Abstracts tagged "Scleroderma, Systemic"

Abstract Number: 2455 • ACR Convergence 2024
Improvement Across Multi-organ Domains and Patient Reported Outcomes in Refractory Juvenile-Onset Systemic Sclerosis (jSSc) up to 4 Years After Autologous Stem Cell Transplantation (ASCT)
Jonathan Li¹, Paulina Horvei², Franziksa Rosser³, Kirsten Rose-Felker⁴, Vibha Sood⁵, Adam Olson⁶, Vickie Vandergrift⁷, Nicole Hogue², Lauren Farver⁸, Devin Mcguire⁹, Haley Havrilla⁷, Jessie Alexander¹⁰, Shawna McIntyre², Paul Szabolcs² and Kathryn Torok¹¹, ¹Division of Rheumatology, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ²Division of Blood and Marrow Transplantation and Cellular Therapies, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ³Division of Pulmonology Medicine, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ⁴Division of Cardiology, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁵Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁶Department of Radiation Oncology, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁷Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁸Department of Physical Therapy, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁹Pediatric Behavioral Health, University of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ¹⁰Pediatric Bone Marrow Transplant and Cellular Therapies, Stanford Children's Hospital, Palo Alto, CA, USA, Pittsburgh, ¹¹Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA
Background/Purpose: Juvenile-onset systemic sclerosis (jSSc) is a systemic autoimmune disease characterized by vasculopathy and multiorgan fibrosis leading to significant morbidity and early mortality. Autologous stem…
Abstract Number: L06 • ACR Convergence 2023
Improvement in Clinical and Patient-Reported Outcomes for Refractory Juvenile-Onset Systemic Sclerosis (jSSc) 6 Months to 2 Years After Autologous Stem Cell Transplantation (ASCT)
Kathryn Torok¹, Paulina Horvei¹, Franziska Rosser¹, Kirsten Rose-felker², Vibha Sood², Adam Olsen², Nicole Hogue², Vickie Vandergrift², Lauren Farver², Devin Mcguire², Jonathan Li³, Haley Havrilla², Jessie Alexander⁴, Shawna McIntyre² and Paul Szabolcs¹, ¹University of Pittsburgh; UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, ²UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, ³University of Pittsburgh Medical Center, Pittsburgh, PA, ⁴Stanford Children's Hospital, Palo Alto, CA
Background/Purpose: Juvenile-onset systemic sclerosis (jSSc) is an inflammatory, fibrotic, and vasculopathic disease that causes severe multi-organ dysfunction leading to significant morbidity and early mortality.When patients…
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: 1523 • ACR Convergence 2023
The Risk of Scleroderma Renal Crisis from Intraarticular Corticosteroid Injection in Systemic Sclerosis
Muruganandam Maheswari, Eyerusalem Akpan, Matthew Mcelwee, Meredith Keller, Angie Ariza - Hutchinson, Rosemina Patel, Wilmer Sibbitt, Frank O'Sullivan, Sharon nunez, N. Suzanne Emil and Roderick Fields, University of New Mexico, Albuquerque, NM
Background/Purpose: Scleroderma renal crisis (SRC) is a rare but a life-threatening complication of systemic sclerosis (SSc), affecting 2-15% of patients with SSc. SRC has been…
Abstract Number: 0628 • ACR Convergence 2023
Interstitial Lung Disease in Very Early Systemic Sclerosis
Sinziana Muraru¹, Carina Mihai¹, Anna-Maria Hoffmann-Vold², Cosimo Bruni¹, Muriel Elhai³, Mike Becker⁴, Suzana Jordan¹, Alexandru Garaiman¹, Oliver Distler¹ and Rucsandra Dobrota¹, ¹Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ²Oslo University Hospital, Oslo, Norway, ³Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland, ⁴Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland
Background/Purpose: Interstitial lung disease (ILD) affects around 50% of patients with systemic sclerosis (SSc) and is the main cause of mortality. Preliminary data from small…
Abstract Number: 1526 • ACR Convergence 2023
Evusheld Efficacy and Safety in Autoimmune Rheumatic Diseases (AIRD) Treated by B Cell Depeting Therapy or Autologous Stem Cell Transplantation: A Prospective Observational Study
Lisa Kaly¹, mark Volevich¹, shiri keret², Abid Awisat³, Aniela Shouval¹, Itzhak Rosner⁴, Michel Rozenbaum¹, Nina Boulman¹ and Doron Rimar¹, ¹Bnai Zion Medical Center, Haifa, Israel, ²Bnai Zion, Atlit, Israel, ³Bney-Zion Medical Center, Baqa Elgharbiya, Israel, ⁴Bnai Zion Medical Center/Technion, Haifa, Israel
Background/Purpose: B cell depleting therapy (rituximab) and Autologous Hematopoietic Stem Cell transplantation (AHSCT) result in impaired ability to mount a humoral immune response. Evusheld (tixagevimab…
Abstract Number: 0630 • ACR Convergence 2023
Prevalence and Risk Factors for Systemic Sclerosis Digital Ischemic Complications in the Collaborative National Quality and Efficacy Registry
Marissa Savoie¹, Monica Harding², Jessica Alvey², John VanBuren², Shervin Assassi³, Elana Bernstein⁴, Lorinda Chung⁵, Luke Evnin⁶, Tracy Frech⁷, Jessica Gordon⁸, Faye Hant⁹, Laura Hummers¹⁰, Dinesh Khanna¹¹, Kimberly Lakin⁸, Dorota Lebiedz-Odrobina¹², Yiming Luo¹³, Ashima Makol¹⁴, Jerry Molitor¹⁵, Duncan Moore¹⁶, Carrie Richardson¹⁷, Nora Sandorfi¹⁸, Ami Shah¹⁹, Ankoor Shah²⁰, Brian Skaug²¹, Virginia Steen²², Elizabeth Volkmann²³ and Flavia Castelino¹, ¹Massachusetts General Hospital, Boston, MA, ²University of Utah, Salt Lake City, UT, ³University of Texas McGovern Medical School at Houston, Houston, TX, ⁴Division of Rheumatology, Department of Medicine, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, NY, ⁵Department of Medicine, Division of Immunology and Rheumatology, Stanford University School of Medicine, Woodside, CA, ⁶Scleroderma Research Foundation, Brisbane, CA, ⁷Vanderbilt University Medical Center, Nashville, TN, ⁸Hospital for Special Surgery, New York, NY, ⁹Medical University of South Carolina, Charleston, SC, ¹⁰Johns Hopkins University, Baltimore, MD, ¹¹University of Michigan, Ann Arbor, MI, ¹²University of Utah, Cottonwood Heights, UT, ¹³Division of Rheumatology, Department of Medicine, Columbia University Irving Medical Center, New York, NY, ¹⁴Mayo Clinic, Rochester, MN, Rochester, MN, ¹⁵University of Minnesota, Minneapolis, MN, ¹⁶Northwestern University, Department of Rheumatology, Chicago, IL, ¹⁷Northwestern University, Chicago, IL, ¹⁸University of Pennsylvania, Philadelphia, PA, ¹⁹Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, Ellicott City, MD, ²⁰Duke University, Durham, NC, ²¹Division of Rheumatology, University of Texas McGovern Medical School, Houston, TX, ²²Georgetown University School of Medicine, Washington, DC, ²³University of California Los Angeles, Los Angeles, CA
Background/Purpose: Digital ischemic ulcers (DIU) develop in 36-44% of patients with systemic sclerosis (SSc).1,2Prior international observational studies have evaluated characteristics of digital pitting and ulcers…
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: 0635 • ACR Convergence 2023
Clinical Characteristics and Longitudinal Outcomes of Patients with Childhood-Onset Systemic Sclerosis at a Tertiary Center
Jessica Nguyen¹, Andrea Ramirez², Maria Pereira², Eyal Muscal³, Manuel Silva-Carmona² and Marietta De Guzman², ¹Baylor College of Medicine, Manvel, TX, ²Baylor College of Medicine, Houston, TX, ³Division of Rheumatology, Department of Pediatrics, Baylor College of Medicine, Houston, TX
Background/Purpose: Childhood-onset systemic sclerosis (SSc) is a rare autoimmune multisystemic condition with immune, fibrotic, and vascular manifestations. Although progress has been made in recognizing this…
Abstract Number: 1663 • ACR Convergence 2023
Clinically Severe Systemic Sclerosis Skin Harbors Inflammatory Fibroblasts Associated with Lymphocytes and Plasmacytoid Dendritic Cells
Kimberly Lakin¹, Robert Spiera², Yaxia Zhang¹, David Oliver¹, Aliza Bloostein¹, Hiranmayi Ravichandran³, Niroshana Anandasabapathy³, Franck Barrat¹, Jessica Gordon¹ and Dana Orange⁴, ¹Hospital for Special Surgery, New York, NY, ²Hosptial for Special Surgery, New York, NY, ³Weill Cornell Medical College, New York, NY, ⁴The Rockefeller University, New York, NY
Background/Purpose: We have previously shown that skin fibroblast activation is related to clinical severity and resolves with improvement in diffuse systemic sclerosis (dcSSc). The immune…
Abstract Number: 0638 • ACR Convergence 2023
Application of the 2022 European Society of Cardiology (ESC) Risk Assessment Model in Australian and Singaporean Systemic Sclerosis Patients with Newly Diagnosed Pulmonary Arterial Hypertension (PAH)
Zoe Brown¹, Dylan Hansen², Wendy Stevens³, Nava Ferdowsi⁴, laura ross⁵, Alannah Quinlivan⁶, Joanne Sahhar⁷, Gene-Siew Ngian⁷, Diane Apostolopoulos⁷, Jennifer G Walker⁸, Susanna Proudman⁹, Gim Gee Teng¹⁰, Andrea Hsiu Ling Low¹¹, Kathleen Morrisroe¹² and Mandana Nikpour¹³, ¹The University of Melbourne, Armadale, Australia, ²Department of Rheumatology, St Vincent’s Hospital Melbourne, Fitzroy, Australia, ³Department of Rheumatology, St Vincent's Hospital Melbourne, Fitzroy, Australia, ⁴St Vincents Hospital, Kew, Australia, ⁵St. Vincent's Hospital Melbourne, Fitzroy, Australia, ⁶St Vincent's Hospital Melbourne, Highett, Australia, ⁷Monash Health and Monash University, Clayton, Australia, ⁸Royal Adelaide Hospital, Adelaide, Australia, ⁹Royal Adelaide Hospital and University of Adelaide, Adelaide, Australia, ¹⁰Alexandra Hospital, Department of Medicine, Division of Rheumatology, National University Health System, Singapore, Singapore, ¹¹Department of Rheumatology and Immunology, Singapore General Hospital, Singapore, Singapore, ¹²The University of Melbourne at St Vincent’s Hospital Melbourne, Fitzroy, Australia, ¹³The University of Melbourne at St. Vincent’s Hospital Melbourne, Departments of Medicine and Rheumatology, Melbourne, Australia
Background/Purpose: Pulmonary arterial hypertension (PAH) patients can be stratified as low, intermediate, or high risk of 1-year mortality based on clinical, biochemical and haemodynamic prognostic…
Abstract Number: 1702 • ACR Convergence 2023
Outcomes in Systemic Sclerosis Patients Treated with Rituximab and Mycophenolate Mofetil Combination Therapy Compared to Autologous Hematological Stem Cell Transplantation
shiri keret¹, Lisa Kaly², Aniela Shouval², Tsila Zuckerman³, Israel Henig⁴, Abid Awisat⁵, Itzhak Rosner⁶, Michel Rozenbaum², Nina Boulman², Yair Molad⁷, ariela Dortort Lazar⁸, Gleb Slobodin² and Doron Rimar², ¹Bnai Zion, Atlit, Israel, ²Bnai Zion Medical Center, Haifa, Israel, ³Hematology department, Rambam Medical Center, Haifa, Israel, ⁴Stem cell transplantation unit, Hematology department, Rambam Medical Center, Haifa, Israel, ⁵Bney-Zion Medical Center, Baqa Elgharbiya, Israel, ⁶Bnai Zion Medical Center/Technion, Haifa, Israel, ⁷Rabin Medical Center, Beilinson Hospital, and Tel Aviv University, Petah-Tikva, Israel, ⁸Rabin Medical Center and Tel Aviv university, Tel Aviv, Israel
Background/Purpose: Autologous hematological stem cell transplantation (AHSCT) is a grade A therapy for early diffuse progressive systemic sclerosis (SSc), that has been validated in three…
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: 1703 • ACR Convergence 2023
Increased Mortality and Cardiovascular Events in Male Patients with Systemic Sclerosis: Left Ventricular Global Longitudinal Strain as Possible Screening Tool
Jeska de Vries-Bouwstra¹, Tea Gegenava², Federico Fortuni³, Nina Van Leeuwen⁴, Anders Tennoe⁵, Anna Maria Hoffmann-Vold⁶, Ruxandra Jurcut⁷, Adrian Giuca⁷, Laura Groseanu⁸, Felix Tanner⁹, Oliver Distler¹⁰, Jeroen Bax¹¹ and Nina Ajmone Marsan¹¹, ¹Leiden University Medical Center, Leiden, Netherlands, ²Department of Cardiology, Heart Lung Centre, Leiden University Medical Centre; Department of Internal Medicine,Tbilisi State Medical University, Tbilisi, Georgia, ³Department of Cardiology, San Giovanni Battista Hospital, Foligno, Italy, ⁴Department of Rheumatology, Leiden University Medical Centre, Leiden, Netherlands, ⁵Department of Rheumatology, Oslo University Hospital, Oslo, Norway, ⁶Oslo University Hospital, Department of Rheumatology, Oslo, Norway, ⁷Department of Cardiology, Emergency Institute for Cardiovascular Diseases “Prof .Dr. C. C. Iliescu”, University of Medicine and Pharmacy "Carol Davila", Bucharest, Romania, ⁸University of Medicine and Pharmacy "Carol Davila", Department of Internal Medicine -Rheumatology; Santa Maria Clinical Hospital, Internal Medicine and Rheumatology, Bucharest, Romania, ⁹Department of Cardiology, University Heart Centre, University Hospital Zurich, Zurich, Switzerland, ¹⁰Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ¹¹Department of Cardiology, Heart Lung Centre, Leiden University Medical Centre, Leiden, Netherlands
Background/Purpose: Systemic sclerosis (SSc) is less frequent in males, but the risk of severe outcomes is higher in males than in females(1). Seven to 30%…
Abstract Number: 0646 • ACR Convergence 2023
Racial Variability in Immune Responses Only Partially Explains Differential Systemic Sclerosis Disease Severity
Kamini Kuchinad¹, Ji Soo Kim¹, Adrianne Woods¹, Gwen Leatherman¹, Laura Gutierrez², Maureen Mayes³, Robyn Domsic⁴, Paula Ramos⁵, Richard Silver⁵, John Varga⁶, Lesley Ann Saketkoo⁷, Suzanne Kafaja⁸, Victoria Shanmugam⁹, Jessica Gordon¹⁰, Lorinda Chung¹¹, Elana Bernstein¹², Pravitt Gourh¹³, Francesco Boin¹⁴, Daniel Kastner¹⁵, Scott Zeger¹⁶, Livia Casciola-Rosen¹, Fred Wigley¹ and Ami Shah¹⁷, ¹Department of Rheumatology, Johns Hopkins Medicine, Baltimore, MD, ²Johns Hopkins Medicine, Baltimore, MD, ³Division of Rheumatology, University of Texas McGovern Medical School, Houston, TX, ⁴University of Pittsburgh, Pittsburgh, PA, ⁵Medical University of South Carolina, Charleston, SC, ⁶University of Michigan, Ann Arbor, MI, ⁷University Medical Center - Comprehensive Pulmonary Hypertension Center and ILD Clinic Programs // New Orleans Scleroderma and Sarcoidosis Patient Care & Research Centeris, New Orleans, LA, ⁸UCLA Department of Medicine, Division of Rheumatology, Los Angeles, CA, ⁹Victoria Shanmugam, MD, Great Falls, VA, ¹⁰Hospital for Special Surgery, New York, NY, ¹¹Department of Medicine, Division of Immunology and Rheumatology, Stanford University School of Medicine, Woodside, CA, ¹²Columbia University, New York, NY, ¹³National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ¹⁴Cedars-Sinai Medical Center, Los Angeles, CA, ¹⁵National Human Genome Research Institute, Bethesda, MD, ¹⁶Johns Hopkins University, Baltimore, MD, ¹⁷Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, Ellicott City, MD
Background/Purpose: Individuals with self-identified Black race have a higher incidence of systemic sclerosis (SSc), develop SSc at a younger age, and have a more severe…

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