Abstracts tagged "Systemic sclerosis"

Abstract Number: 1070 • ACR Convergence 2022
Discordance of Patient, Physician and Nurse Practitioner Assessment of Disease Burden in Systemic Sclerosis
Maylen Nordgård Carstens¹, Håvard Fretheim¹, Imon Barua¹, Henriette Didriksen², Torhild Garen¹, Øyvind Midtvedt¹, Øyvind Molberg³ and Anna-Maria Hoffmann-Vold¹, ¹Oslo University Hospital, Oslo, Norway, ²Oslo University Hospital, Moss, Norway, ³Department of Rheumatology, Oslo University Hospital, Oslo, Norway, Oslo, Norway
Background/Purpose: Assessment of disease burden is crucial in the management of systemic sclerosis (SSc). The patient global assessment (PatGA) is a patient self-reported measure widely…
Abstract Number: 1231 • ACR Convergence 2022
[68Ga]- CPCR4 Trifluoroacetate PET/CT Imaging of Chemokine Receptor CXCR4- Expression in Systemic Sclerosis Related Interstitial Lung Disease
Chirag Rajkumar Kopp¹, Shefali Sharma², Venkata Subramanian Krishnaraju³, Chandra Bhushan Prasad³, Jagdeep Singh³, Shashi Anand³, Ranjana Minz³, Ashwani Sood³, Anindita Sinha³, Rajender Kumar Basher³, Sahajal Dhooria³, Varun Dhir⁴ and sanjay jain³, ¹Post Graduate Institute of Medical Education and Research, Chandigarh, India, ²PGIMER< Chandigarh, Chandigarh, India, ³Postgraduate Institute of Medical Education and Research, Chandigarh, India, ⁴PGIMER, CHD, INDIA, Chandigarh, India
Background/Purpose: Interstitial lung disease (ILD) in Systemic sclerosis (SSc) is a major contributor of morbidity and mortality. CXCR4/CXCL12 axis has been implicated in the pathogenesis…
Abstract Number: 1542 • ACR Convergence 2022
Exploratory Clinical Subgroup Clustering in Systemic Sclerosis – Results from the Indian Progressive Systemic Sclerosis Registry
Shery Susan Philip¹, Ramya Janardana¹, Revanesh S Mirji¹, Chengappa Kavadichanda², Devender Bairwa³, Geetabali Sircar⁴, Parasar Ghosh⁵, Anupam Wakhlu⁶, Padmanabha Shenoy⁷, Sumithra Selvam⁸ and Vineeta Shobha¹, ¹St. John’s Medical College Hospital, Bangalore, India, ²JIPMER, Pondicherry, Puducherry, India, ³Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, Puducherry, India, ⁴Institute of Postgraduate Medical Education and Research, Kolkata, West Bengal, India, ⁵Govt of West Bengal, Kolkata, India, Kolkata, West Bengal, India, ⁶Apollo Medics Super Speciality Hospitals, Lucknow, India, ⁷Centre for Arthritis and Rheumatism Excellence (CARE), Cochi, India, ⁸Division of Epidemiology and Biostatistics, St. John’s Research Institute, St. John's Medical College Hospital, Bangalore, India
Background/Purpose: Classification of systemic sclerosis (SSc) into subsets has been a challenge due to it's heterogeneity. This study attempts to identify SSc subsets based on…
Abstract Number: 2169 • ACR Convergence 2022
Cardiac Rhythm and Conduction Abnormalities in Systemic Sclerosis-meta-analysis
Drashti Antala¹, Angkawipa Trongtorsak¹, Qingqing Meng¹, Akshaya Ramachandran¹, Aanshi Patel², Pabitra Adhikari¹, Prasun Pudasainee¹ and Krutarth Pandya³, ¹Ascension Saint Francis hospital, Evanston, IL, ²B.J. Medical College, Ahmedabad, Ahmedabad, India, ³Trumbull Regional Medical Centre, Warren, OH
Background/Purpose: Systemic sclerosis( SSc) can primarily cause repeated focal ischaemic insults in the myocardium and result in irreversible myocardial fibrosis. Myocardial involvement, when becomes clinically…
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…
Abstract Number: 1624 • ACR Convergence 2022
Blocking IL-1, IL-33 and IL-36 Signaling with the Anti-IL1RAP Antibody mCAN10 Ameliorates Inflammation and Fibrosis in Preclinical Models of Systemic Sclerosis
Caitriona Grönberg¹, Sara Rattik¹, Meik Kunz², Thoung Trinh-Minh³, Cuong Tran-Manh³, Xiang Zhou³, Petter Skoog¹, David Liberg¹ and Jörg Distler³, ¹Cantargia AB, LUND, Sweden, ²Friedrich-Alexander University (FAU) Erlangen-Nuremberg, Erlangen, Germany, ³Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany
Background/Purpose: The IL-1 receptor accessory protein (IL1RAP) is a co-receptor required for signaling through the IL-1, IL-33, and IL-36 receptors. IL1RAP-dependent signaling has been implicated…
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: 0712 • ACR Convergence 2022
Disease Prevalence and Patient Characteristics in Patients with and Without Scleroderma Renal Crisis – A National US-based Inpatient Comparison Study
Anila Hussain¹, Pushti Khandwala² and Thais Moldovan³, ¹Crozer Chester Medical Center, Glen Mills, PA, ²Einstein Medical Center Philadelphia, Glen Mills, PA, ³Rheumatology Care Center, Media, PA
Background/Purpose: Scleroderma renal crisis is a rare complication of Systemic Sclerosis (SSc) with high mortality and morbidity. Most patients present with acute symptomatic increase in…

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