Abstracts tagged "Systemic sclerosis"

Abstract Number: 1050 • ACR Convergence 2023
Diagnostic Accuracy of Lung Ultrasound for Detecting Interstitial Lung Disease Among Patients with Systemic Sclerosis: A Comparative Study of Two Scanning Protocols
Fabian Carranza Enriquez¹, Carina Soto-Fajardo¹, Sinthia Solorzano Flores¹, Abish Angeles-Acuña¹, Mayra Mejia-Avila², Amyrari Vázquez-Ortega³, Paola Flores-Ordoñez², Karina Arias Callejas², Alejandra Enriquez-Luna⁴, Graciliano Ramon-Diaz⁴, Brenda Bravo-Zarate², Tatiana Sofía Rodríguez-Reyna³ and Carlos Pineda⁴, ¹Instituto Nacional de Rehabilitación "Luis Guillermo Ibarra Ibarra", Mexico City, Mexico, ²Instituto Nacional de Rehabilitación "Luis Guillermo Ibarra Ibarra", Mexico City, Mexico, ³Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, ⁴Instituto Nacional de Rehabilitacion, Mexico City, Mexico
Background/Purpose: Lung ultrasound (LUS) has proven useful to detect interstitial lung disease (ILD) among patients with SSc when compared to high-resolution computerized tomography (HRCT) as…
Abstract Number: 1654 • ACR Convergence 2023
A Peer Health Coached Resilience-Based Energy Management Program Was Effective in Improving Fatigue and Other Outcomes in People with Systemic Sclerosis: Results of a Randomized Controlled Trial
Susan Murphy¹, Yen Chen², Mary Alore², Sheri Hicks², Adam Pape², Afton Hassett², Anna Kratz², Daniel Whibley², Alexandra Harper³, Suiyuan Huang², Gina Jay², Shannen Bolde² and Dinesh Khanna², ¹University of Michigan, Plymouth, MI, ²University of Michigan, Ann Arbor, MI, ³University of Michigan, Ypsilanti Charter Twp, MI
Background/Purpose: Systemic sclerosis (SSc), a rare autoimmune condition, has a high chronic symptom burden that can have dramatic, life-altering effects on function and quality of…
Abstract Number: 2355 • ACR Convergence 2023
Proteome-based Stratification of Therapeutic Target Population for MT-6194 in Systemic Sclerosis with Pulmonary Arterial Hypertension
Yuuichi Ono¹, Akira Mogami¹, Ryuta Saito², Noriyasu Seki¹, Sho Ishigaki³, Hiroshi Takei³, Keiko Yoshimoto³, Kenji Chiba¹, Tsutomu Takeuchi⁴ and Yuko Kaneko⁵, ¹Mitsubishi Tanabe Pharma Corporation, Yokohama, Japan, ²Mitsubishi Tanabe Pharma Corporaion, Yokohama, Japan, ³Keio University School of Medicine, Tokyo, Japan, ⁴Keio University School of Medicine and Saitama Medical University, Tokyo, Japan, ⁵Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan
Background/Purpose: SSc-related pulmonary arterial hypertension (SSc-PAH) is the one of the leading causes of death in SSc. Early diagnosis and effective therapy for SSc-PAH may…
Abstract Number: 2597 • ACR Convergence 2023
Fecal Microbiota Transplantation in Patients with Systemic Sclerosis and Lower Gastrointestinal Tract Symptoms: Data from the ReSScue Phase 2 Randomized Clinical Trial
Anna-Maria Hoffmann-Vold¹, Havard Fretheim¹, Imon Barua¹, Maylen Nordgård Carstens¹, Henriette Didriksen², Vikas Sarna¹, Knut AE Lundin¹, Oliver Distler³, Dinesh Khanna⁴, Elizabeth Volkmann⁵, Oyvind Midtvedt¹, Tore Midtvedt⁶, Alvilde Dhainaut⁷, Anne-Kristine H Halse⁸, Gunnstein Bakland⁹, Inge Olsen¹, Maiju E Pesonen¹ and Oyvind Molberg¹⁰, ¹Oslo University Hospital, Oslo, Norway, ²Oslo University Hospital, Moss, Norway, ³Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁴University of Michigan, Ann Arbor, MI, ⁵University of California Los Angeles, Los Angeles, CA, ⁶Karolinska Institutet, Stockholm, Sweden, ⁷St. Olav, Trondheim, Norway, ⁸University Hospital Bergen, Bergen, Norway, ⁹University Hospital of North Norway, Tromsø, Norway, ¹⁰Dept of Rheumatology, University Hospital Oslo, Oslo, Norway
Background/Purpose: Lower gastrointestinal tract (GIT) complications are common in patients with systemic sclerosis (SSc), associate with a high disease burden, and current treatment alternatives are…
Abstract Number: 026 • 2023 Pediatric Rheumatology Symposium
Performance of the UCLA Scleroderma Clinical Trials Consortium Gastrointestinal Tract 2.0 Instrument in a Juvenile Systemic Sclerosis Cohort
Sophie Stefancic, Amanda Robinson, Haley Havrilla, Samantha Branton, Vibha Sood and Kathryn Torok, University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Gastrointestinal (GI) manifestations in juvenile onset systemic sclerosis (jSSc) reflect adult disease with a range of involvement along the GI tract, including oropharyngeal dysphagia…
Abstract Number: 074 • 2023 Pediatric Rheumatology Symposium
Clinical Characteristics of Juvenile Systemic Sclerosis in Korea: A 30-year Single Center Study
Jieun Jeong¹, Minji Kim¹, Jiwon Jung², Seon Hee Lim³ and Seong Heon Kim¹, ¹Department of Pediatrics, Seoul National University Children’s Hospital, Seoul, Republic of Korea, ²Department of Pediatrics, Asan Medical Center Children’s Hospital, University of Ulsan College of Medicine, Seoul, Republic of Korea, ³Department of Pediatrics, Pusan National University Children’s Hospital, Yangsan, Republic of Korea
Background/Purpose: Juvenile systemic sclerosis (jSSc) is a rare autoimmune, chronic, multisystem, connective tissue disease characterized by progressive tissue fibrosis of the skin and internal organs.…
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: 0520 • ACR Convergence 2022
Combination Therapy of Mycophenolate Mofetil and Pirfenidone vs. Mycophenolate Alone: Results from the Scleroderma Lung Study III
Dinesh Khanna¹, Cathie Spino², Elana Bernstein³, Jonathan Goldin⁴, Donald Tashkin⁴, Michael roth⁴ and On Behalf of SLS III Investigators², ¹Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI, ²University of Michigan, Ann Arbor, MI, ³Columbia University, New York, NY, ⁴University of California Los Angeles, Los Angeles, CA
Background/Purpose: Scleroderma Lung Study (SLS) II established mycophenolate mofetil (MMF) as an active therapy for scleroderma-related interstitial lung disease (SSc-ILD) and the need to consider…
Abstract Number: 1126 • ACR Convergence 2022
Functional NOTCH4 Variants Increase Notch Signaling and Susceptibility for Systemic Sclerosis
Urvashi Kaundal¹, Emilee Stenson¹, Mousumi Sahu¹, Krishan Kumar Thakur¹, Janet Wang¹, Ami Shah², Maureen Mayes³, Ayo Doumatey⁴, Amy Bentley⁴, Daniel Shriner⁴, Robyn Domsic⁵, Thomas Medsger⁶, Paula Ramos⁷, Richard Silver⁷, Virginia Steen⁸, John Varga⁹, Vivien Hsu¹⁰, Lesley Ann Saketkoo¹¹, Elena Schiopu¹², Dinesh Khanna¹³, Jessica Gordon¹⁴, Lindsey Criswell¹⁵, Heather Gladue¹⁶, Chris Derk¹⁷, Elana Bernstein¹⁸, S. Louis Bridges, Jr.¹⁴, Victoria Shanmugam¹⁹, Lorinda Chung²⁰, Suzanne Kafaja²¹, Reem Jan²², Marcin Trojanowski²³, Avram Goldberg²⁴, Benjamin Korman²⁵, Jim Mullikin⁴, Stefania Dell'Orso¹, Adebowale Adeyemo⁴, Charles Rotimi⁴, Elaine Remmers⁴, Daniel Kastner⁴, Fredrick Wigley²⁶, Francesco Boin²⁷ and Pravitt Gourh²⁸, ¹National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ²Johns Hopkins Rheumatology, Baltimore, MD, ³Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, TX, ⁴National Human Genome Research Institute, Bethesda, MD, ⁵University of Pittsburgh, Pittsburgh, PA, ⁶University of Pittsburgh School of Medicine, Pittsburgh, PA, ⁷Medical University of South Carolina, Charleston, SC, ⁸Georgetown University School of Medicine, Washington, DC, ⁹University of Michigan, Ann Arbor, MI, ¹⁰Rutgers-RWJ Medical School, South Plainfield, NJ, ¹¹University Medical Center - Comprehensive Pulmonary Hypertension Center and ILD Clinic Programs // New Orleans Scleroderma and Sarcoidosis Patient Care & Research Centeris, New Orleans, LA, ¹²Michigan Medicine, Ann Arbor, MI, ¹³Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI, ¹⁴Hospital for Special Surgery, New York, NY, ¹⁵National Human Genome Research Institute, NIH, Bethesda, MD, ¹⁶Arthritis & Osteoporosis Consultants of the Carolinas, Charlotte, NC, ¹⁷University of Pennsylvania, Philadelphia, PA, ¹⁸Columbia University, New York, NY, ¹⁹George Washington University, Great Falls, VA, ²⁰Stanford University, Stanford, CA, ²¹UCLA Department of Medicine, Division of Rheumatology, Los Angeles, CA, ²²University of Chicago, Chicago, IL, ²³Boston University School of Medicine, Boston, MA, ²⁴NYU Langone Medical Center - NYU Hospital for Joint Diseases, Lake Success, NY, ²⁵University of Rochester, Rochester, NY, ²⁶Johns Hopkins University, Baltimore, MD, ²⁷Cedars-Sinai Medical Center, Los Angeles, CA, ²⁸National Institutes of Health, Bethesda, MD
Background/Purpose: Genome wide association studies (GWAS) in systemic sclerosis (SSc) have identified several genetic loci, but the search for the causal variant and gene continues.…
Abstract Number: 1364 • ACR Convergence 2022
Diffuse Juvenile Systemic Sclerosis Patients Show Distinct Organ Involvement and Have More Severe Disease in the Largest jSSc Cohort of the World. Results from the the Juvenile Scleroderma Inception Cohort
Ivan Foeldvari¹, Jens Klotsche², Ozgur Kasapcopur³, Amra Adrovic⁴, Kathryn Torok⁵, Maria Teresa Terreri⁶, Ana Paula Sakamoto⁷, Brian Feldman⁸, FLAVIO SZTAJNBOK⁹, Valda Stanevica¹⁰, Jordi Anton¹¹, Sindu Johnson¹², Raju Khubchandani¹³, Ekaterina Alexeeva¹⁴, Maria Katsicas¹⁵, Sujata Sawhney¹⁶, Vanessa Smith¹⁷, Simone Appenzeller¹⁸, Tadey Avcin¹⁹, Mikhail Kostik²⁰, Thomas Lehman²¹, Hana Malcova²², Edoardo Marrani²³, Clare Pain²⁴, Dieneke Schonenberg-Meinema²⁵, Walter Alberto Sifuentes-Giraldo²⁶, Natalia Vasquez-Canizares²⁷, Patricia Costa Reis²⁸, Mahesh Janarthanan²⁹, Dana Nemcova³⁰, Anjali Patwardhan³¹, Maria José Santos³², Sima Abu Alsaoud³³, Cristina Battagliotti³⁴, Lillemor Berntson³⁵, Blanca Bica³⁶, Jürgen Brunner³⁶, Rolando Cimaz³⁷, Despina Eleftheriou³⁸, Liora Harel³⁹, Gerd Horneff⁴⁰, Daniela Kaiser⁴¹, Tilmann Kallinich⁴², Dragana Lazarevic⁴³, Farzana Nuruzzaman⁴⁴ and Nicola Helmus⁴⁵, ¹Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ²German Rheumatism Research Center, Berlin, Germany, ³Istanbul University-Cerrahpaşa, Cerrahpaşa Medical School, Istanbul, Turkey, ⁴Cerrahpaşa Medical School, Istanbul University, Istanbul, Turkey, ⁵Pediatric Rheumatology, Children's Hospital of UPMC, Pittsburgh, PA, ⁶Universidad Federal São Paulo, São Paulo, Brazil, ⁷Federal University of Sao Paulo (UNIFESP), São Paulo, Brazil, ⁸Division of Rheumatology, The Hospital for Sick Children; Child Health Evaluative Services, SickKids Research Institute; Department of Paediatrics, University of Toronto, Toronto, ON, Canada, ⁹UFRJ/UERJ, São Paulo, Brazil, ¹⁰Children's Clinical University Hospital, Zemgales priekšpilseta, Riga, Latvia, ¹¹Pediatric Rheumatology, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain, ¹²University of Toronto, Toronto, ON, Canada, ¹³SRCC Children's Hospital, Mumbai, India, ¹⁴National Medical Research Center of Children's Health, Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation,, Moscow, Russia, ¹⁵Hospital Garrahan, Buenos Aires, Argentina, ¹⁶Sir Ganga Ram Hospital, Sector 37 noida, Uttar Pradesh, India, ¹⁷Department of Rheumatology, Ghent University Hospital – Department of Internal Medicine, Ghent University, Belgium – Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Gent, Belgium, ¹⁸Unicamp, Campinas, São Paulo, Brazil, ¹⁹University Children's Hospital University Medical Center Ljubljana, Ljubljana, Slovenia, ²⁰Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ²¹Hospital for Special Surgery, New York, NY, ²²Motol University Hospital, Prague, Czech Republic, ²³University of Florence, Firenze, Italy, ²⁴Alder Hey Children’s NHS Foundation Trust, Liverpool, United Kingdom, ²⁵Emma Children’s Hospital, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, Netherlands, ²⁶Hospital Universitario Ramon y Cajal, Madrid, Spain, ²⁷Children’s Hospital at Montefiore, Bronx, NY, ²⁸Hospital de Santa Maria, Lisbon, Portugal, ²⁹SRI RAMACHANDRA INSTITUTE OF HIGHER EDUCATION AND RESEARCH, Chennai, India, ³⁰Charles University, Prague, Czech Republic, ³¹University of Missouri, Columbia, MO, ³²Hospital Garcia de Orta, Almada, Charneca da Caparica, Portugal, ³³Makassed, Jerusalem, Israel, ³⁴Hospital de Niños Dr Orlando Alassia, Santa Fe, Argentina, ³⁵Dept. of Women’s and Children’s Health, Uppsala University, Uppsala, Sweden, ³⁶UNIVERSIDADE FEDERAL DO RIO DE JANEIRO, Rio de Janeiro, Brazil, ³⁷University of Milano, Milano, Italy, ³⁸Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, ³⁹Schneider Children's Medical center, Tel Aviv University, Nettnja, Israel, ⁴⁰Pediatrics, Asklepios Klinik Sankt Augustin GmbH, Sankt Augustin, Germany, ⁴¹Children's Hospital Lucerne, Lucerne, Switzerland, ⁴²Charité - Universitätsmedizin Berlin, Nuremberg, Germany, ⁴³Faculty of Medicine, University of Niš, Nis, Serbia, ⁴⁴Stony Brook Children's Hospital, East Setauket, NY, ⁴⁵Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany
Background/Purpose: Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. In adult patients there are significant…
Abstract Number: 1621 • ACR Convergence 2022
Genome-wide Association Study in African American Systemic Sclerosis Patients Identifies a Novel Target – Transforming Growth Factor-β3 (TGFβ3)
Urvashi Kaundal¹, Julia Hartman², Emilee Stenson¹, Sarah Safran¹, Chloe Borden¹, Mousumi Sahu¹, Janet Wang¹, Andrea Conte¹, Ami Shah³, Maureen Mayes⁴, Ayo Doumatey⁵, Amy Bentley⁵, Daniel Shriner⁵, Robyn Domsic⁶, Thomas Medsger⁷, Paula Ramos⁸, Richard Silver⁸, Virginia Steen⁹, John Varga¹⁰, Vivien Hsu¹¹, Lesley Ann Saketkoo¹², Elena Schiopu¹³, Dinesh Khanna¹⁴, Jessica Gordon¹⁵, Lindsey Criswell¹⁶, Heather Gladue¹⁷, Chris Derk¹⁸, Elana Bernstein¹⁹, S. Louis Bridges, Jr.¹⁵, Victoria Shanmugam²⁰, Lorinda Chung²¹, Suzanne Kafaja²², Reem Jan²³, Marcin Trojanowski²⁴, Avram Goldberg²⁵, Benjamin Korman²⁶, Settara Chandrasekharappa⁵, Yongbing Zhao²⁷, Stephen Brooks²⁷, Stefania Dell'Orso¹, Adebowale Adeyemo⁵, Charles Rotimi⁵, Elaine Remmers⁵, Daniel Kastner⁵, Francesco Boin²⁸, Rafael Casellas²⁷, Fredrick Wigley²⁹ and Pravitt Gourh³⁰, ¹National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ²National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Washington, DC, ³Johns Hopkins Rheumatology, Baltimore, MD, ⁴Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, TX, ⁵National Human Genome Research Institute, Bethesda, MD, ⁶University of Pittsburgh, Pittsburgh, PA, ⁷University of Pittsburgh School of Medicine, Pittsburgh, PA, ⁸Medical University of South Carolina, Charleston, SC, ⁹Georgetown University School of Medicine, Washington, DC, ¹⁰University of Michigan, Ann Arbor, MI, ¹¹Rutgers-RWJ Medical School, South Plainfield, NJ, ¹²University Medical Center - Comprehensive Pulmonary Hypertension Center and ILD Clinic Programs // New Orleans Scleroderma and Sarcoidosis Patient Care & Research Centeris, New Orleans, LA, ¹³Michigan Medicine, Ann Arbor, MI, ¹⁴Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI, ¹⁵Hospital for Special Surgery, New York, NY, ¹⁶National Human Genome Research Institute, NIH, Bethesda, MD, ¹⁷Arthritis & Osteoporosis Consultants of the Carolinas, Charlotte, NC, ¹⁸University of Pennsylvania, Philadelphia, PA, ¹⁹Columbia University, New York, NY, ²⁰George Washington University, Great Falls, VA, ²¹Stanford University, Stanford, CA, ²²UCLA Department of Medicine, Division of Rheumatology, Los Angeles, CA, ²³University of Chicago, Chicago, IL, ²⁴Boston University School of Medicine, Boston, MA, ²⁵NYU Langone Medical Center - NYU Hospital for Joint Diseases, Lake Success, NY, ²⁶University of Rochester, Rochester, NY, ²⁷National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ²⁸Cedars-Sinai Medical Center, Los Angeles, CA, ²⁹Johns Hopkins University, Baltimore, MD, ³⁰National Institutes of Health, Bethesda, MD
Background/Purpose: Transforming Growth Factor-β (TGFβ) isoforms play an important role in extracellular matrix biology. Increased TGFβ-regulated gene signature has been observed in lesional skin and…
Abstract Number: 2172 • ACR Convergence 2022
Pulmonary Function in Patients Diagnosed of Early Systemic Sclerosis: 10 Years-Experience
Samuel Leal Rodriguez¹, Francisco Miguel Ortiz Sanjuan², José Ivorra Cortés³, Laura Mas Sanchez³, Pablo Muñoz Martinez⁴, Carmen Riesco Bárcena⁵, Anderson Huaylla Quispe⁶, Cristobal Pavez Perales³, Inés Cánovas Olmos³, Luis Gonzalez Puig⁷, Elena Grau García³, Isabel Martínez-Cordellat³, Carmen Nájera Herranz³, Rosa Negueroles Albuixech³, JOSE ELOY OLLER RODRIGUEZ⁸, Elvira Vicens Bernabeu², Alba Torrat Novés⁵, Daniel Ramos Castro³ and José andrés Román ivorra¹, ¹Hospital Universitari i Politécnic La Fe, València, Spain, ²Hospital Universitario de La Fe, València, ³Rheumatology Department. Hospital Universitario y Politécnico La Fe, València, Spain, ⁴Rheumatology Department. Hospital Universitario y Politécnico La Fe, Sagunto, Spain, ⁵Rheumatology Department. Hospital Universitario y Politécnico La Fe, València, ⁶Medicina, València, Spain, ⁷Hospital La Fe, Torrente, Valencia, Spain, ⁸Hospital Universitario de La Fe, València, Spain
Background/Purpose: Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc), often progressive and has a poor prognosis. A restrictive ventilatory defect could…
Abstract Number: 0522 • ACR Convergence 2022
Cohort Enrichment Strategies for Progressive Interstitial Lung Disease in Systemic Sclerosis from EUSTAR
Anna-Maria Hoffmann-Vold¹, Cathrine Brunborg¹, Paolo Airò², Lidia P. Ananyeva³, László Czirják⁴, Serena Guiducci⁵, Eric Hachulla⁶, MENGTAO LI⁷, Carina Mihai⁸, Gabriela Riemekasten⁹, Petros P. Sfikakis¹⁰, Gabriele Valentini¹¹, Otylia Kowal-Bielecka¹², Yannick Allanore¹³ and Oliver Distler⁸, ¹Oslo University Hospital, Oslo, Norway, ²Rheumatology and Clinical Immunology Unit, ASST Spedali Civili, Brescia, Italy, ³V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia, ⁴Medical school of Pécs, Pecs, Hungary, ⁵University of Firenze, Firenze, Italy, ⁶University of Lille, Lille, France, ⁷Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science & Technology, Beijing, China, ⁸Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ⁹University Clinic Schleswit-Holstein (UKSH), Luebeck, Germany, ¹⁰Joint Academic Rheumatology Program, National and Kapodistrian University of Athens, School of Medicine, Athens, Greece, ¹¹Università della Campania “Luigi Vanvitelli”, Napoli, Italy, ¹²Medical University of Bialystok, Bialystok, Poland, ¹³Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France
Background/Purpose: Enrichment strategies from clinical trials for progressive systemic sclerosis-associated interstitial lung disease (SSc-ILD) have been partly successful but have not been tested in a…
Abstract Number: 1168 • ACR Convergence 2022
Dermal Fibroblast-derived Exosomes Drive Profibrotic Macrophage Activation in Systemic Sclerosis
Heetaek Yang¹, Rajan Bhandari¹, Noelle Kosarek², Jonathan Garlick³, Michael Whitfield⁴ and Patricia Pioli¹, ¹Geisel School of Medicine at Dartmouth, Lebanon, NH, ²Dartmouth Geisel School of Medicine, Lebanon, NH, ³Tufts University School of Dental Medicine, Boston, MA, ⁴Geisel School of Medicine, Lebanon, NH
Background/Purpose: Macrophage (MØ) activation derives from coordination of signals received in local tissue microenvironments. In prior studies, we demonstrated that cocultured MØs and fibroblasts from…
Abstract Number: 1367 • ACR Convergence 2022
Patient and Physician Reported Outcomes of Juvenile Systemic Sclerosis Patients Significantly Improve over 12 Months Observation Period in the Juvenile Systemic Scleroderma Inception Cohort
Ivan Foeldvari¹, Jens Klotsche², Ozgur Kasapcopur³, Amra Adrovic⁴, Kathryn Torok⁵, Maria Teresa Terreri⁶, Brian Feldman⁷, Jordi Anton⁸, Maria Katsicas⁹, Valda Stanevica¹⁰, FLAVIO SZTAJNBOK¹¹, Simone Appenzeller¹², Tadey Avcin¹³, Mikhail Kostik¹⁴, Edoardo Marrani¹⁵, Walter Alberto Sifuentes-Giraldo¹⁶, Sindu Johnson¹⁷, Raju Khubchandani¹⁸, Dana Nemcova¹⁹, Maria José Santos²⁰, Cristina Battagliotti²¹, Lillemor Berntson²², Blanca Bica²³, Jürgen Brunner²³, Rolando Cimaz²⁴, Despina Eleftheriou²⁵, Liora Harel²⁶, Gerd Horneff²⁷, Mahesh Janarthanan²⁸, Tilmann Kallinich²⁹, Kirsten Minden³⁰, Anjali Patwardhan³¹, Dieneke Schonenberg-Meinema³², Vanessa Smith³³ and Nicola Helmus³⁴, ¹Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ²German Rheumatism Research Center, Berlin, Germany, ³Istanbul University-Cerrahpaşa, Cerrahpaşa Medical School, Istanbul, Turkey, ⁴Cerrahpaşa Medical School, Istanbul University, Istanbul, Turkey, ⁵Pediatric Rheumatology, Children's Hospital of UPMC, Pittsburgh, PA, ⁶Universidad Federal São Paulo, São Paulo, Brazil, ⁷Division of Rheumatology, The Hospital for Sick Children; Child Health Evaluative Services, SickKids Research Institute; Department of Paediatrics, University of Toronto, Toronto, ON, Canada, ⁸Pediatric Rheumatology, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain, ⁹Hospital Garrahan, Buenos Aires, Argentina, ¹⁰Children's Clinical University Hospital, Zemgales priekšpilseta, Riga, Latvia, ¹¹UFRJ/UERJ, São Paulo, Brazil, ¹²Unicamp, Campinas, São Paulo, Brazil, ¹³University Children's Hospital University Medical Center Ljubljana, Ljubljana, Slovenia, ¹⁴Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ¹⁵University of Florence, Firenze, Italy, ¹⁶Hospital Universitario Ramon y Cajal, Madrid, Spain, ¹⁷University of Toronto, Toronto, ON, Canada, ¹⁸SRCC Children's Hospital, Mumbai, India, ¹⁹Charles University, Prague, Czech Republic, ²⁰Hospital Garcia de Orta, Almada, Charneca da Caparica, Portugal, ²¹Hospital de Niños Dr Orlando Alassia, Santa Fe, Argentina, ²²Dept. of Women’s and Children’s Health, Uppsala University, Uppsala, Sweden, ²³UNIVERSIDADE FEDERAL DO RIO DE JANEIRO, Rio de Janeiro, Brazil, ²⁴University of Milano, Milano, Italy, ²⁵Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, ²⁶Schneider Children's Medical center, Tel Aviv University, Nettnja, Israel, ²⁷Pediatrics, Asklepios Klinik Sankt Augustin GmbH, Sankt Augustin, Germany, ²⁸SRI RAMACHANDRA INSTITUTE OF HIGHER EDUCATION AND RESEARCH, Chennai, India, ²⁹Charité - Universitätsmedizin Berlin, Nuremberg, Germany, ³⁰Charité Universitätsmedizin Berlin, Berlin, Germany, ³¹University of Missouri, Columbia, MO, ³²Emma Children’s Hospital, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, Netherlands, ³³Department of Rheumatology, Ghent University Hospital – Department of Internal Medicine, Ghent University, Belgium – Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Gent, Belgium, ³⁴Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany
Background/Purpose: Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. The Juvenile Systemic Scleroderma Inception cohort…

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