Abstracts tagged "Scleroderma, Systemic"

Abstract Number: 1572 • ACR Convergence 2025
Compromised intracellular oxygen availability and severe mitochondrial dysfunction in the skeletal muscle of patients with systemic sclerosis
Gwenael Layec¹, Tracy Frech² and Anthony Donato³, ¹University of Nebraska, Omaha, NE, ²Vanderbilt University Medical Center, Nashville, TN, ³Salt Lake Veterans Affairs Medical Center, Salt Lake City, UT
Background/Purpose: Muscle weakness and fatigue are frequent manifestations associated with systemic sclerosis (SSc). However, significant gaps remain in our understanding of the effects of SSc…
Abstract Number: 0698 • ACR Convergence 2025
A Retrospective Comparison of Transplant Outcomes with Different Induction Methods and Maintenance Prednisone in Systemic Sclerosis Patients
Lilit Grigoryan, Sumbal Wajid, Giovanni Faddoul and Swati Mehta, Albany medical center, Albany, NY
Background/Purpose: Systemic sclerosis (SSc) or scleroderma is a rare systemic autoimmune disease with multiple organ involvement. Renal involvement in SSc remains a significant concern, with…
Abstract Number: 2487 • ACR Convergence 2025
Cardiovascular and venous thromboembolic events in systemic sclerosis: Epidemiological analysis of the Clinical Practice Research Datalink
John Pauling¹, Rachel Charlton², Laura Ross³, Neil McHugh⁴ and Anita McGrogan², ¹North Bristol NHS Trust, Bristol, United Kingdom, ²University of Bath, Bath, ³St. Vincent's Hospital Melbourne, Fitzroy, Victoria, Australia, ⁴University of Bath, Bath, United Kingdom
Background/Purpose: Cardiovascular disease is a leading cause of mortality in systemic sclerosis (SSc). We investigated the association between SSc and the occurrence of both cardiovascular…
Abstract Number: 1570 • ACR Convergence 2025
Hydroxychloroquine and Disease Progression in Systemic Sclerosis: Insights from Antibody-Stratified Survival Analyses
Francesco Bonomi¹, Ilaria Bisconti², Ilenia Mallia³, Greta Pellegrino⁴, Gabriele Ciuti⁵, Gloria Muolo², Martina Salerno², Simona Truglia², Cristiano Barbetta³, Silvia Peretti⁵, Serena Guiducci⁶, Valeria Riccieri⁷ and Silvia Bellando Randone³, ¹University Hospital Careggi, Florence, Florence, Toscana, Italy, ²Sapienza University of Rome, Rome, Rome, Italy, ³University of Florence, Florence, Italy, ⁴Rheumatology Unit, IRCCS Ospedale Galeazzi Sant'Ambrogio, Milano, Italy; Department of Biomedical and Clinical Sciences, University of Milan, Milan, Italy, ⁵University Hospital Careggi, Florence, Italy, ⁶Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, Florence, Italy, ⁷Sapienza University of Rome, Rheumatology Clinic, Rome, Italy, Roma, Italy
Background/Purpose: Hydroxychloroquine (HCQ) is an antimalarial drug that interferes with dendritic cells and monocytes, acidifies endosomes, and modulates toll-like receptors, reducing autoimmune responses and cell…
Abstract Number: 0690 • ACR Convergence 2025
Quantitative 18F-FDG PET-CT of the lungs detects treatment induced changes in patients with early severe diffuse cutaneous systemic sclerosis and interstitial lung disease
Bo Broens¹, esther Nossent¹, Lilian Meijboom¹, Gerben Zwezerijnen¹, julia Spierings², Jeska de Vries-Bouwstra³, Jacob M. van Laar⁴, Conny van der Laken¹ and Alexandre Voskuijl⁵, ¹Amsterdam UMC, Amsterdam, Netherlands, ²Department of Rheumatology & Clinical Immunology, University Medical Center, Ultrecht, The Netherlands, Utrecht, Netherlands, ³Leiden University Medical Center, Leiden, Netherlands, ⁴UMC Utrecht, Utrecht, Netherlands, ⁵Amsterdam University Medical Center, Amsterdam, Netherlands
Background/Purpose: Patients with early severe diffuse cutaneous systemic sclerosis (dcSSc) with interstitial lung disease (ILD) have poor prognosis. New tools are needed to improve treatment…
Abstract Number: L01 • ACR Convergence 2024
Targeted Exosite Inhibition of STING Activation of TBK1 Selectively Blocks Type I Interferon and NFκB Responses for Treatment of Autoimmune Diseases
Matthew Martin¹, Erik Wilker¹, Diana Gikunju¹, Usha Narayanan¹, Unnati Pandya¹, Vijetha Prakash¹, Ashley Edwards¹, Sameer Kawatkar¹, Tenghui Chen¹, Ragunath Chandran¹, Sai Sunder¹, Sumathi Biradar¹, Joerg Distler², Alexandra Joseph¹, Stephanos Ioannidis¹ and Bhavatarini Vangamudi¹, ¹Exo Therapeutics, Cambridge, MA, ²Exo Therapeutics, Düsseldorf, Germany
Background/Purpose: The cGAS-TBK1-STING pathway senses nucleic acids for innate immunity. Aberrant activation of the pathway is linked to autoimmune diseases including Systemic and Cutaneous Lupus…
Abstract Number: 0684 • ACR Convergence 2024
The Diagnostic Accuracy of Ultrasound for Low Muscle Mass in Women with Systemic Sclerosis
Lucas Dória¹, Vanessa Hax², André Mallmann², Rafaela Santo³, Leonardo Santos², Stephanie Pilotti², Daniel Moraes², Isabella Boening², Pedro Glanze², ODIRLEI MONTICIELO¹, Ricardo Xavier⁴ and Rafael Chakr⁵, ¹Hospital de Clinicas de Porto Alegre, Porto Alegre, Rio Grande do Sul, Brazil, ²Hospital de Clínicas de Porto Alegre, Porto Alegre, Rio Grande do Sul, Brazil, ³Health Research and Innovation Science Centre, Klaipeda University, Klaipeda, Lithuania, Porto Alegre, Rio Grande do Sul, Brazil, ⁴Universidade Federal do Rio Grande do Sul, Porto Alegre/RS, Rio Grande do Sul, Brazil, ⁵Universidade Federal do Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil
Background/Purpose: Patients with systemic sclerosis (SSc) often experience body composition changes, including low muscle mass (myopenia), associated with physical disability, hospitalization and death. Our purpose…
Abstract Number: 1585 • ACR Convergence 2024
Characterization of the Fecal Metabolome in Early Systemic Sclerosis
Arissa Young¹, Kristofer Andreasson², Jen Labus³, Nedas Matulianos⁴, Blake Wilde¹, Jonathan Jacobs³, Heather Christofk⁴ and Elizabeth Volkmann⁵, ¹UCLA, Los Angeles, CA, ²Lund University, Lund, Sweden, ³UCLA, Los Angeles, ⁴University of California Los Angeles, Los Angeles, CA, ⁵University of California, Department of Medicine, Los Angeles, CA, USA, Los Angeles
Background/Purpose: Previous studies have shown that alterations in gut microbiota are present early in systemic sclerosis (SSc) (Andréasson et al. 2022) and are associated with…
Abstract Number: 2473 • ACR Convergence 2024
Criterion Validity of Modified Rodnan Skin Score: Does It Capture Skin Thickness or Hardening? Results of a Large Skin Histology Study
Shervin Assassi¹, Ruhani Desai², Jeffrey Browning³, Samuel Theodore¹, Meng Zhang¹, Brian Skaug¹, Jerry Alonso¹, Zsuzsanna McMahan¹, Maureen Mayes¹ and Minghua Wu¹, ¹UTHealth Houston Division of Rheumatology, Houston, TX, ²UTHealth Houston Division of Rheumatology, DeLand, FL, ³Boston University, Cambridge, MA
Background/Purpose: Modified Rodnan Skin Score is a widely used outcome measure for skin involvement in systemic sclerosis (SSc). Its criterion validity (comparison to the gold…
Abstract Number: 0702 • ACR Convergence 2024
AISA 021, a Novel Calcium Channel Antagonist in Development for Raynaud’s & Systemic Sclerosis, Has Antagonistic Activity at Sodium Channel Targets for Pain Relief and Treats Scleroderma Pain Better Than Current Calcium Channel Blockers in a Phase 2A Study
Andrew Sternlicht¹ and Meredith Todd², ¹Tufts University Medical Center and Aisa Pharma, Inc., Boston, MA, ²Aisa Pharma Pty Ltd., East Lindfield, New South Wales, Australia
Background/Purpose: Improved treatments for Raynaud's phenomenon in Systemic Sclerosis (SSc) are needed. A novel oral calcium channel blocker (cilnidipine), with N-type channel antagonism is being…
Abstract Number: 1591 • ACR Convergence 2024
Domains and Outcome Measures for the Assessment of Digital Vasculopathy and Raynaud Phenomenon in Juvenile Systemic Sclerosis: A Systematic Review
Valerio maniscalco¹, Simone Appenzeller², Jennifer lemon³, Lucy stead⁴, Natalia Vasquez Canizares⁵, Gabriele Simonini⁶, Suzanne Li⁷ and Clare Pain⁸, and IJOG study, ¹Santo Stefano Hospital; Meyer Children Hospital IRCCS, Firenze, Toscana, Italy, ²Unicamp, Campinas, SP, Brazil, ³Alder Hey Children's NHS Foundation Trust, Liverpool, ⁴Liverpool University, Liverpool, ⁵Children's Hospital at Montefiore; Albert Einstein College of Medicine;, New York, NY, ⁶Meyer Children Hospital IRCCS; NEUROFARBA Department, University of Florence, Florence, Italy, ⁷Hackensack University Medical Center, Hackensack, ⁸Alderhey Children's NHS Foundation Trust, Liverpool, United Kingdom
Background/Purpose: Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease that primary affect the vascular system determining multiple organ damage and reducing quality of life…
Abstract Number: 2477 • ACR Convergence 2024
Quantifying Tissue Microstructural Changes in Scleroderma Skin Using a Novel, Point-of-care, High-frequency-ultrasound Instrument
Kimberly Lakin¹, Cameron Hoerig², Jessica Gordon³, Elmira Ghahramani², Mia Diaz¹, Robert Spiera⁴, dana Orange⁵ and Jonathan Mamou², ¹Hospital for Special Surgery, New York, NY, ²Weill Cornell Medical College, New York, NY, ³Division of Rheumatology, Weill Cornell Medical College, New York, NY, ⁴Scleroderma, Vasculitis, and Myositis Center, Hospital for Special Surgery, Weill Cornell Medical College, New York, NY, ⁵Rockefeller University, New York, NY
Background/Purpose: A highly reproducible, quantitative method of measuring skin severity in systemic sclerosis (SSc) trials and patient care is needed. While conventional skin US, operating…
Abstract Number: 0706 • ACR Convergence 2024
Single Center Prospective Cohort of Systemic Sclerosis Patients Who Are At-Risk for Pulmonary Hypertension
Carleigh Zahn¹, Scott Visovatti², Rosemary Gedert¹, Suiyuan Huang¹, Victor Moles¹, Amber Young¹, Vallerie McLaughlin¹ and Dinesh Khanna¹, ¹University of Michigan, Ann Arbor, MI, ²The Ohio State University, Columbus
Background/Purpose: Pulmonary hypertension (PH) is a leading cause of mortality in systemic sclerosis (SSc). We launched an IRB approved prospective study (NOVEL) in 2013 at…
Abstract Number: 1691 • ACR Convergence 2024
Treatment Response Biomarkers for Systemic Sclerosis-Associated Interstitial Lung Disease
Elizabeth Volkmann¹, Holly Wilhalme², Donald Tashkin², Grace Kim², Jonathan Goldin², Alana Haussmann², Masataka Kuwana³, Michael Roth² and Shervin Assassi⁴, ¹University of California, Department of Medicine, Los Angeles, CA, USA, Los Angeles, ²UCLA, Los Angeles, ³Department of Allergy and Rheumatology, Nippon Medical School, Tokyo, Japan, Tokyo, Japan, ⁴UTHealth Houston Division of Rheumatology, Houston, TX
Background/Purpose: Despite the expanding treatment landscape for systemic sclerosis-associated interstitial lung disease (SSc-ILD), the lack of early and valid treatment response biomarkers (measurable in the…
Abstract Number: 2641 • ACR Convergence 2024
Identification of Systemic Sclerosis Intrinsic Subtypes in the ASSET Clinical Trial Using PBC Gene Expression
Zhiyun Gong¹, Timothy Sullivan², Tammara Wood³, David Fox⁴, Dinesh Khanna⁵ and Michael Whitfield⁶, ¹Dartmouth College, Lebanon, NH, ²Geisel School of Medicine at Dartmouth, Lebanon, NH, ³Dartmouth, Hanover, NH, ⁴University of Michigan, Dexter, MI, ⁵University of Michigan, Ann Arbor, MI, ⁶Geisel School of Medicine at Dartmouth, Hanover, NH
Background/Purpose: Systemic sclerosis (SSc) is molecularly heterogeneous and distinct subtypes of patients have been identified based on gene expression in skin. Although treatments have improved,…

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