Abstracts tagged "Scleroderma, Systemic"

Abstract Number: 086 • 2023 Pediatric Rheumatology Symposium
Systemic Sclerosis Overlap Syndrome: A Case Series from a Single Large Pediatric Center
Jessica Nguyen¹, Miriah Gillispie-Taylor¹, Eyal Muscal¹ and Marietta Deguzman², ¹Baylor College of Medicine, Houston, TX, ²Baylor College of Medicine, Texas Children's Hospital, Houston, TX
Background/Purpose: Childhood-onset systemic sclerosis (SSc) is a rare but potentially life-threatening autoimmune condition with features including immune, fibrotic, and vascular manifestations affecting the skin and…
Abstract Number: 106 • 2023 Pediatric Rheumatology Symposium
Caregivers’ Perspectives on Barriers to Care in Juvenile Localized and Systemic Scleroderma
Leigh Stubbs¹, Andrew Ferry², Danielle Guffey¹, Christina Loccke³, Erin Moriarty Wade³, Pamela Pour³, Kaveh Ardalan⁴, Peter Chiraseveenuprapund⁵, Ingrid Ganske⁶, Daniel Glaser⁷, Gloria Higgins⁸, Nadia Luca⁹, Katharine Moore¹⁰, Vidya Sivaraman¹¹, Katie Stewart¹, Natalia Vasquez Canizares¹², Raegan Hunt¹, Renata Maricevich¹, Kathryn Torok¹³ and Suzanne Li¹⁴, ¹Baylor College of Medicine, Houston, TX, ²Oregon Health and Science University, Portland, OR, ³n/a, ⁴Duke University School of Medicine, Durham, NC, ⁵University of California - San Diego, San Diego, CA, ⁶Boston Children’s Hospital and Harvard Medical School, Boston, MA, ⁷Yale University School of Medicine, New Haven, CT, ⁸Nationwide Childrens Hospital/ The Ohio State University, Columbus, OH, ⁹University of Calgary, Calgary, AB, Canada, ¹⁰University of Colorado / Children's Hospital Colorado, Denver, CO, ¹¹Nationwide Children's Hospital/ The Ohio State University, Columbus, OH, ¹²Children's Hospital at Montefiore, New York, NY, ¹³University of Pittsburgh, Pittsburgh, PA, ¹⁴Hackensack Meridian School of Medicine, Joseph M. Sanzari Children's Hospital, Hackensack, NJ
Background/Purpose: Juvenile localized scleroderma (LS) and systemic sclerosis (SSc) are rare rheumatic diseases often associated with severe morbidities. Delays in diagnosis are common, putting children…
Abstract Number: 116 • 2023 Pediatric Rheumatology Symposium
Clinical Significance of Anti-Scl-70 Antibodies in Pediatric Lupus Patients: A Single Center Cohort
Dawn Janysek¹, Yiressy Pina², Danielle Guffey³ and Marietta De Guzman¹, ¹Baylor College of Medicine, Texas Children's Hospital, Houston, TX, ²Baylor College of Medicine, Texas Children's Hospital, Houston, TX, ³Baylor College of Medicine, Houston, TX
Background/Purpose: Production of autoantibodies is a hallmark of SLE, with ANAs as a required diagnostic feature and anti-dsDNA and anti-Smith antibodies being disease-specific. Anti-Scl-70 antibodies…
Abstract Number: 0435 • ACR Convergence 2022
Onset of ANCA-associated Vasculitides in Systemic Sclerosis: Phenotype, Management and Outcomes
deborah Eshagh¹, Thomas Quéméneur², Alexandre Karras³, Viviane Queyrel⁴, Jean Francois Augusto⁵, Christian Agard⁶, Vincent Audard⁷, Marion Couderc⁸, PIerre Duffau⁹, Cecile-Audrey Durel¹⁰, Stan Faguer¹¹, Noemie Jourde Chiche¹², Aurelie Lavergne¹³, Christian Lavigne⁵, Nicolas Limal¹, Amélie Servettaz¹⁴, Perrine Smets¹⁵, Alexis Regent¹⁶, Luc Mouthon¹⁶ and Benjamin Terrier¹⁶, ¹APHP, Paris, France, ²Valenciennes Hospital, Valenciennes, France, ³HEGP, Paris, France, ⁴CHU NIce, Nice, France, ⁵CHU Angers, Angers, France, ⁶Internal medicine, Nantes University Hospital, Nantes, France, ⁷CHU Henri Mondor, Creteil, France, ⁸University Hospital, Clermont-Ferrand, France, ⁹CHU Bordeaux, Bordeaux, France, ¹⁰CHU Lyon, Lyon, France, ¹¹CHU Toulouse, Toulouse, France, ¹²APHM, Marseille, France, ¹³CHU Rennes, Rennes, France, ¹⁴CHU Reims, Reims, France, ¹⁵CHU Clermont Ferrand, Clermont-Ferrand, France, ¹⁶National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France
Background/Purpose: Systemic sclerosis (SSc) is a complex autoimmune disease characterized by autoantibody production, skin and visceral fibrosis, and vasculopathy. The onset of ANCA-associated vasculitides (AAV)…
Abstract Number: 2160 • ACR Convergence 2022
The Role of CCL21 in Serum Samples from Systemic Sclerosis Patients
Henriette Didriksen¹, Øyvind Molberg², Adi Mehta³, Suzana Jordan⁴, Håvard Fretheim³, Einar Gude³, Thor Ueland³, Cathrine Brunborg³, Vyacheslav Palchevskiy⁵, Torhild Garen³, Øyvind Midtvedt³, Arne Andreassen³, Fridtjof Lund-Johansen³, Oliver Distler⁴, John Belperio⁵ and Anna-Maria Hoffmann-Vold³, ¹Oslo University Hospital, Moss, Norway, ²Department of Rheumatology, Oslo University Hospital, Oslo, Norway, Oslo, Norway, ³Oslo University Hospital, Oslo, Norway, ⁴Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ⁵University of California Los Angeles, Los Angeles, CA
Background/Purpose: Chemokines appear to be important for the pathogenesis of autoimmune diseases. Humans may generate antibodies targeting chemokines leading to inhibition of signaling and driving…
Abstract Number: 0518 • ACR Convergence 2022
Changes in Skin Fibroblast Polarization Gene Expression Herald Clinical Improvement in Early, Diffuse Cutaneous Systemic Sclerosis
Kimberly Showalter Lakin¹, Deanna Jannat-Khah, DrPH, MSPH¹, Yajas Shah², Olivier Elemento², Aliza Bloostein¹, Jessica Gordon¹, Michael Whitfield³, Barbara White⁴, Dana Orange⁵ and Robert Spiera¹, ¹Hospital for Special Surgery, New York, NY, ²Weill Cornell Medicine, New York, NY, ³Dartmouth Geisel School of Medicine, Department of Biomedical Data Science, Lebanon, NH, ⁴Corbus Pharmaceuticals, Pleasanton, CA, ⁵The Rockefeller University, New York, NY
Background/Purpose: We previously identified a group of genes associated with high alpha-smooth muscle actin (aSMA) and low CD34 fibroblast staining in lesional systemic sclerosis (SSc)…
Abstract Number: 2162 • ACR Convergence 2022
Systemic Sclerosis in Alaska Native/American Indian People in Alaska
Vivek Mehta and Elizabeth Ferucci, Alaska Native Tribal Health Consortium, Anchorage, AK
Background/Purpose: Systemic sclerosis (SSc) is known to have more severe manifestations and higher mortality in Black populations, while fewer studies have examined disparities in epidemiology…
Abstract Number: 1059 • ACR Convergence 2022
Vision Transformer Assisting Rheumatologists in Screening for Capillaroscopy Changes in Systemic Sclerosis: An Artificial Intelligence Solution
Alexandru Garaiman¹, Farhad Nooralahzadeh², Carina Mihai¹, Nikitas Gkikopoulos¹, Nicolas Perez Gonzalez², Mike Becker¹, Oliver Distler¹, Michael Krauthammer³ and Britta Maurer⁴, ¹Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ²Department of Biomedical Informatics, University of Zurich, Zürich, Switzerland, ³Department of Quantitative Biomedicine, University of Zurich, Zürich, Switzerland, ⁴Department of Rheumatology and Immunology, University Hospital Bern, University of Bern, Bern, Switzerland
Background/Purpose: An accurate assessment of nailfold capillaroscopy (NFC) images has great importance in the diagnosis and prognosis of systemic sclerosis (SSc). To overcome some of…
Abstract Number: 2165 • ACR Convergence 2022
Patients with Systemic Sclerosis Have Impaired Forced Vital Capacity, Even in the Absence of Interstitial Lung Disease
Håvard Fretheim, Imon Barua, Øyvind Midtvedt, Torhild Garen, Phoung Phoung Diep, Michael Durheim, Cathrine Brunborg and Anna-Maria Hoffmann-vold, Oslo University Hospital, Oslo, Norway
Background/Purpose: Spirometry, in particular forced vital capacity (FVC), is a widely implemented tool in the initial diagnostic workup and monitoring of systemic sclerosis-related interstitial lung…
Abstract Number: 1119 • ACR Convergence 2022
Investigating Macrophage Heterogeneity in the Esophagus and Lungs of SSc Patients
Hadijat Makinde¹, Carla Cuda¹, Miranda Gurra¹, Mary Carns², Kathleen Aren², Gaurav Gadhvi¹, Salina Dominguez¹, Jane Dematte³, Darren Brenner⁴, John Pandolfino⁵, G. R. Scott Budinger⁴, Deborah Winter⁶ and Harris Perlman¹, ¹Northwestern University, Chicago, IL, ²Northwestern University Division of Rheumatology, Chicago, IL, ³Northwestern University, Elmhurst, IL, ⁴Northwestern University Feinberg School of Medicine, Chicago, IL, ⁵Northwestern University, Feinberg School of Medicine, Wilmette, IL, ⁶Northwestern University, Skokie, IL
Background/Purpose: Our group has made important contributions to an emerging understanding of monocytes and macrophages as central to SSc pathogenesis. There are numerous studies that…
Abstract Number: 2174 • ACR Convergence 2022
Influence of Immunosuppressive Therapy on Gastrointestinal Symptoms in Patients with Systemic Sclerosis
Lea Stamm¹, Alexandru Garaiman¹, Norina Zampatti¹, Mike Becker¹, Cosimo Bruni¹, Rucsandra Dobrota¹, Muriel Elhai¹, Sherif Ismail², Suzana Jordan¹, Aurora Tatu³, Oliver Distler¹ and Carina Mihai¹, ¹Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ²Department of Internal Medicine, National Research Center, Cairo, Egypt, ³Department of Gastroenterology, University Hospital Zurich, University of Zurich, Zürich, Switzerland
Background/Purpose: The gastrointestinal (GI) tract is frequently affected in systemic sclerosis (SSc), leading to considerable morbidity and even mortality. While important progress has been made…
Abstract Number: 1127 • ACR Convergence 2022
Unsupervised Clustering Analysis of the Serum Proteome Identifies Systemic Sclerosis Patients with Distinctive Clinical Features
Chary Lopez-Pedrera¹, Rafaela Ortega-Castro¹, Tomás Cerdó², Pilar Font¹, María Martinez-Monllor³, Laura Muñoz-Barrera¹, Ismael Sánchez-Pareja², María Carmen Ábalos-Aguilera³, Nuria Barbarroja¹, Eduardo Collantes¹, María Ángeles Aguirre-Zamorano⁴ and Carlos Pérez-Sánchez¹, ¹IMIBIC/University of Cordoba/Reina Sofia Hospital, Cordoba, Spain, ²IMIBIC/University of Cordoba/Reina Sofia Hospital, Córdoba, Spain, ³Imibic/ University of Córdoba/ Reina Sofía Hospital, Córdoba, Spain, ⁴Reina Sofía University Hospital/ Rheumatology Department, Córdoba, Spain
Background/Purpose: Systemic sclerosis (SSc) is an heterogeneous autoimmune disease. There is an unmet need of biomarkers for diagnosis, progression and therapeutic response.This study aimed to…
Abstract Number: 2176 • ACR Convergence 2022
Novel Tissue Turnover Blood Markers Are Associated with Pulmonary Arterial Hypertension and NYHA Stages in Patients with Systemic Sclerosis
Signe Holm Nielsen¹, Sine Søndergaard Korsholm², Axel Diederichsen³, Morten Karsdal¹, John Bonde Knudsen⁴, Anne-Christine Bay-Jensen¹, Louise Diederichsen⁵ and Christoffer Tandrup Nielsen⁶, ¹Nordic Bioscience, Herlev, Denmark, ²Center for Rheumatology and Spine Diseases, Rigshospitalet, Copenhagen, Denmark, Dept of Rheumatology, Odense University Hospital (OUH), Odense, Denmark, ³Dept of Cardiology, OUH, Odense, Denmark, Odense, ⁴Dept of Rheumatology, OUH, Odense, Denmark., Odense, ⁵Copenhagen University Hospital, Rigshospitalet, Dragør, Denmark, ⁶Center for Rheumatology and Spine Diseases, Rigshospitalet, Copenhagen, Denmark
Background/Purpose: Systemic sclerosis (SSc) is characterized by vasculopathy and fibrosis of the skin and internal organs. Cardiac involvement, pulmonary arterial hypertension (PAH), and interstitial lung…
Abstract Number: 1167 • ACR Convergence 2022
Towards an Autologous 3D Skin-like Tissue Harboring Patient-Derived Fibroblasts, Keratinocytes, T-cells and Macrophages
Sasha Shenk¹, Jonathan Garlick¹, Lev Brown², Jamie Riesenberg², Christian Evans², Julia Jaffe Zweifach², Andrew Macklin², Tamar Abel³, Noelle Kosarek⁴, Mengqi Huang⁵, Avi Smith¹, Tammara Wood³, Gretel Torres⁶, Patricia pioli⁶ and Michael Whitfield⁷, ¹Tufts University School of Dental Medicine, Boston, MA, ²Tufts University, Boston, MA, ³Dartmouth College, Hanover, NH, ⁴Dartmouth Geisel School of Medicine, Lebanon, NH, ⁵University of Pittsburgh, Pittsburgh, PN, ⁶Geisel School of Medicine at Dartmouth, Lebanon, NH, ⁷Dartmouth Geisel School of Medicine, Department of Biomedical Data Science, Lebanon, NH
Background/Purpose: Scleroderma (Systemic Sclerosis; SSc) is an autoimmune disease of unknown etiology that is characterized by vascular dysfunction, fibrosis, and inflammation. A lack of human…
Abstract Number: PP23 • ACR Convergence 2022
The Journey to Collaborative Care and Patient-Centric Educational Opportunities for a Scleroderma Patient
Amy Gietzen and Hannah Bowen, The Ann Steffens Scleroderma Foundation, Albany, NY
Background/Purpose: In 2001 I started to experience forearm, elbow, and wrist pain. My fingers were constantly swollen, and sensitive. After 6 months of living in…

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