Abstracts tagged "Scleroderma, Systemic"

Abstract Number: 1364 • ACR Convergence 2021
Autologous Hematopoietic Stem Cell (HSC) Transplantation for Systemic Sclerosis, North American Registry: Updated Outcomes and the Impact of CD34+ HSC Enrichment
George Georges¹, Keith Sullivan², Dinesh Khanna³, Matthew Mei⁴, Maureen Mayes⁵, Dan Furst⁶, Mark Wener⁷, Suzanne Kafaja⁸, Gwynn Long², Vaishali Sanchorawala⁹, Kareem Jamani¹⁰, Attaphol Pawarode³, Ashish Masurekar¹¹, Brian Lam¹², Richard Nash¹³, Marcin Trojanowski⁹, Chitra Hosing¹⁴, Philip Clements¹⁵, Tanya Helms², Bernadette McLaughlin¹⁶, Linda Griffith¹⁷, Kyle Hebert¹⁸, Mary Eapen¹⁸, Jan Storek¹⁰ and Harold Atkins¹¹, ¹Fred Hutchison Cancer Research Center and University of Washington, Seattle, WA, ²Duke University School of Medicine, Durham, NC, ³University of Michigan, Ann Arbor, MI, ⁴City of Hope Comprehensive Cancer Center, Duarte, CA, ⁵Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, TX, ⁶University of California Los Angeles, Los Angeles, CA, ⁷University of Washington, Seattle, WA, ⁸University of California Los Angeles Division of Rheumatology, Los Angeles, CA, ⁹Boston University School of Medicine, Boston, MA, ¹⁰University of Calgary, Calgary, AB, Canada, ¹¹Ottawa Hospital Research Institute, Ottawa, ON, Canada, ¹²University of Texas McGovern Medical School, Houston, TX, ¹³Colorado Blood Cancer Institute, Denver, CO, ¹⁴University of Texas MD Anderson Cancer Center, Houston, TX, ¹⁵University of California Los Angeles School of Medicine, Department of Rhemumatology, Los Angeles, CA, ¹⁶Fred Hutchison Cancer Research Center, Seattle, WA, ¹⁷Division of Allergy Immunology and Transplantation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Rockville, MD, ¹⁸Medical College of Wisconsin, Milwaukee, WI
Background/Purpose: Autologous hematopoietic stem cell transplantation (AHSCT) is now established as a preferred standard of care treatment for patients with severe scleroderma with internal organ…
Abstract Number: 0405 • ACR Convergence 2021
Retrospective Study on the Prognostic Value of Cardiac Magnetic Resonance Imaging Abnormalities in Systemic Sclerosis
Hao Cheng Shen¹, Catherine Faucher², Anne Chin³, Carl Chartrand-Lefebvre³, Ragui Chehata⁴, Julia Cadrin-Tourigny⁵, Francois-Pierre Mongeon⁶, Jean-Paul Makhzoum⁷, Océane Landon-Cardinal⁸, Josiane Bourré-Tessier⁸, Éric Rich⁹, Jean-Richard Goulet⁸, Paul R Fortin¹⁰, Jean-Luc Senécal⁸ and Sabrina Hoa⁸, ¹Department of Medicine, Université de Montréal, Montréal, QC, Canada, ²Department of Medicine, Université Laval, Québec, QC, Canada, ³Department of Radiology, Centre hospitalier de l’Université de Montréal, Montréal, QC, Canada, ⁴Department of Medicine, Université de Montréal, Montreal, Montréal, QC, Canada, ⁵Division of Non-Invasive Cardiology, Department of Specialized Medicine, Montreal Heart Institute; Department of Medicine, Université de Montréal, Montréal, QC, Canada, ⁶Division of electrophysiology, Department of Specialized Medicine, Montreal Heart Institute; Department of Medicine, Université de Montréal, Montréal, QC, Canada, ⁷Division of Internal Medicine, Hôpital Sacré-Coeur de Montréal; Department of Medicine, Université de Montréal, Montréal, QC, Canada, ⁸Division of Rheumatology, Centre hospitalier de l'Université de Montréal; Department of Medicine, Université de Montréal, Montréal, QC, Canada, ⁹Division of Rheumatology, Centre hospitalier de l’Université de Québec-Université Laval, Montréal, QC, Canada, ¹⁰CHU de Quebec - Universite Laval, Québec City, QC, Canada
Background/Purpose: Cardiac involvement is a leading cause of death in systemic sclerosis (SSc). Cardiac MRI (CMR) is useful in the early assessment of cardiac disease.…
Abstract Number: 1369 • ACR Convergence 2021
Adipose Tissue-Derived Stromal Vascular Fraction Plus Fat Grafts for Hand Therapy in Patients with Systemic Sclerosis. a Randomized Controlled Clinical Trial
Martín Iglesias¹, Iván Torre-Villalvazo¹, Armando Tovar-Palacio¹, Alejandro Zentella-Dehesa¹, Patricia Butrón-Gandarillas¹, Marta Durand-Carbajal¹, Erik Torre-Anaya², Martha Guevara² and Tatiana Rodriguez-Reyna², ¹Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, ²Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico
Background/Purpose: Adipose tissue-derived Stromal Vascular Fraction (ADSVF) has been proposed as regenerative treatment for hand deformities and digital ulcer healing in patients with Systemic Sclerosis…
Abstract Number: 0408 • ACR Convergence 2021
A Histogram-based Densitometry Index to Support the Identification and the Assessment of Severity of Interstitial Lung Disease in Systemic Sclerosis: Applicability in Conventional and Low-dose Computed Tomography
COSIMO BRUNI¹, Alexandru Garaiman², Lorenzo Tofani³, Suzana Jordan⁴, Carmen-Marina Mihai⁴, Rucsandra Dobrota⁴, Muriel ELHAI⁵, Mike Oliver Becker⁴, Marco Matucci-Cerinic³ and Oliver Distler⁶, ¹University of Florence, Department of Rheumatology, Florence, Italy & Department of Rheumatology, University Hospital Zurich, University of Zurich, Switzerland, Florence, Italy, ²University Hospital Zurich, University of Zurich, Zurich, Switzerland, ³University of Florence, Florence, Italy, ⁴Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁵Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland, ⁶Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich/University of Zurich, Zurich, Switzerland
Background/Purpose: High resolution computed tomography (conventional CT) represents the diagnostic gold standard for systemic sclerosis-related interstitial lung disease (SSc-ILD). A low-dose 9-slices (reduced) CT protocol…
Abstract Number: 1376 • ACR Convergence 2021
Outcome Reporting in Systemic Sclerosis-Related Digital Ulcers: A Scoping Review
Michael Hughes¹, Nancy Maltez², Edith Brown³, Virginia Hickey⁴, Heiyoung Park⁵, Beverley Shea², John Pauling⁶, Susanna Proudman⁷, Peter Merkel⁸ and Ariane Herrick⁹, ¹Tameside and Glossop Integrated NHS Foundation Trust, Manchester, United Kingdom, ²University of Ottawa, Ottawa, ON, Canada, ³n/a, Manchester, United Kingdom, ⁴n/a, Adelaide, Australia, ⁵5. National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, ⁶Royal National Hospital for Rheumatic Diseases, Bath, United Kingdom, ⁷University of Adelaide, Medindie, Australia, ⁸University of Pennsylvania, Philadelphia, PA, ⁹University of Manchester, Salford, United Kingdom
Background/Purpose: Digital ulcers (DUs) are a major cause of pain and disability in patients with systemic sclerosis (SSc). Despite the availability of a range of…
Abstract Number: 0411 • ACR Convergence 2021
Pharmacokinetics, Safety, and Efficacy of Subcutaneous Brodalumab for Systemic Sclerosis with Moderate-to-severe Skin Thickening: A Single-arm, Open-label, Multi-dose, Phase 1 Trial
Takemichi Fukasawa¹, Ayumi Yoshizaki², Satoshi Ebata² and Shinichi Sato², ¹Department of Dermatology, The University of Tokyo, Graduate School of Medicine, Tokyo, Japan, ²University of Tokyo Graduate School of Medicine, Tokyo, Japan
Background/Purpose: The exact role of interleukin-17 in systemic sclerosis (SSc) has not been established. This trial assessed the pharmacokinetics (PK), safety, and efficacy of multiple…
Abstract Number: 1377 • ACR Convergence 2021
Assessment of Autoantibodies and Clinical Associations in SSc Patients with ANA Positivity & Negative for Prototypic Autoantibodies
Karen Kruzer¹, Roberta G Marangoni¹, Ilana Heckler², Aya Elhage², Varga John³, Monique Hinchcliff⁴, Mary Carns⁵, Kathleen Aren⁵, Amy Wielgosz¹, Marc Nuzzo⁶, Iswariya Venkataraman² and Benjamin Korman¹, ¹University of Rochester, Rochester, NY, ²EUROIMMUN US, Mountain Lakes, NJ, ³University of Michigan, Ann Arbor, MI, ⁴Yale School of Medicine, Westport, CT, ⁵Northwestern University Division of Rheumatology, Chicago, IL, ⁶Division of Allergy, Immunology, and Rheumatology, School of Medicine and Dentistry, University of Rochester Medical Center, Rochester, NY
Background/Purpose: Systemic sclerosis (SSc) is a clinically heterogenous disease typically characterized by a positive ANA (ANA+), and prototypical antibodies including anti-centromere, anti-topoisomerase, and anti-RNA polymerase…
Abstract Number: 0413 • ACR Convergence 2021
Predictors of ILD Development and Timing of Onset in Systemic Sclerosis: A Canadian Cohort
Jessica Kapralik, Robert Morton, Malik Farooqi, Karen Beattie, Nathan Hambly and Maggie Larche, McMaster University, Hamilton, ON, Canada
Background/Purpose: In patients with SSc, interstitial lung disease (SSc-ILD) and pulmonary hypertension affect 50-70% of patients and are the leading cause of death. Recent trials…
Abstract Number: 1379 • ACR Convergence 2021
Serum Levels of the Soluble Receptor for Advanced Glycation Endproducts Are Prospectively Associated with Pulmonary Arterial Hypertension in Systemic Sclerosis
Isabella Atzeni¹, Yehya Al-Adwi², Berber Doornbos-van der Meer², Anniek Van Roon², Caroline Roozendaal¹, Andries Smit², Alja J. Stel², Harry Van Goor², Tji-Joong Gan¹, Johanna Westra² and Douwe J Mulder², ¹University of Groningen, University Medical Centre Groningen, Groningen, Groningen, Netherlands, ²University of Groningen, University Medical Centre Groningen, Groningen, Netherlands
Background/Purpose: Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are the leading causes of death in Systemic Sclerosis (SSc). Markers for early detection of…
Abstract Number: 0500 • ACR Convergence 2021
Clinical Phenotypes of Patients with Systemic Sclerosis with Distinct Molecular Signatures in the Skin
Monica Yang¹, Vivien Goh², Monica Espinoza³, Yiwei Yuan⁴, Julia Lee⁵, Mary Carns⁶, Dinesh Khanna⁷, Zsuzsanna McMahan⁸, Michael Whitfield⁹ and Monique Hinchcliff¹⁰, ¹University of California San Francisco, San Francisco, CA, ²Northwestern University, Chicago, IL, ³Geisel School of Medicine, Hanover, NH, ⁴Dartmouth College, Hanover, NH, ⁵Northwestern University Feinberg School of Medicine, Chicago, IL, ⁶Northwestern University Division of Rheumatology, Chicago, IL, ⁷University of Michigan, Ann Arbor, MI, ⁸Johns Hopkins Rheumatology, Baltimore, MD, ⁹Geisel School of Medicine, Lebanon, NH, ¹⁰Yale School of Medicine, Westport, CT
Background/Purpose: Although two subsets in systemic sclerosis (SSc) have been identified based on degree of skin disease, the current classification system, limited vs. diffuse cutaneous,…
Abstract Number: 1382 • ACR Convergence 2021
Survey on Treatment Practices in Subclinical Interstitial Lung Disease in Systemic Sclerosis
Sabrina Hoa¹, Murray Baron² and Marie Hudson³, ¹Division of Rheumatology, Centre hospitalier de l'Université de Montréal; Department of Medicine, Université de Montréal, Montréal, QC, Canada, ²Jewish General Hospital, Montréal, QC, Canada, ³McGill University, Montréal, QC, Canada
Background/Purpose: Interstitial lung disease (ILD) is a leading cause of mortality in systemic sclerosis (SSc). Early detection and treatment of SSc-ILD may lead to improved…
Abstract Number: 0541 • ACR Convergence 2021
N-formyl Methionine Peptide-mediated Neutrophil Activation in Systemic Sclerosis
Runa Kuley¹, Ryan Stultz², Bhargavi Duvvuri², Roger Hesselstrand³, J Lee Nelson⁴ and Christian Lood², ¹Division of Rheumatology, University of Washington, Seattle, WA, ²University of Washington, Seattle, WA, ³Lund University, Lund, Sweden, ⁴Fred Hutchinson Cancer Research Center, University of Washington, Seattle, WA
Background/Purpose: Systemic sclerosis (SSc) is a complex systemic autoimmune disease, and its etiology is unknown. Exaggerated neutrophil activation and formation of neutrophil extracellular traps (NETs)…
Abstract Number: 1383 • ACR Convergence 2021
Prediction of Digital Ulcers in Patients with Systemic Sclerosis Based on the Use of Platelet Inhibitors and Other Parameters – A EUSTAR Study on Derivation and Validation of a Clinical Prediction Model
Alexandru Garaiman¹, Klaus Steigmiller², Catherine Gebhard³, Marco Matucci-Cerinic⁴, Jorg Henes⁵, Jeska de Vries-Bouwstra⁶, Vanessa Smith⁷, Andrea Doria⁸, Yannick Allanore⁹, Lorenzo Dagna¹⁰, Branimir Anic¹¹, Carlomaurizio Montecucco¹², Otylia Kowal-Bielecka¹³, Mickael Martin¹⁴, Yoshiya Tanaka¹⁵, Anna-Maria Hoffmann-Vold¹⁶, Ulrike Held², Oliver Distler¹⁷ and Mike Oliver Becker¹⁸, ¹University Hospital Zurich, University of Zurich, Zurich, Switzerland, ²Epidemiology, Biostatistics and Prevention Institute, Department of Biostatistics, University of Zurich, Zurich, Switzerland, ³Department of Nuclear Medicine, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁴University of Florence, Florence, Italy, ⁵University Hospital Tuebingen, Tuebingen, Germany, ⁶Leiden University Medical Center, Leiden, Netherlands, ⁷Department of Rheumatology and Internal Medicine, Ghent University Hospital, Ghent, Belgium, ⁸University of Padova, Padova, Italy, ⁹Université de Paris, Paris, France, ¹⁰10Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Milano, Italy, ¹¹Division of Clinical Immunology and Rheumatology, Department of Internal Medicine, University Hospital Centre Zagreb and University of Zagreb, School of Medicine, Zagreb, Croatia, ¹²Department of Rheumatology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy, ¹³Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland, ¹⁴Internal Medicine, Poitiers University Hospital, Poitiers, France, ¹⁵University of Occupational and Environmental Health, Kitakyushu, Japan, ¹⁶Department of Rheumatology, Oslo University Hospital, Oslo, Norway, ¹⁷Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich/University of Zurich, Zurich, Switzerland, ¹⁸Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland
Background/Purpose: Digital ulcers (DUs) affect half of the patients with systemic sclerosis (SSc) and can be complicated by gangrene and amputation. Platelets are known to…
Abstract Number: 0544 • ACR Convergence 2021
Targeting CD13/aminopeptidase N as a Novel Therapeutic Approach for Scleroderma Fibrosis
Eliza Pei-Suen Tsou, M.Asif Amin, Phillip Campbell, Mikel Gurrea-Rubio, Morgan Omara, Ellen Model, Pamela Palisoc, Mustafa Ali, Sirapa Vichaikul, Jonatan Hervoso, Jeffrey Ruth, Dinesh Khanna and David Fox, University of Michigan, Ann Arbor, MI
Background/Purpose: Aminopeptidase N, also known as CD13, is a Zn2+-dependent membrane bound ectopeptidase widely expressed in mammalian cells including rheumatoid arthritis (RA) fibroblast-like synoviocytes (FLS),…
Abstract Number: 1389 • ACR Convergence 2021
Subclinical ILD Is Frequent and Progresses Across Different Connective Tissue Diseases
Anna-Maria Hoffmann-Vold¹, Helena Andersson², Silje Reiseter², Håvard Fretheim², Imon Barua¹, Torhild Garen³, Øyvind Midtvedt¹, Ragnar Gunnarsson², Michael Durheim², Trond Mogens AAløkken² and Øyvind Molberg¹, ¹Department of Rheumatology, Oslo University Hospital, Oslo, Norway, ²Oslo University Hospital, Oslo, Norway, ³Department of Rheumatology, Oslo University Hospital, Oslo, Nepal
Background/Purpose: Based on the argument that symptoms-define-disease, physicians commonly apply the terms pre-clinical or sub-clinical disease to describe patients with disease-related findings, but no accompanying…

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