Abstracts tagged "Scleroderma"

Abstract Number: 0093 • ACR Convergence 2023
Single Cell RNA-seq and Mass Cytometry Reveal a Cytotoxic CD8 Effector T Cell Population Associated with Interstitial Lung Disease in Systemic Sclerosis Patients
Ye Cao¹, Takanori Sasaki², Richard Ainsworth³, Kim Taylor⁴, Nunzio Bottini⁵, Mehreen Elahee⁶, Edy Kim⁷, Francesco Boin³ and Deepak Rao⁷, ¹Brigham and Women's Hospital, Boston, MA, ²Brigham and Women's Hospital and Harvard Medical School, Boston, MA, ³Cedars-Sinai Medical Center, Los Angeles, CA, ⁴University of California San Francisco, San Francisco, CA, ⁵University of California, San Francisco, CA, ⁶University of Pittsburgh Medical Center, Pittsburgh, PA, ⁷Brigham and Women's Hospital, Boston, MA
Background/Purpose: Interstitial lung disease (ILD) is a major cause of morbidity and mortality in systemic sclerosis (SSc). We aimed to identify features of circulating immune…
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: 0944 • ACR Convergence 2023
Macrophages Regulate Adipocyte Differentiation and Proliferation in Skin Fibrosis
Chanhyuk Park, Helen Jarnagin, Michael Whitfield and Patricia Pioli, Geisel School of Medicine at Dartmouth, Hanover, NH
Background/Purpose: Autoimmune systemic sclerosis (SSc) is characterized by inflammation, vasculopathy, and dermal and internal organ fibrosis. A widely-reported but poorly understood aspect of SSc skin…
Abstract Number: 1527 • ACR Convergence 2023
Acute Effects of Intravenous Iloprost on Finger Power Doppler Ultrasound in Scleroderma Patients
Sofia Testoni¹, Luca Magnani², Caterina Tomassini³, Alessia Laneri³, Carlo Salvarani⁴ and PIERLUIGI MACCHIONI², ¹Università degli Studi di Modena e Reggio Emilia, San Giovanni in Persiceto, Italy, ²Azienda USL -IRCCS di Reggio Emilia, Reggio Emilia, Italy, ³Università degli Studi di Modena e Reggio Emilia, Modena, Italy, ⁴Azienda USL -IRCCS di Reggio Emilia and Università di Modena e Reggio Emilia, Reggio Emilia, Italy
Background/Purpose: So far few studies explored ultrasound (US) as a tool to assess vascular subcutaneous involvement in patients affected by systemic sclerosis (SSc). We aim…
Abstract Number: 2368 • ACR Convergence 2023
Is Skin Disease a Local Manifestation of Systemic Tissue Turnover? Serological Collagen Biomarkers Provide Important Information on Skin Diseases Arising from Mutations in Collagen Genes
Signe Holm Nielsen¹, Matej Anđelić², Dovile Sinkeviciute¹, Anne-Christine Bay-Jensen¹ and Morten Karsdal¹, ¹Nordic Bioscience, Herlev, Denmark, ²Nordic Bioscience, Copenhagen, Denmark
Background/Purpose: Collagens are the main constituents of the skin. Genetic mutations in type VI, VII, and XVII collagen cause skin diseases, such as atopic dermatitis,…
Abstract Number: 0283 • ACR Convergence 2023
Transcriptomic Profiles in Muscle Biopsies from Systemic Sclerosis Patients with Different Autoantibodies
Maria Casal-Dominguez¹, Jose Cesar Milisenda², Iago Pinal-Fernandez¹, Katherine Pak³, Sandra Muñoz-Braceras¹, Jose Jiram Torres-Ruiz¹, Stefania Dell´Orso⁴, Faiza Naz⁴, Gustavo Gutierrez-Cruz⁴, Shamima Islam¹, Yaiza Duque-Jaimez², Ana Matas-Garcia², Francesc J Garcia-Garcia², Mariona Guitart-Manpel², Gloria Garrabou², Ernesto Trallero-Araguas⁵, Brian Wallit⁶, Lisa Christopher-Stine⁷, Tom Lloyd⁸, Alfredo Guillen-Del-Castillo⁹, Carmen Pilar Simeon-Aznar¹⁰, Josep Maria Grau², Albert Selva-O’Callaghan⁵ and Andrew Mammen¹¹, ¹Muscle Disease Unit, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ²Muscle Research Unit, Internal Medicine Service, Hospital Clinic, Barcelona, Spain, ³National Institutes of Health, Bethesda, MD, ⁴National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ⁵Systemic Autoimmune Disease Unit, Vall d’Hebron Institute of Research, Barcelona, Spain, ⁶National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, MD, ⁷Johns Hopkins University, Baltimore, MD, ⁸Johns Hopkins University School of Medicine, Baltimore, MD, ⁹Unit of Autoimmune Diseases, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain, ¹⁰Department of Internal Medicine, Systemic Autoimmune Diseases Unit, Hospital Universitario Vall d'Hebronh, Barcelona, Spain, ¹¹NIH, Bethesda, MD
Background/Purpose: The inflammatory myopathies (IM) include dermatomyositis (DM), antisynthetase syndrome (AS), immune-mediated necrotizing myopathy (IMNM), inclusion body myositis (IBM), and overlap myositis (OM), in which…
Abstract Number: 0641 • ACR Convergence 2023
The Evaluation of Lower Extremities Arterial Disease in Systemic Sclerosis Patients Through Rheumatologist-Vascular Surgeon Multidisciplinary Management: Preliminary Data from an Italian Single Center Study
Maria Grazia Lazzaroni¹, Liala Moschetti¹, Eleonora Pedretti¹, Paolo Baggi², Franco Franceschini³, Stefano Bonardelli² and Paolo Airò¹, ¹Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, University of Brescia, Brescia, Italy, ²Vascular Surgery, ASST Spedali Civili di Brescia, University of Brescia, Brescia, Italy, ³Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy
Background/Purpose: The occurrence of lower limbs macrovascular complications configuring lower extremities arterial disease (LEAD) has been reported in Systemic Sclerosis (SSc) patients. Recently, digital ulcers…
Abstract Number: 0945 • ACR Convergence 2023
The Nuclear Receptor TR4 Orchestrates Cytoskeletal Organization in a Gα12/ROCK-dependent Manner to Promote Myofibroblast Differentiation and Tissue Fibrosis in Systemic Sclerosis
Yun Zhang¹, Lichong Shen², yi-nan Li¹, Hermina Györfi¹ 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, ²Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany
Background/Purpose: Members of the superfamily of nuclear receptors have been implicated in inflammatory processes and pathologic tissue remodeling and have emerged as attractive targets for…
Abstract Number: 1528 • ACR Convergence 2023
Intravenous or Subcutaneous Immunoglobulins as Potential Treatment for Gastrointestinal, Cutaneous and Vascular Involvement in Systemic Sclerosis: Data from an Italian Cohort of 65 Patients
Francesco Bonomi¹, Arianna Damiani¹, Carmela Coccia¹, Juela Levani¹, elisa Fiorentini², silvia peretti¹, Gemma Lepri¹, martina orlandi¹, francesca bartoli¹, marco Matucci Cerinic³, silvia bellando randone¹ and serena guiducci¹, ¹Department of Experimental and Clinical medicine, Division of Rheumatology, Azienda Ospedaliero Universitaria Careggi, University of Florence, Florence, Italy, ²Department of Experimental and Clinical medicine, Division of Rheumatology, Azienda Ospedaliero Universitaria Careggi, University of Florence, Firenze, Italy, ³Unit of Immunology, Rheumatology, Allergy and Rare diseases, IRCCS San Raffaele Hospital, Milan, Milan, Italy
Background/Purpose: Treatment of systemic sclerosis (SSc) is challenging, and in the last 2 decades there has been an increasing interest for immunoglobulin (Igs) treatment as…
Abstract Number: 2369 • ACR Convergence 2023
Investigating Macrophage Heterogeneity in the Esophagus of SSc Patients
Hadijat Makinde¹, Matthew Dapas², Salina Dominguez², Tyler Therron¹, Miranda Gurra¹, Mary Karns³, Kathleen Aren³, Alexandra Soriano², Lutfiyya Muhammad⁴, Carla Cuda¹, Dustin Carlson¹, Jane Dematte⁵, Darren Brenner¹, John Pandolfino⁶, Harris Perlman¹, Deborah Winter⁷ and Marie-Pier Tetreault¹, ¹Northwestern University, Chicago, IL, ²Northwestern University FSM Division of Rheumatology, Chicago, IL, ³Northwestern University Division of Rheumatology, Chicago, IL, ⁴Northwestern University Feinberg School of Medicine, Chicago, IL, ⁵Northwestern University, Elmhurst, IL, ⁶Northwestern University, Feinberg School of Medicine, Wilmette, IL, ⁷Northwestern University, Skokie, IL
Background/Purpose: Systemic sclerosis (SSc) is characterized by an initial inflammatory phase followed by fibrosis. Esophageal dysfunction in SSc is associated with Gastroesophageal Reflux Disease (GERD),…
Abstract Number: 0332 • ACR Convergence 2023
Initial Characterization of a Skin Symptom Questionnaire for Patients with Systemic Sclerosis
Jeong Min Yu¹, John VanBuren², Angela Child², Jessica Alvey², Lisa Mandl³, Laura Pinheiro⁴, Shervin Assassi⁵, Elana Bernstein⁶, Flavia Castelino⁷, Lorinda Chung⁸, Luke Evnin⁹, Tracy Frech¹⁰, Faye Hant¹¹, Laura Hummers¹², Dinesh Khanna¹³, Kimberly Lakin¹, Dorota Lebiedz-Odrobina¹⁴, Yiming Luo¹⁵, Ashima Makol¹⁶, Jerry Molitor¹⁷, Duncan Moore¹⁸, Carrie Richardson¹⁹, Nora Sandorfi²⁰, Ami Shah²¹, Ankoor Shah²², Victoria Shanmugam²³, Brian Skaug²⁴, Virginia Steen²⁵, Elizabeth Volkmann²⁶ and Jessica Gordon¹, ¹Hospital for Special Surgery, New York, NY, ²University of Utah, Salt Lake City, UT, ³Hospital for Special Surgery, Weill Cornell Medicine, New York, NY, ⁴Weill Cornell Medicine College, New York, NY, ⁵University of Texas McGovern Medical School at Houston, Houston, TX, ⁶Columbia University, New York, NY, ⁷Massachusetts General Hospital, Boston, MA, ⁸Department of Medicine, Division of Immunology and Rheumatology, Stanford University School of Medicine, Woodside, CA, ⁹Scleroderma Research Foundation, Brisbane, CA, ¹⁰Vanderbilt University Medical Center, Nashville, TN, ¹¹Medical University of South Carolina, Charleston, SC, ¹²Johns Hopkins University, Baltimore, MD, ¹³University of Michigan, Ann Arbor, MI, ¹⁴University of Utah, Cottonwood Heights, UT, ¹⁵Division of Rheumatology, Department of Medicine, Columbia University Irving Medical Center, New York, NY, ¹⁶Mayo Clinic, Rochester, MN, Rochester, MN, ¹⁷University of Minnesota, Minneapolis, MN, ¹⁸Northwestern Memorial Hospital, Chicago, IL, ¹⁹Northwestern University, Riverside, IL, ²⁰University of Pennsylvania, Philadelphia, PA, ²¹Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, Ellicott City, MD, ²²Duke University, Durham, NC, ²³Victoria Shanmugam, MD, Great Falls, VA, ²⁴Division of Rheumatology, University of Texas McGovern Medical School, Houston, TX, ²⁵Georgetown University School of Medicine, Washington, DC, ²⁶University of California Los Angeles, Los Angeles, CA
Background/Purpose: Skin disease is a hallmark of systemic sclerosis (SSc). The modified Rodnan skin score (mRSS) is physician performed measurement that assesses the extent and…
Abstract Number: 0642 • ACR Convergence 2023
Clinical Characteristics of Systemic Sclerosis, Systemic Sclerosis Overlap Syndromes and Systemic Sclerosis-mixed Connective Tissue Disease in an Asia-Pacific Cohort- an APLAR Scleroderma Special Interest Group Study
Sue-Ann Ng¹, Seyed Ehsan Saffari², Wendy Stevens³, JASMIN RAJA⁴, Suparaporn Wangkaew⁵, Grace Ho⁶, Gim Gee Teng⁷, Susanna Proudman⁸, Mandana Nikpour⁹ and Andrea Hsiu Ling Low¹⁰, ¹Singapore General Hospital, Singapore, Singapore, ²Center for Quantitative Medicine, Duke-NUS Medical School, National University of Singapore, Singapore, Singapore, ³Department of Rheumatology, St Vincent's Hospital Melbourne, Fitzroy, Australia, ⁴Division of Rheumatology, Department of Medicine, University Malaya, Malaysia, Subang Jaya, Malaysia, ⁵Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand, ⁶Department of Medicine, Queen Elizabeth Hospital, Hong Kong, Hong Kong, Hong Kong, ⁷Alexandra Hospital, Department of Medicine, Division of Rheumatology, National University Health System, Singapore, Singapore, ⁸Royal Adelaide Hospital and University of Adelaide, Adelaide, Australia, ⁹The University of Melbourne at St. Vincent’s Hospital Melbourne, Departments of Medicine and Rheumatology, Melbourne, Australia, ¹⁰Department of Rheumatology and Immunology, Singapore General Hospital, Singapore, Singapore
Background/Purpose: Systemic sclerosis (SSc) patients with clinical features of another connective tissue disease (CTD) may have different clinical characteristics from patients with SSc alone. Our…
Abstract Number: 0950 • ACR Convergence 2023
KL-6 and IL-18 Levels Are Negatively Correlated with Respiratory Function Tests and ILD Extent Assessed on HRCT in Patients with Systemic Sclerosis-related Interstitial Lung Disease (SSc-ILD)
Cristiana Sieiro Santos¹, Sara Calleja², Javier de la Calle Lorenzo², Carmen López Garay², Clara Moriano², Elena Bollo de miguel² and Elvira Díez Álvarez², ¹Complejo Asistencial Universitario de León, León, Spain, ²CAULE, León, Spain
Background/Purpose: Interstitial lung disease (ILD) is one of the leading causes of mortality in patients with systemic sclerosis (SSc). Serum biomarkers have been suggested as…
Abstract Number: 1530 • ACR Convergence 2023
Outcomes in Patients with Systemic Sclerosis Following Lung Transplantation: An Italian Multicentre Experience
Claudia Iannone¹, Maria Rosa Pellico¹, Letizia Corinna Corinna Morlacchi², Valeria Rossetti², Marco Vicenzi², Paolo Airò³, Marta Saracco⁴, Annamaria iagnocco⁴, Lorenzo Beretta⁵, Adriana Severino⁵, Eleonora Zaccara⁶, paola Faggioli⁶, Fabio Cacciapaglia⁷, Stefano Stano⁸, Silvia Cavalli⁹, Antonina Minniti⁹, giorgia Trignani⁹, francesco Blasi⁵, Roberto F. Caporali¹⁰ and Nicoletta Del Papa¹¹, ¹ASST Gaetano Pini CTO, Milan, Italy, ²Policlinico di Milano, Milan, Italy, ³Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, University of Brescia, Brescia, Italy, ⁴AO Mauriziano di Torino, Torino, Italy, ⁵Policlinico di Milano, Milano, Italy, ⁶Legnano-ASST-Ovest Milanese, Legnano, Italy, ⁷Rheumatology Unit, Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J), University of Bari, Bari, Italy, ⁸Policlinico di Bari, Bari, Italy, ⁹ASST Gaetano Pini CTO, Milano, Italy, ¹⁰Department of Clinical Sciences and Community Health, University of Milan, and Department of Rheumatology and Medical Sciences, ASST Gaetano Pini-CTO, Milano, Italy, ¹¹Scleroderma Clinic, Dipartimento di Reumatologia, ASST Gaetano Pini CTO, Milano, Italy
Background/Purpose: Lung transplantation (LT) is gaining ground in managing advanced ILD in SSc patients. However, concerns remain among surgeons due to SSc's complexity, multiorgan involvement…
Abstract Number: 2593 • ACR Convergence 2023
FT011 for the Treatment of Systemic Sclerosis. Results from a Phase II Study
Christopher Denton¹, Wendy Stevens², Nicole Kruger³, Michelle Papadimitriou⁴, Fay Khong⁴, Michelle Bradney³, Darren Kelly⁴ and Robert Lafyatis⁵, ¹University College London, London, United Kingdom, ²Department of Rheumatology, St Vincent's Hospital Melbourne, Fitzroy, Australia, ³Certa Therapeutics, Melbourne, Australia, ⁴Certa Therapeutics and Melbourne University, Melbourne, Australia, ⁵University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Systemic Sclerosis (SSc) is an autoimmune disease characterized by vascular damage, inflammation, and fibrosis of the skin and organs, with no approved disease modifying…

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