Abstracts tagged "Scleroderma"

Abstract Number: 1532 • ACR Convergence 2023
Safety and Microvascular Effects of Long-term Treatment with Aminaphtone in Systemic Sclerosis Patients: A Retrospective Analysis
Alberto Sulli¹, Rosanna Campitiello¹, Emanuele Gotelli¹, Andrea Cere¹, Elvis Hysa², Tamara Vojinovic², Carlotta Schenone¹, Carmen Pizzorni¹, Sabrina Paolino¹, Elisa Alessandri¹, Vanessa Smith³ and Maurizio Cutolo⁴, ¹Laboratory of Experimental Rheumatology and Academic Division of Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino Polyclinic Hospital, Genoa, Italy, ²Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino Polyclinic Hospital, Genova, Italy, ³Ghent University Hospital, Gent, Belgium, ⁴Laboratory of Experimental Rheumatology, Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino Polyclinic, Genova, Italy
Background/Purpose: The progressive endothelial damage is one of the hallmarks of systemic sclerosis (SSc), an autoimmune connective tissue disease characterized by organ fibrotic involvement. Aminaphtone…
Abstract Number: 2594 • ACR Convergence 2023
Trajectories of Forced Vital Capacity (FVC) in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD)
Oliver Distler¹, Madelon Vonk², Arata Azuma³, Maureen Mayes⁴, Dinesh Khanna⁵, Kristin B Highland⁶, Gerrit Toenges⁷, Margarida Alves⁸ and Yannick Allanore⁹, ¹Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ²Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands, ³Pulmonary Medicine and Oncology, Nippon Medical School, Tokyo, Japan, and Meisei Hospital, Saitama, Japan, ⁴Division of Rheumatology, University of Texas McGovern Medical School, Houston, TX, ⁵University of Michigan, Ann Arbor, MI, ⁶Cleveland Clinic, Cleveland, OH, ⁷Boehringer Ingelheim Pharma GmbH & Co. KG, Ingelheim am Rhein, Germany, ⁸Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, ⁹Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France
Background/Purpose: The SENSCIS trial enrolled patients with SSc-ILD without a requirement for them to have evidence of recent progression. During the trial, nintedanib reduced the…
Abstract Number: 0610 • ACR Convergence 2023
Nailfold Capillaroscopy for Prediction of Novel Severe Organ Involvement in Systemic Sclerosis
Cristiana Sieiro Santos¹, Rúben Rego Salgueiro², Clara Moriano Morales³, Carolina Álvarez Castro³ and Elvira Díez Álvarez³, ¹Complejo Asistencial Universitario de León, León, Spain, ²ULS Guarda, Guarda, Portugal, ³CAULE, León, Spain
Background/Purpose: Nailfold capillaroscopy (NFC) has been suggested as a potential biomarker of disease severity in systemic sclerosis (SSc). Several studies report the association between capillary…
Abstract Number: 0652 • ACR Convergence 2023
Ethnic Variations in Systemic Sclerosis Associated Interstitial Lung Disease
Patrick Donio¹, Wesley Fidler², Juan Pablo Diaz-Martinez³, Elvira Bangert⁴, Zareen Ahmad⁵, Medha Soowamber⁶ and Sindhu Johnson⁷, ¹Thunder Bay Regional Health Sciences Centre, Northern Ontario School of Medicine University, Queen's University, Thunder Bay, ON, Canada, ²Northern Ontario School of Medicine, Thunder Bay, ON, Canada, ³Toronto Western Hospital, Toronto, ON, Canada, ⁴Toronto Scleroderma Program, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, ⁵Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, University of Toronto, Toronto, ON, Canada, ⁶Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, University of Toronto, Woodbridge, ON, Canada, ⁷Institute of Health Policy, Management, and Evaluation, University of Toronto, Toronto, ON, Canada
Background/Purpose: Systemic sclerosis (SSc) is a multisystem fibrosing autoimmune disease with a high mortality rate. Ethnicity can influence prevalence and disease characteristics in SSc. Less…
Abstract Number: 0954 • ACR Convergence 2023
Non-canonical WNTA Promotes Cytoskeletal Rearrangement and Integrin Alpha V Clustering via JNK and ROCK to Control the Activation of Latent TGFβ
Thuong Trinh-Minh¹, Chih-Wei Chen², Cuong Tran Manh¹, yi-nan Li¹, Honglin Zhu³, Debomita Chakraborty⁴, Yun Zhang¹, Simon Rauber⁵, Clara Dees⁵, Christina Bergmann⁶, Alexander Kreuter⁷, Christiane Reuter⁸, Florian Groeber-Becker⁸, Beate Eckes⁹, Oliver Distler¹⁰, Andreas Ramming¹¹, Georg Schett¹² and Joerg Distler¹, ¹Clinic for Rheumatology University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University, Düsseldorf, Germany; Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University, Düsseldorf, Germany, ²3 Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany. 4 Deutsches Zentrum für Immuntherapie, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany., Erlangen, Germany, ³3 Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany. 4 Deutsches Zentrum für Immuntherapie, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany. 5 Department of Rheumatology, Xiangya Hospital, Central South University, Changsha, China, ⁴3 Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany. 4 Deutsches Zentrum für Immuntherapie, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, ⁵3 Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany. 4 Deutsches Zentrum für Immuntherapie, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany., Erlangen, Germany, ⁶Department Internal Medicine, University Hospital Erlangen, Germany, Erlangen, Germany, ⁷Clinic for Dermatology, Venereology and Allergology, HELIOS St. Elisabeth Clinic, Oberhausen, Germany, ⁸Translational Center for Regenerative Therapies, Fraunhofer Institute for Silicate Research (ISC) Würzburg, Würzburg, Germany, ⁹Translational Matrix Biology, University of Cologne, Cologne, Germany. 9 Cologne Excellence Cluster on Cellular Stress Responses in Aging-Associated Diseases (CECAD), University of Cologne, Köln, Germany, ¹⁰Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ¹¹Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany; Deutsches Zentrum für Immuntherapie, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, ¹²Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany
Background/Purpose: Systemic sclerosis (SSc) is a chronic autoimmune disorder with vasculopathy, inflammation, and fibrosis of the skin and organs. Fibrosis is caused by the abnormal…
Abstract Number: 1621 • ACR Convergence 2023
Maxillofacial MRI Augments Detection of Non-CNS Abnormalities in Pediatric Craniofacial Scleroderma
Daniel Glaser¹, Amy Metry², Elena DeRosas², Subramanian Subramanian² and Kathryn Torok³, ¹Yale University School of Medicine, New Haven, CT, ²University of Pittsburgh, Pittsburgh, PA, ³University of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA
Background/Purpose: Craniofacial scleroderma is a rare subtype of linear scleroderma affecting the head and neck. Up to 40% of patients experience extracutaneous manifestations (ECMs), but…
Abstract Number: PP16 • ACR Convergence 2023
Peer Health Coaches Help Scleroderma Patients Cope with Isolation
Mary Alore¹, Yen Chen² and Susan Murphy³, ¹Troy, MI, ²University of Michigan, Ann Arbor, MI, ³University of Michigan, Plymouth, MI
Background/Purpose: In 2011, I felt extremely isolated with my new diagnosis of diffuse systemic sclerosis and sought support online and in support groups. In addition…
Abstract Number: 0614 • ACR Convergence 2023
Self-Reported Sexual Dysfunction and Perceptions of Rheumatologist Engagement on This Issue in Patients with Systemic Sclerosis
Liza Morales¹, Robert Spiera², Jessica Gordon¹, Deanna Jannat-Khah¹ and Kimberly Lakin¹, ¹Hospital for Special Surgery, New York, NY, ²Hosptial for Special Surgery, New York, NY
Background/Purpose: Few studies have investigated the prevalence and risk factors of sexual dysfunction among individuals with systemic sclerosis (SSc). The purpose of this study is…
Abstract Number: 0655 • ACR Convergence 2023
A Novel Association Between Lipodermatosclerosis and Key Vascular Outcomes in Systemic Sclerosis
Srijana Davuluri¹, Puneet Kapoor², Swarna Nandyala², David Fiorentino³, Julia Simard⁴ and Lorinda Chung⁵, ¹Stanford University- School of Medicine, Sunnyvale, CA, ²Stanford University- School of Medicine, Palo Alto, CA, ³Department of Dermatology, Stanford University, Redwood City, CA, ⁴Department of Epidemiology & Population Health, Stanford University School of Medicine, Stanford, CA, ⁵Department of Medicine, Division of Immunology and Rheumatology, Stanford University School of Medicine, Woodside, CA
Background/Purpose: Lipodermatosclerosis (LDS) is a progressive manifestation of chronic venous insufficiency characterized by distinctive lower extremity changes, including an "inverted champagne bottle" appearance, skin hyperpigmentation,…
Abstract Number: 0955 • ACR Convergence 2023
GRB2 Serves as a Viable Target Against Skin Fibrosis in Systemic Sclerosis by Regulating Endothelial Cell Apoptosis
Yan Huang, Han Zhao, Xiangguang Shi, Jing Liu, Jui-Ming Lin, Qianqian Ma, Shuai Jiang, Weilin Pu, Yanyun Ma, Jianlan Liu, Wenyu Wu, Jiucun Wang and Qingmei Liu, Shanghai, Shanghai, China
Background/Purpose: Systemic Sclerosis (SSc) is an autoimmune disease characterized by vascular and immune system dysfunction, along with tissue fibrosis. Vascular damage has been recognized as…
Abstract Number: 1661 • ACR Convergence 2023
Spatial Transcriptional Alterations in the Cellular Landscape of Pediatric Scleroderma Skin
Kathryn Torok¹, Ying-Ju Lai², Zhongli Xu³, Anwesha Sanyal³, Giffin Werner³, Theresa Hutchins³, Claire Cheng³ and Wei Chen³, ¹University of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, ²University of Pittbsurgh, Pittsburgh, PA, ³University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Recent single-cell RNA sequencing (scRNA seq) data has been generated across several tissues in autoimmune diseases, helping to understand the cellular and molecular underpinnings…
Abstract Number: 0616 • ACR Convergence 2023
Does Systemic Sclerosis Affect the Interpretation of Mammograms? A Retrospective Cohort Study
Lea Meir¹, Tegveer Sandhu², Weiwei Chi² and Gabriela Santiago², ¹Icahn School of Medicine, New York, NY, ²Icahn School of Medicine at Mount Sinai, New York, NY
Background/Purpose: Systemic Sclerosis is an autoimmune disorder marked by thickened and hardened skin. Cutaneous cutis, the deposition of insoluble calcium salts in the skin and…
Abstract Number: 0658 • ACR Convergence 2023
Evaluating the Associations Between Autonomic Dysfunction, Clinical Phenotype and Gastrointestinal Transit in Patients with Systemic Sclerosis
Maria Paula Alvarez Hernandez¹, Brittany Adler², Jamie Perin³, Michael Hughes⁴ and Zsuzsanna McMahan⁵, ¹Hospital Clinico San Carlos, Madrid, Spain, ²Johns Hopkins University, Baltimore, MD, ³Johns Hopkins University School of Medicine, Baltimore, MD, ⁴Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Salford, United Kingdom, ⁵Johns Hopkins Rheumatology, Lutherville, MD
Background/Purpose: The gastrointestinal (GI) tract is the second most commonly impacted organ in systemic sclerosis (SSc). However, the pathogenesis and clinical expression of GI involvement…
Abstract Number: 0957 • ACR Convergence 2023
Rationale for Targeting Insulin-like Growth Factor Signalling in Systemic Sclerosis
Voon Ong¹, Shiwen Xu², Li Xue², Brajesh Kumar³ and Christopher Denton², ¹UCL Medical School Royal Free Campus, London, United Kingdom, ²University College London, London, United Kingdom, ³Horizon Therapeutics, South San Francisco, CA
Background/Purpose: Previous studies have shown that insulin like growth factor (IGF) pathways may promote extracellular matrix deposition and be upregulated in systemic sclerosis (SSc). Teprotumumab,…
Abstract Number: 1666 • ACR Convergence 2023
TNF-mediated Pulmonary Hypertension Is Marked by Aberrant Bone Morphogenic Protein (BMP) and Integrin/Basement Membrane Ligand-Receptor Signaling
Javier Rangel-Moreno¹, Maria de la Luz Garcia-Hernandez², Qingfu Xu³ and Benjamin Korman¹, ¹University of Rochester, Rochester, NY, ²University of Rochester, West Henrietta, NY, ³University of Rochester Medical Center, Rochester, NY
Background/Purpose: : We recently described TNF-transgenic mice as a novel model of pulmonary hypertension (PH) and have shown that they express altered endothelial and mesenchymal…

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