Abstracts tagged "Systemic sclerosis"

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…
Abstract Number: 0957 • ACR Convergence 2024
Increased Collagen Deposition and Altered Immune Cell Profiles Are Present in Early and Late Stage Systemic Sclerosis with Gastrointestinal Involvement
Laura Much¹, Elena Pachera², Andrea Laimbacher¹, Henriette Didriksen³, Lars Aabakken⁴, Knut Ea Lundin⁴, Lumeng Li¹, Astrid Hofman¹, Pietro Bearzi⁵, Sophie Wagner⁶, Michael Scharl⁷, Øyvind Molberg⁸, Håvard Fretheim⁹, Oliver Distler¹⁰ and Anna-Maria Hoffmann-Vold⁹, ¹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ²University Hospital Zurich, Zurich, Switzerland, ³Oslo University Hospital, Moss, Norway, ⁴Department of Transplantation Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway, ⁵Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland, ⁶University of Zurich, Schlieren, Switzerland, ⁷Department of Gastroenterology and Hepatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁸Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway, ⁹Oslo University Hospital, Oslo, Norway, ¹⁰Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland
Background/Purpose: Gastrointestinal tract (GIT) involvement is highly prevalent among patients with systemic sclerosis (SSc) and is associated with high morbidity and mortality. However, treatment options…
Abstract Number: 1560 • ACR Convergence 2024
Changes of Cerebral Structure and Perfusion Vary in Different Subtypes of Systemic Sclerosis: A Brain Magnetic Resonance Imaging Study
Xinyu Tong¹, Huilin He², Shihan Xu², Zihan Ning¹, Rui Shen¹, Xiaofeng Zeng³, qian wang⁴, Dong Xu⁵, Xihai zhao¹ and Zuoxiang He¹, ¹Tsinghua University, Beijing, China, ²Peking Union Medical College Hospital, Beijing, China, ³Department of Rheumatology, Peking Union Medical College Hospital (PUMCH), Chinese Academy of Medical Sciences National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China, ⁴Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China 2National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, Beijing, China, ⁵Peking Union Medical College Hospital, Dong Cheng District, Beijing, China
Background/Purpose: The characteristics of brain impairment in different subtypes of systemic sclerosis (SSc) (dcSSc, diffuse cutaneous SSc; lcSSc, limited cutaneous SSc) remain unclear. This study aimed to characterize cerebral structure and perfusion…
Abstract Number: 1586 • ACR Convergence 2024
The Relationship Between the Presence, Quantity and Distribution of Cutaneous Telangiectasia and Other Vascular Manifestations of Systemic Sclerosis
Matthew Wells¹, Robyn Domsic², Ami Shah³, Laura Hummers⁴, Aishwarya Anilkumar¹, Tracy Frech⁵, Ariane Herrick⁶, Christopher Denton⁷, Dinesh Khanna⁸ and John Pauling¹, ¹North Bristol NHS Trust, Bristol, United Kingdom, ²Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, ³Division of Rheumatology, Johns Hopkins University, Ellicott City, MD, ⁴Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Ellicott City, MD, ⁵Vanderbilt University Medical Center, Nashville, TN, ⁶University of Manchester, UK, Aberdeen, United Kingdom, ⁷University College London, Northwood, United Kingdom, ⁸University of Michigan, Ann Arbor, MI
Background/Purpose: Telangiectasia are permanently dilated dermal postcapillary venules and are the 2nd most common manifestation (~80%) of systemic sclerosis (SSc) after Raynaud’s phenomenon (RP). The…

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