Abstracts tagged "Systemic sclerosis"

Abstract Number: 0698 • ACR Convergence 2024
Association of Systemic Sclerosis with ANCA-Associated Vasculitis and Large Vessel Vasculitis: Impact of Pulmonary Arterial Hypertension
Brett Dinner¹, Ahmed Abdelmaksoud², Ann Igoe³, Taylor Viggiano⁴ and Vivek Nagaraja⁵, ¹Creighton University, Paradise Valley, AZ, ²University of California, Riverside, Riverside, CA, ³Flow, Tempe, AZ, ⁴Mayo Clinic, Phoenix, ⁵Mayo Clinic Arizona, Scottsdale, AZ
Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disorder with excessive fibrosis and vasculopathy. While typically non-inflammatory, frank vasculitis can complicate SSc. Concomitant SSc with ANCA-associated…
Abstract Number: 0954 • ACR Convergence 2024
Increased Proliferating Natural Killer Cells Exhibit an Aberrant Pro-Inflammatory Gene Signature in Systemic Sclerosis Patients
Pietro Bearzi¹, Elena Pachera², Sophie Wagner³, Astrid Hofman⁴, Lumeng Li⁴, Laura Much⁴, Mike Becker⁵, Kristina Bürki⁴, Luca Navarini⁶, Anna-Maria Hoffmann-Vold⁷, Roberto Giacomelli⁸ and Oliver Distler⁹, ¹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland, ²University Hospital Zurich, Zurich, Switzerland, ³University of Zurich, Schlieren, Switzerland, ⁴Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁵Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ⁶Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, University of Rome "Campus Bio-Medico", Rome, Italy, ⁷Oslo University Hospital, Oslo, Norway, ⁸Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, University of Rome "Campus Bio-Medico", Roma, Italy, ⁹Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland
Background/Purpose: Recent transcriptomic data suggest a prominent involvement of innate immune cells in the pathogenesis of SSc. In this regard, contrasting data on NK cells…
Abstract Number: 1557 • ACR Convergence 2024
Estimation of Cardiovascular Risk in Patients with Systemic Sclerosis – Accuracy of Tools Based on SCORE and Its Modifications Compared to Ultrasound Examination of Subclinical Atherosclerosis
Sabína Oreská¹, Aneta Prokopcová², Hana Storkanova¹, Jaroslav Kudlicka³, Vladimir Tuka⁴, Ondrej Mikes⁴, Zdislava Krupickova⁴, Martin Satny⁴, Eva Chytilova⁴, Jan Kvasnicka⁴, Maja Spiritovic⁵, Barbora Hermankova⁶, Petr Cesak⁷, Marian Rybar⁸, Karel Pavelka⁹, Ladislav Šenolt², Radim Becvar¹, Jiří Vencovský², Michal Vrablik⁴ and Michal Tomcik¹, ¹Institute of Rheumatology and Department of Rheumatology, First Faculty of Medicine, Charles University, Prague, Czech Republic, Prague, Czech Republic, ²Institute of Rheumatology and Department of Rheumatology, First Faculty of Medicine, Charles University, Prague, Czech Republic, ³Institute of Rheumatology, Prague, Czech Republic, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic, Prague, Czech Republic, ⁴3rd Department of Internal Medicine, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic, Prague, ⁵Institute of Rheumatology, Prague, Czech Republic, Prague, ⁶Institute of Rheumatology, Prague, Czech Republic, Prague, Czech Republic, ⁷Department of Human Movement Laboratory, Faculty of Physical Education and Sport, Charles University, Prague, Czech Republic, Prague, ⁸Department of Biomedical Technology, Faculty of Biomedical Engineering, Czech Technical University in Prague, Kladno, Czech Republic, Kladno, ⁹Institute of Rheumatology and Charles University, Praha, Czech Republic
Background/Purpose: Patients with systemic sclerosis (SSc) have increased cardiovascular (CV) risk due to accelerated atherosclerosis (ATS) caused by systemic inflammation, and vascular impairment. To date,…
Abstract Number: 1581 • ACR Convergence 2024
Neutrophil-to- lymphocyte Ratio: A Possible Biomarker for Clinical Response After Autologous Hematopoietic Stem Cell Transplantation
shiri keret¹, Lisa Kaly², Georg Schett³, Christina Bergmann⁴, Joerg Henes⁵, Gleb Slobodin² and Doron Rimar², ¹Rheumatology unit, Bnai-Zion medical center and the faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel., Atlit, Israel, ²Rheumatology unit, Bnai-Zion medical center and the faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel., Haifa, Israel, ³Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany, ⁴Department Internal Medicine III, Friedrich-Alexander-University (FAU) Erlangen-Nurnber, Frankfurt, Germany, ⁵University Hospital Tuebingen, Tuebingen, Germany
Background/Purpose: Baseline high Neutrophil- to- lymphocyte ratio (NLR) (higher than 2.95) is associated with severe progressive skin and lung disease and with reduced 5-year survival…
Abstract Number: 1821 • ACR Convergence 2024
Single-Cell RNA SequencingBioinformatic Pipeline Optimized for Non-Coding GenesIdentified LINC01503 and MIR193-BHG as Potential Pathogenic Effectors in Systemic Sclerosis Fibroblasts
Sophie Wagner¹, Elena Pachera², Gino Andrea Bonazza³, Celina Geiss⁴, Astrid Hofman⁴, Laura Much⁴, Lumeng Li⁴, Pietro Bearzi³, Anna-Maria Hoffmann-Vold⁵ and Oliver Distler⁶, ¹University of Zurich, Schlieren, Switzerland, ²University Hospital Zurich, Zurich, Switzerland, ³Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland, ⁴Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁵Oslo University Hospital, Oslo, Norway, ⁶Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland
Background/Purpose: Long non-coding RNAs (lncRNA) are a class of transcripts that regulate gene expression. Currently, only few lncRNAs have been linked to SSc pathogenesis. As…
Abstract Number: 2204 • ACR Convergence 2024
Guidance for Stem Cell Therapy for Juvenile Systemic Sclerosis Patients
Ivan Foeldvari¹, Kathryn Torok², Juliana Silva³, Paulina Horvei⁴, Christopher Denton⁵, Franziksa Rosser⁶, Tamas Constantin⁷, Patricia Costa Reis⁸, Megan Curran⁹, Maurizio Cutolo¹⁰, Joerg Henes¹¹, Bernd Hinrichs¹², Kim Fligelstone¹³, Suzanne Li¹⁴, Susan Maillard³, Pia Moinzadeh¹⁵, Catherine Orteu¹⁶, Clare Pain¹⁷, Clarissa Pilkington¹⁸, Linda Schraven¹⁹ and vanessa smith²⁰, ¹Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ²Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ³Great Ormond Street Hospital, London, United Kingdom, ⁴Division of Blood and Marrow Transplantation and Cellular Therapies, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁵University College London, Northwood, United Kingdom, ⁶Division of Pulmonology Medicine, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ⁷Semmelweis University, Budapest, Hungary, ⁸Hospital de Santa Maria, Lisbon, Portugal, ⁹Children's Hospital Colorado, Colorado, ¹⁰Laboratory 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, ¹¹University Hospital Tuebingen, Tuebingen, Germany, ¹²Children's pulmonology, Asklepios Klinik Nord – Heidberg, Hamburg, Germany, ¹³FESCA, London, United Kingdom, ¹⁴Hackensack University Medical Center, Hackensack, ¹⁵University Hospital Cologne, Cologne, Germany, ¹⁶Royal Free London NHS Foundation Trust, London, United Kingdom, ¹⁷Alder Hey Children’s Foundation NHS Trust, Liverpool, United Kingdom, ¹⁸Great Ormond Street Hospital, London, United Kingdom, ¹⁹Dutch representative of Fesca, London, United Kingdom, ²⁰Ghent University Hospital, Gent, Belgium
Background/Purpose: Hemopoetic stem cell transplantation(HSTC) and cellular therapies(CT) are a promising therapeutic options for juvenile systemic sclerosis(jSSc) patients. As pediatric patients have a unique potential…
Abstract Number: 2465 • ACR Convergence 2024
Subclinical Loss of Lung Volumes in Very Early SSc: Evidence from Two Independent Cohorts in EUSTAR
Vishal Kakkar¹, Stefano Di Donato², Silvia Bellando-Randone³, Riccardo Bixio⁴, Marco Matucci-Cerinic⁵, Yannick Allanore⁶ and Francesco Del Galdo¹, ¹University of Leeds, Leeds, United Kingdom, ²Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK, Leeds, United Kingdom, ³University of Florence, Florence, Florence, Italy, ⁴University of Verona, Verona, Verona, Italy, ⁵University San Raffaele Milano, Milano, Milan, Italy, ⁶Université Paris Cité, Paris, France
Background/Purpose: Interstitial lung disease (ILD) remains as the major driver of mortality in systemic sclerosis (SSc). The literature indicates that at the time of diagnosis…
Abstract Number: 0699 • ACR Convergence 2024
Cardiac Biomarkers for Predicting Cardiopulmonary Toxicity of Autologous Haematopoietic Stem Cell Transplantation for Systemic Sclerosis
Ross Penglase¹, Samadhi Gunawardhana¹, Graham Jones¹, Andrew Jabbour¹, Eugene Kotlyar¹, Laila Girgis², David Ma¹ and John Moore¹, ¹St Vincent's Hospital Sydney, Darlinghurst, New South Wales, Australia, ²St Vincent's Hospital, Sydney, Darlinghurst, New South Wales, Australia
Background/Purpose: Autologous Haematopoietic Stem Cell Transplantation (AHSCT) is an effective treatment for severe systemic sclerosis (SSc), with demonstrated benefit for pulmonary and cutaneous outcomes and…
Abstract Number: 0955 • ACR Convergence 2024
Inhibition of Interleukin-2-Inducible T Cell Kinase with Soquelitinib Demonstrates Efficacy in Preventing Lung Damage in Murine Models of Systemic Sclerosis
Goncalo Boleto¹, Anne Cauvet², James Rose³, Lih-Yun Hsu³, Richard A. Miller³ and Yannick Allanore⁴, ¹Rheumatology Department, Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisboa, Portugal., Lisbon, Portugal, ²Université de Paris, Institut Cochin, INSERM U1016 CNRS UMR8104, Paris, France, ³Corvus Pharmaceuticals, 863 Mitten Road, Burlingame, Burlingame, CA, ⁴Université Paris Cité, Paris, France
Background/Purpose: T cells abnormal activation plays a pivotal role in the early inflammatory stages of systemic sclerosis (SSc). Compelling evidence supports that SSc has a…
Abstract Number: 1559 • ACR Convergence 2024
Vectorcardiography for Identification of Systemic Sclerosis Patients at Risk for Pulmonary Hypertension
S. Ahmed¹, Eva Hoekstra², Katie van der Wouden², Sum-Che Man², Kees Swenne², Madelon Vonk³, Anna-Maria Hoffmann-Vold⁴, Cosimo Bruni⁵, Udo Mulder⁶, Thomas Huizinga⁷, H.W. Vliegen² and Jeska de Vries-Bouwstra², ¹Leiden University Medical Centre, Leiden, Netherlands, ²Leiden University Medical Center, Leiden, Netherlands, ³Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands, ⁴Oslo University Hospital, Oslo, Norway, ⁵University of Zurich, Zurich, Switzerland, ⁶Universitair Medisch Centrum Groningen, Groningen, Netherlands, ⁷Department of Rheumatology, Leiden University, Leiden, Netherlands
Background/Purpose: Pulmonary hypertension (PH) in SSc causes significant disease burden and requires invasive diagnosis with right heart catheterization (RHC). Comprehensive work-up is needed to identify…
Abstract Number: 1582 • ACR Convergence 2024
Impact of Immunosuppressive Treatment on Development and Survival in Systemic Sclerosis Associated Pulmonary Arterial Hypertension (SSc-PAH)
Stefano Rodolfi¹, Cheryl Chun Man Ng², Ana Maria Ruiz Bejerano², Medha Kanitkar², Voon Ong³ and Christopher Denton⁴, ¹Centre for Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, ²University College London, London, United Kingdom, ³University College London, London, England, United Kingdom, ⁴University College London, Northwood, United Kingdom
Background/Purpose: Pulmonary arterial hypertension (PAH) is a severe and frequently life-threatening complication of systemic sclerosis (SSc). Treatment of SSc-PAH follows the same approach of idiopathic…
Abstract Number: 1822 • ACR Convergence 2024
Analysis of Differential Activation of Lysophosphatidic Acid Regulated Genes in Diffuse and Limited Cutaneous Systemic Sclerosis
Medha Kanitkar¹, Philip Yee², Stefano Rodolfi¹, Kristina Clark³, Voon Ong⁴ and Christopher Denton⁵, ¹University College London, London, United Kingdom, ²UCL, London, United Kingdom, ³University of Oxford, Oxford, United Kingdom, ⁴University College London, London, England, United Kingdom, ⁵University College London, Northwood, United Kingdom
Background/Purpose: Lysophosphatidic Acid (LPA) is a lipid mediator implicated in the pathogenesis of SSc and idiopathic pulmonary fibrosis. Phase 2 clinical trials targeting this biological…
Abstract Number: 2205 • ACR Convergence 2024
Presence of Nailfold Capillary Changes Correlates with More Severe Organ Involvement in Juvenile Systemic Scleroderma. Results of the Juvenile Scleroderma Inception Cohort
Ivan Foeldvari¹, Jens Klotsche², Kathryn Torok³, Ozgur Kasapcopur⁴, Amra Adrovic⁵, Brian Feldman⁶, Jordi Anton⁷, Sindhu R. Johnson⁸, Flavio Sztajnbok⁹, Maria Teresa Terreri¹⁰, Ana Sakamoto¹¹, Valda Stanevica¹², Dieneke Schonenberg-Meinema¹³, Ekaterina Alexeeva¹⁴, Maria Katsicas¹⁵, Raju Khubchandani¹⁶, Sujata Sawhney¹⁷, vanessa smith¹⁸, Eslam Al-Abadi¹⁹, Simone Appenzeller²⁰, Tadey Avcin²¹, Mikhail Kostik²², Thomas Lehman²³, Hana Malcova²⁴, edoardo marrani²⁵, Clare Pain²⁶, Anjali Patwardhan²⁷, Walter Alberto Sifuentes-Giraldo²⁸, Natalia Vasquez-Canizares²⁹, Sima Abu Alsaoud³⁰, Patricia Costa Reis³¹, Mahesh Janarthanan³², Dana Nemcova³³, Maria Jose Santos³⁴ and Nicola Helmus³⁵, ¹Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ²German Rheumatism Research Center, Berlin, Germany, ³Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ⁴Istanbul University-Cerrahpasa, Cerrahpasa Medical School, istanbul, Turkey, ⁵Cerrahpasa Medical School, Istanbul, Turkey, ⁶Division of Rheumatology, The Hospital for Sick Children; Departments of Pediatrics and Medicine, Faculty of Medicine; The Institute for Health Policy, Management and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, ON, Canada, ⁷Hospital Sant Joan de Déu. Universitat de Barcelona, Esplugues de Llobregat (Barcelona), Spain, ⁸University of Toronto, Toronto, Canada, ⁹UFRJ/UERJ, SAO PAULO, Brazil, ¹⁰UNIFESP, São Paulo, SP, Brazil, ¹¹Federal University of São Paulo (UNIFESP), São Paulo, SP, Brazil, ¹²Childrens Clinical University Hospital, Zemgales priekšpilseta, Riga, Latvia, ¹³Emma Children’s Hospital, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, Netherlands, ¹⁴National Medical Research Center of Children's Health, Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation, Moscow, Russia, ¹⁵Hospital Garrahan, Buenos Aires, Argentina, ¹⁶SRCC Childrens Hospital, Mumbai, India, ¹⁷Sir Ganga Ram Hospital, Sector 37 noida, Uttar Pradesh, India, ¹⁸Ghent University Hospital, Gent, Belgium, ¹⁹Birmingham Women’s and Children’s Hospital NHSFT, Birmingham, United Kingdom, ²⁰Unicamp, Campinas, SP, Brazil, ²¹University Children's Hospital University Medical Center Ljubljana, Ljubljana, Slovenia, ²²Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ²³Hospital for Special Surgery, New York, NY, ²⁴Motol University Hospital, Prague, Czech Republic, ²⁵Dipartimento Neurofarba, Università di Firenze, Firenze, Italy, ²⁶Alderhey Children's NHS Foundation Trust, Liverpool, United Kingdom, ²⁷University of Missouri-Columbia, Department of Child Health, 404 N Keene Street, Columbia MO 65210, Columbia, ²⁸Hospital Universitario Ramon y Cajal, Madrid, Spain, ²⁹Children’s Hospital at Montefiore, Bronx, NY, ³⁰Caritas baby Hospital, East Jerusalem, Israel, ³¹Hospital de Santa Maria, Lisbon, Portugal, ³²SRI RAMACHANDRA INSTITUTE OF HIGHER EDUCATION AND RESEARCH, Chennai, Tamil Nadu, India, ³³MD, Prague, Czech Republic, ³⁴Hospital Garcia de Orta and Centro Académico de Medicina de Lisboa, Lisboa, Portugal, ³⁵Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany
Background/Purpose: Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence in 3 in 1 000 000 children. Positive nailfold capillaroscopy (NF+) finding correlate with more…
Abstract Number: 2466 • ACR Convergence 2024
Ranked Composite Important Difference (RCID) Scores in Patients with Diffuse Cutaneous Systemic Sclerosis and Interstitial Lung Disease
Francesco Del Galdo¹, Lesley-Anne Bissell², Rozeta Simonovska³ and Margarida Alves⁴, ¹University of Leeds, Leeds, United Kingdom, ²Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, ³mainanalytics GmbH, Sulzbach (Taunus), Germany, Sulzbach (Taunus), Germany, ⁴Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, Ingelheim, Germany
Background/Purpose: The ACR Composite Response Index in diffuse cutaneous Systemic Sclerosis (CRISS) was developed to measure the probability of improvement in response to treatment in…
Abstract Number: 0703 • ACR Convergence 2024
Preliminary Results from the RECONNOITER Trial, a Phase 2 Study of AISA 021 in the Treatment of Secondary Raynaud’s, Primarily Due to Systemic Sclerosis
Andrew Sternlicht¹, Michael Shanahan², Erin Morton², Meredith Todd³, Ivana Hunt², Zoey Reed², amanda weragoda⁴, Lashika Weerakoon² and Elizabeth Briggs², ¹Tufts University Medical Center and Aisa Pharma, Inc., Boston, MA, ²Flinders Medical Centre, Adelaide, Australia, ³Aisa Pharma Pty Ltd., East Lindfield, New South Wales, Australia, ⁴F, Adelaide, Australia
Background/Purpose: While 95% of Systemic Sclerosis (SSc) patients have Raynaud’s phenomenon(RP) and many patients rate it as the most bothersome and severe symptom of their…

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