cardiovascular Research Centre and Biomedical Imaging Science Department, University of Leeds, Leeds, United Kingdom., Leeds, United Kingdom, ³1. Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, 2. NIHR Leeds Biomedical Research Centre, United Kingdom, Leeds, United Kingdom, ⁴1. Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, 2. NIHR Leeds Biomedical Research Centre, United Kingdom, Leeds, ⁵University of Leeds, Leeds, United Kingdom, ⁶Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom., Leeds, United Kingdom, ⁷Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom
Background/Purpose: Primary cardiac involvement in systemic sclerosis (SSc) is heterogenous, poorly defined and associated with significant cardiac morbidity and mortality. The underlying mechanism is not…
Abstract Number: 1590 • ACR Convergence 2024
Milder ILD with Preserved Lung Function Significantly Contributes to Respiratory Caused Mortality in SSc
Anna-Maria Hoffmann-Vold¹, Håvard Fretheim¹, Emily Langballe², Hilde Jenssen Bjørkekjær³, Henriette Didriksen¹, Phuong Phuong Diep⁴, Anna Helena Andersson¹, Ragnar Gunnarsson¹, Michael T Durheim¹, Torhild Garen¹, Øyvind Midtvedt⁵, Trond Mogens Aaløkken⁶, Øyvind Molberg⁷ and Oliver Distler⁸, ¹Oslo University Hospital, Oslo, Norway, ²Oslo University Hospital, Department of Rheumatology, Oslo, Norway, ³Department of Rheumatology, Hospital of Southern Norway, Kristiansand, Kristiansand, Norway, ⁴Oslo University Hospital, Department of Respiratory Disease, Oslo, Norway, ⁵Oslo University Hospital, Oslo, Oslo, Norway, ⁶Medicine, Oslo, Norway, Oslo, Norway, ⁷Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway, ⁸Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland
Background/Purpose: Interstitial lung disease (ILD) is the main cause of death in systemic sclerosis (SSc). While impaired lung function with forced vital capacity (FVC) <…
Abstract Number: 1827 • ACR Convergence 2024
Characterization of Fibroblast Subpopulations and Their Cellular Local Environments in Systemic Sclerosis Using Imaging Mass Cytometry
Aleix Rigau¹, Minrui Liang², Veda Devakumar², Ranjana Neelagar², Alexandru-Emil Matei², Andrea-Hermina Györfi², Christina Bergmann³, Tim Filla², Vladyslav Fedorchenko¹, Georg Schett⁴, Jörg Distler⁵ and Yi-Nan Li², ¹Friedrich-Alexander Universität Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, ²Universitätsklinikum Düsseldorf and Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Germany, ³Department Internal Medicine III, Friedrich-Alexander-University (FAU) Erlangen-Nurnber, Frankfurt, Germany, ⁴Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany, ⁵Universitätsklinikum Düsseldorf and Heinrich-Heine-Universität Düsseldorf, Duesseldorf, Germany
Background/Purpose: Single-cell transcriptomic data has shown that fibroblasts exhibit heterogeneity, with subpopulations that have diverse functional characteristics. However, the conventional single-cell methods are unable to…
Abstract Number: 2445 • ACR Convergence 2024
Effects of Aminaphtone on TGF-beta1 Serum Concentration as Concomitant Treatment to Standard Therapy in Systemic Sclerosis Patients: A Pilot Study
Alberto Sulli¹, Stefano Soldano², Rosanna Campitiello³, Elvis Hysa⁴, Tamara Vojinovic⁵, Carmen Pizzorni⁶, Sabrina Paolino⁷, Maurizio Cutolo⁵ and Emanuele Gotelli⁵, ¹Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genoa, Italy., Genova, Italy, ²Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Genoa, Italy, Genoa, Liguria, Italy, ³Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genoa, Italy, Genova, Liguria, Italy, ⁴Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Genoa, Italy, Genoa, Italy, ⁵Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genoa, Italy, Genova, Italy, ⁶Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genoa, Italy, Genoa, Italy, ⁷Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (Di.M.I.), University of Genova; IRCCS Ospedale Policlinico San Martino, Genova, Liguria, Italy
Background/Purpose: Aminaphtone (AMI) is a vasoactive drug licensed to treat microvascular disorders and able to increase peripheral blood perfusion and to reduce Raynaud's phenomenon (RP)…
Abstract Number: 2475 • ACR Convergence 2024
The Critical Role of Annual HRCT in Identifying ILD Progression and Assessing Outcome in SSc
Anna-Maria Hoffmann-Vold¹, Simon Beyeler², Liubov Petelytska³, Håvard Fretheim¹, Trond Mogens Aaløkken⁴, Mike Becker⁵, Hilde Jenssen Bjørkekjær⁶, Cathrine Brunborgg¹, Cosimo Bruni⁷, Christian Clarenbach⁸, Phuong Phuong Diep⁹, Rucsandra Dobrota⁷, Michael T Durheim¹, Muriel Elhai¹⁰, Thomas Frauenfelder⁸, Suzana Jordan⁷, Emily Langballe¹¹, Øyvind Midtvedt¹², Carina Mihai⁷, Adela Sarbu⁸, Marco Sprecher⁸, Øyvind Molberg¹³ and Oliver Distler¹⁴, ¹Oslo University Hospital, Oslo, Norway, ²University Hospital Zurich, Zürich, Zurich, Switzerland, ³University Hospital Zurich, Zurich, Switzerland, ⁴Medicine, Oslo, Norway, Oslo, Norway, ⁵Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ⁶Department of Rheumatology, Hospital of Southern Norway, Kristiansand, Kristiansand, Norway, ⁷University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁸University Hospital Zurich, Zurich, Zurich, Switzerland, ⁹Oslo University Hospital, Department of Respiratory Disease, Oslo, Norway, ¹⁰University Hospital zurich, Zürich, Switzerland, ¹¹Oslo University Hospital, Department of Rheumatology, Oslo, Norway, ¹²Oslo University Hospital, Oslo, Oslo, Norway, ¹³Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway, ¹⁴Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland
Background/Purpose: Progression of interstitial lung disease (ILD) reduces long-term survival in patients with systemic sclerosis (SSc). Monitoring with lung function testing every 3–6 months for…
Abstract Number: 0677 • ACR Convergence 2024
Patterns of Raynaud’s Phenomenon Management in Patients with Systemic Sclerosis: A Real-World Data from Community-Based Practices in the United States
Gulsen Ozen¹, Sofia Pedro², Kaleb Michaud³ and Robyn Domsic⁴, ¹University of Nebraska Medical Center, Bellevue, NE, ²Forward, The National Databank for Rheumatic Diseases, Wichita, KS, ³University of Nebraska Medical Center, Omaha, NE, ⁴Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA
Background/Purpose: Raynaud’s phenomenon (RP) and the complex vasculopathy of systemic sclerosis (SSc) can lead to chronic digital ischemia, ulcerations, and necrosis with significant pain and…
Abstract Number: 0711 • ACR Convergence 2024
Molecular Signatures in the Skin as Predictors of Longitudinal Clinical Trajectories in Patients with Systemic Sclerosis
Natania Field¹, Monica Yang², Niket Gupta¹, Maxwell Shramuk¹, Lutfiyya Muhammad¹, Vivien Goh¹, Monica Espinoza³, Yiwei Yuan³, Rezvan Parvizi³, Kathleen Aren⁴, Mary Carns⁴, Isaac Goldberg⁵, Lorinda Chung⁶, Dinesh Khanna⁷, Zsuzsanna McMahan⁸, Michael Whitfield³ and Monique Hinchcliff⁹, ¹Northwestern Feinberg School of Medicine, Chicago, IL, ²UCSF, San Francisco, CA, ³Geisel School of Medicine at Dartmouth, Hanover, NH, ⁴Northwestern University Division of Rheumatology, Chicago, IL, ⁵Wayne State University School of Medicine, Detroit, MI, ⁶Stanford University, Woodside, CA, ⁷University of Michigan, Ann Arbor, MI, ⁸UTHealth Houston Division of Rheumatology, Houston, TX, ⁹Yale School of Medicine, Westport, CT
Background/Purpose: It has become clear that classification by phenotype is insufficient to explain the heterogeneity in clinical progression for many rheumatic diseases. Systemic sclerosis (SSc)…
Abstract Number: 0962 • ACR Convergence 2024
Characterizing the Contribution of Myeloid Cells to Limited and Diffuse Cutaneous Systemic Sclerosis
Parker Jones¹, Salina Dominguez², Miranda Gurra³, Gaurav Gadhvi⁴, Tyler Therron², Kathleen Aren⁵, Carla Cuda³, Monique Hinchcliff⁶, Harris Perlman³, Hadijat Makinde³ and Deborah Winter⁷, ¹Northwestern University Feinberg School of Medicine, Chicago, IL, ²Northwestern University, Chicago, ³Northwestern University, Chicago, IL, ⁴University of Michigan, Ann Arbor, MI, ⁵Northwestern University Division of Rheumatology, Chicago, IL, ⁶Yale School of Medicine, Westport, CT, ⁷Northwestern University, Skokie, IL
Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disease characterized by multiorgan fibrosis. The two main subtypes are diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc…
Abstract Number: 1569 • ACR Convergence 2024
Scleroderma Renal Crisis – Different Phenotypes and Patient Trajectories
Katya Dolnikov¹, Kochava Toledano¹, Marius Braun², Doron Markovits¹, Sami Giryes¹, Yonit Tavor¹, Alexandra Balbir-Gurman³ and Yolanda Braun Moscovici¹, ¹Rheumatology Institute, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion-Israeli Institute of Technology, HAIFA, Israel, ²Liver Insitute, Beilinson Hospital, Petach Tikva, Petach Tikva, Israel, ³Rheumatology Institute, Rambam Health Care Campus and The Rappaport Faculty of |Medicine, Technion, Haifa, Israel
Background/Purpose: Scleroderma renal crisis (SRC) is a life-threatening complication that occurs in a minority of patients, particularly those with diffuse cutaneous systemic sclerosis ( SSc).…

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