Abstracts tagged "Scleroderma"

Abstract Number: 0516 • ACR Convergence 2022
Identifying Current Assessment and Treatment Strategies in Juvenile Systemic Sclerosis: An International Effort to Improve Patient Outcomes
Natalia Vasquez Canizares¹, Clare Pain², Marinka Twilt³, Amra Adrovic Yildiz⁴, Simone Appenzeller⁵, Edoardo Marrani⁶, Emanuela Del Giudice⁷, Antonella Petaccia⁸, Francesca Tirelli⁹, Gabriele Simonini¹⁰, Marco Cattalini¹¹, Ozgur Kasapcopur¹², Raju Khubchandani¹³, Kathryn Torok¹⁴, Betul Sozeri¹⁵, Biagio Castaldi¹⁶, Katherine Clarke¹⁷, Phuoc Duong¹⁷, Aybuke Gunalp¹⁸, meiping lu¹⁹, Hanna Lythgoe², Amanda Robinson¹⁴, Lauren Robinson²⁰, Paulo Rogerio Julio²¹, Susan Shenoi²², Emily Willis²³, Mustafa Çakan¹⁵, Valentina Leone²⁴, Rongjun Zheng¹⁹, Phillip Lawrence¹⁷, Franziska Rosser²⁵, Sarah Mayell², Lusine Ambartsumyan²⁶, Aurelia Minuti²⁷, Francesco Zulian²⁸ and Suzanne Li²⁹, ¹Children's Hospital at Montefiore/ Albert Einstein College of Medicine, New York, NY, ²Alder Hey Children’s NHS Foundation Trust, Liverpool, United Kingdom, ³Alberta Children's Hospital, Calgary, AB, Canada, ⁴School of Medicine, KOC University, Istanbul, Turkey, ⁵Unicamp, Campinas, São Paulo, Brazil, ⁶AOU Meyer, Florence, Italy, ⁷Sapienza University of Rome, Pediatric and Neonatology Unit, Maternal and Child Department, Rome, Italy, ⁸Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy, ⁹Women's and Child's Health, University Hospital of Padova, Padova, Italy, ¹⁰Meyer Children's Hospital, Florence, Italy, ¹¹Pediatric Clinic, University of Brescia, Brescia, Italy, ¹²Istanbul University-Cerrahpaşa, Cerrahpaşa Medical School, Istanbul, Turkey, ¹³SRCC Children's Hospital, Mumbai, India, ¹⁴Pediatric Rheumatology, Children's Hospital of UPMC, Pittsburgh, PA, ¹⁵University of Health Sciences, Istanbul, Turkey, ¹⁶Women's and Child's Health, University Hospital of Padova, Padua, Italy, ¹⁷Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom, ¹⁸Istanbul University Cerrahpaşa Medical School, Istanbul, Turkey, ¹⁹Children’s Hospital of Zhejiang University Medical School, Zhejiang, China, ²⁰Hospital for Special Surgery, New York, NY, ²¹University of Campinas, Campinas, São Paulo, Brazil, ²²Seattle Children's Hospital and Research Center / University of Washington, Seattle, WA, ²³Royal Manchester Children’s Hospital, Manchester, United Kingdom, ²⁴Ismett, UPMC, Palermo, Italy, ²⁵University of Pittsburgh, Children’s Hospital of UPMC, Pittsburgh, PA, ²⁶Seattle Children's Hospital, University of Washington, Seattle, WA, ²⁷D. Samuel Gottesman Library Research and Education, Albert Einstein College of Medicine, Bronx, NY, ²⁸University of Padua, Padua, Italy, ²⁹Hackensack Meridian School of Medicine, Joseph M. Sanzari Children's Hospital, West Orange, NJ
Background/Purpose: Juvenile Systemic Sclerosis (jSSc) is an autoimmune and fibrosing disease associated with significant morbidity and mortality risk. Data on treatment is limited and based…
Abstract Number: 1069 • ACR Convergence 2022
Renal Involvement Other Than Renal Crisis in Systemic Sclerosis: A Retrospective Monocentric Cohort Study
Gaelle Becel¹, Bertrand Dunogue¹, Benjamin Chaigne¹, Benjamin Thoreau², Alexis Regent¹, Alexandre Karras³, Benjamin Terrier¹ and Luc Mouthon¹, ¹National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, ²AP-HP, Université Paris Cité, INSERM U1016, CNR UMR 8104, Paris, France, ³HEGP, Paris, France
Background/Purpose: Scleroderma renal crisis has extensively been described over the past decades because of its severity. The use of angiotensin-converting enzyme inhibitors has clearly improved…
Abstract Number: 1528 • ACR Convergence 2022
Nintedanib in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD) and Organ Damage: Data from the SENSCIS Trial
Janet Pope¹, Susanna Proudman², Wendy Stevens³, Joerg Henes⁴, Rozeta Simonovska⁵, Margarida Alves⁶ and Yannick Allanore⁷, ¹University of Western Ontario, London, ON, Canada, ²Rheumatology Unit, Royal Adelaide Hospital, and Discipline of Medicine, University of Adelaide, Adelaide, Australia, ³Department of Rheumatology, St Vincent’s Hospital, Melbourne, Australia, Melbourne, Australia, ⁴Center for Interdisciplinary Clinical Immunology, Rheumatology and Auto-inflammatory Diseases (INDIRA), University Hospital Tübingen, Tübingen, Germany, ⁵mainanalytics GmbH, Sulzbach (Taunus), Germany, Sulzbach (Taunus), Germany, ⁶Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, ⁷Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France
Background/Purpose: The Scleroderma Clinical Trials Consortium Damage Index (SCTC-DI) was developed to quantify organ damage in patients with SSc. We assessed outcomes in the SENSCIS…
Abstract Number: 2159 • ACR Convergence 2022
Gastroesophageal Reflux Disease in an Australian Scleroderma Cohort – Associations and Effect of Treatment
Alannah Quinlivan¹, Dennis Neuen², Dylan Hansen³, Wendy Stevens³, Laura Ross⁴, Nava Ferdowsi³, Susanna Proudman⁵, Jenny Walker⁶, Jo Sahhar⁷, Gene-Siew Ngian⁸, Diane Apostolopoulos⁹, Lauren Host¹⁰, Gabor Major¹¹, Kathleen Morrisroe³ and Mandana Nikpour¹², ¹St Vincent's Hospital Melbourne and the University of Melbourne, Melbourne, Australia, ²Liverpool Hospital, Sydney, Australia, ³St Vincent's Hospital Melbourne, Melbourne, Australia, ⁴The University of Melbourne at St. Vincent's Hospital, Brunswick, Australia, ⁵University of Adelaide, Medindie, Australia, ⁶Flinders Medical Centre, Flinders University, Daw Park, Australia, ⁷Monash Health, Melbourne, Australia, ⁸Melbourne Health, Northcote, Australia, ⁹Monash University, Melbourne, Australia, ¹⁰Fiona Stanley Hospital, London, United Kingdom, ¹¹Hunter New England Health Service, Rankin Park - Newcastle, New South Wales, Australia, ¹²The University of Melbourne at St. Vincent's Hospital Melbourne, Melbourne, Australia
Background/Purpose: To investigate the association between gastroesophageal reflux disease (GORD) and interstitial lung disease (ILD) and determine the effect of GORD treatment on survival in…
Abstract Number: 0519 • ACR Convergence 2022
Myeloablation Followed by Hematopoietic Stem Cell Transplantation Leads to Long-term Normalization of Systemic Sclerosis Molecular Signatures
Nancy Wareing¹, Xuan Wang², Lynette Keyes-Elstein³, Ellen Goldmuntz⁴, Marka Lyons⁵, Peter McSweeney⁶, Daniel Furst⁷, Richard Nash⁶, Leslie Crofford⁸, Beverly Welch⁹, Ashley Pinckney¹⁰, Maureen Mayes¹¹, Keith Sullivan¹² and Shervin Assassi¹³, ¹UTHealth Houston McGovern Medical School, Houston, TX, ²Baylor Institute for Immunology Research, Dallas, TX, ³Rho, Chapel Hill, NC, ⁴NIAID/ NIH, Washington, DC, ⁵University of Texas McGovern Medical School at Houston, Houston, TX, ⁶Colorado Blood Cancer Institute, Denver, CO, ⁷University of California Los Angeles, Los Angeles, CA, ⁸Vanderbilt University Medical Center, Nashville, TN, ⁹NIAID/NIH, Bethesda, MD, ¹⁰Rho, St. Louis Park, MN, ¹¹Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, TX, ¹²Duke University, Durham, NC, ¹³McGovern Medical School, University of Texas, Houston, TX
Background/Purpose: In the randomized Scleroderma: Cyclophosphamide Or Transplantation (SCOT trial), normalization of systemic sclerosis (SSc) peripheral blood gene expression signatures at the 26-month visit was…
Abstract Number: 1114 • ACR Convergence 2022
Computational Drug Repositioning and 3D Skin Models Identify EGFR and PI3K Inhibitors as Anti-fibrotic Targets for Systemic Sclerosis
Dillon Popovich¹, Noelle Kosarek², Rezvan Parvizi³, Tamar Abel⁴, Mengqi Huang⁵, Monica Espinoza⁶, Avi Smith⁷, Sasha Shenk⁷, Jonathan Garlick⁷ and Michael Whitfield⁸, ¹Dartmouth College, West Lebanon, NH, ²Dartmouth Geisel School of Medicine, Lebanon, NH, ³Dartmouth, Lebanon, NH, ⁴Dartmouth College, Lebanon, NH, ⁵University of Pittsburgh, Pittsburgh, PN, ⁶Dartmouth College, Hanover, NH, ⁷Tufts University School of Dental Medicine, Boston, MA, ⁸Geisel School of Medicine, Lebanon, NH
Background/Purpose: Genome-wide analyses of gene expression in systemic sclerosis (SSc) skin biopsies have identified differential responses based on the mechanism of action of the therapy.…
Abstract Number: 1529 • ACR Convergence 2022
Clinical Nailfold Capillaroscopy Using Dinolite Microscope in Patients with Systemic Sclerosis
Krishnasai Abhishek Madathanapalli¹, Alyssa Williams², Baran Ilayda Gunes², Francis Perry Wilson³, John Pauling⁴, Robyn Domsic⁵, Lan Yu⁶ and Monique Hinchcliff⁷, ¹University of Connecticut School of Medicine, Hartford, CT, ²Yale University, New Haven, CT, ³Yale school of medicine, New Haven, CT, ⁴North Bristol NHS Trust, Bristol, United Kingdom, ⁵University of Pittsburgh, Pittsburgh, PA, ⁶University of Pittsburgh School of Medicine, Pittsburgh, ⁷Yale School of Medicine, Westport, CT
Background/Purpose: The clinical importance of nailfold capillaroscopy (NFC) has grown since its inclusion in the 2013 ACR/EULAR Classification Criteria for systemic sclerosis (SSc), yet few…
Abstract Number: 2161 • ACR Convergence 2022
Alterations of Nutritional Status of Scleroderma Patients and Associations with Disease-specific Features
Sabína Oreská¹, Hana Storkanova¹, Maja Spiritovic², Barbora Hermankova², Michal Vrablík³, Karel Pavelka⁴, Jiří Vencovský⁵, Ladislav Šenolt⁵, Radim Becvar¹ and Michal Tomcik¹, ¹Institute of Rheumatology, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic, Prague, Czech Republic, ²Institute of Rheumatology, Department of Physiotherapy, Faculty of Physical Education and Sport, Charles University, Prague, Czech Republic, Prague, Czech Republic, ³3rd Department of Internal Medicine, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic, Prague, Czech Republic, ⁴Institute of Rheumatology, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic, Praha, Czech Republic, ⁵Institute of Rheumatology and Department of Rheumatology, First Faculty of Medicine, Charles University, Prague, Czech Republic
Background/Purpose: Systemic sclerosis (SSc) is characterized by skin and organ involvement and chronic disease course. In particular, involvement of the gastrointestinal tract and systemic inflammation…
Abstract Number: 0521 • ACR Convergence 2022
Examination of Differential Response to Treatment with Mycophenolate Mofetil in Black and White Patients with Systemic Sclerosis
Lauren Smith¹, Jamie Perin², Adrianne Woods², Fredrick Wigley³, Laura Hummers⁴ and Ami Shah⁵, ¹Johns Hopkins, District Heights, MD, ²John Hopkins University, Baltimore, MD, ³Johns Hopkins University, Baltimore, MD, ⁴Johns Hopkins Univerisity, Baltimore, MD, ⁵Johns Hopkins Rheumatology, Baltimore, MD
Background/Purpose: Black patients with systemic sclerosis (SSc) develop SSc at a younger age and have more severe disease than White patients, including a higher prevalence…
Abstract Number: 1166 • ACR Convergence 2022
Scleroderma Fibroblasts Induce Alternative Macrophage Activation via an ERK1/2-dependent Mechanism
Taha Dinc¹, Juan-Pablo Zertuche¹, Fatima El Adili², Maria Trojanowska³ and Andreea Bujor³, ¹Boston University School of Medicine, Boston, MA, ²Boston University School of Medicine, Revere, MA, ³Boston University, Boston, MA
Background/Purpose: Activation of the innate immune system in systemic sclerosis (SSc), with an increased number of profibrotic/alternatively-activated macrophages in affected tissues, is an important aspect…
Abstract Number: 1530 • ACR Convergence 2022
Scleroderma Renal Crisis: Time to Initiation of Therapy Does Not Affect Mortality
Aditi Patel¹, Chao Zhang¹ and Soumya Chatterjee², ¹Cleveland Clinic, Cleveland, OH, ²Cleveland Clinic, Richmond Heights, OH
Background/Purpose: Scleroderma renal crisis (SRC) is a rare complication in patients with systemic sclerosis (SSc), characterized by accelerated hypertension and acute kidney injury. ACE inhibitors…
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: 0523 • ACR Convergence 2022
The Development of the Ranked Composite Important Difference in Diffuse Cutaneous Systemic Sclerosis; A Clinical and Patient Meaningful Anchor to the ACR-Composite Response Index in SSc
Lesley-Anne Bissell¹, Daniel Furst², sindhu johnson³, Paul hansen⁴, Esmeralda Recalde⁵, Dinesh Khanna⁶ and Francesco Del Galdo⁷, ¹University of Leeds, Leeds, United Kingdom, ²University of California Los Angeles, Los Angeles, CA, ³Rheumatology, Department of Medicine, Schroeder Arthritis Institute, Toronto Western Hospital, Mount Sinai Hospital; Institute of Health Policy, Management, and Evaluation, University of Toronto, Toronto, ON, Canada, ⁴University of Otago, Dunedin, New Zealand, ⁵World Scleroderma Foundation, Florence, Italy, ⁶Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI, ⁷Leeds Institute of Rheumatic and Musculoskeletal Medicine, Faculty of Medicine and Health and NIHR Biomedical Research Centre, University of Leeds, Leeds, United Kingdom
Background/Purpose: The ACR Composite Response Index in Systemic Sclerosis ( ACR-CRISS) is one of the first composite outcome measures in diffuse cutaneous Systemic sclerosis (dcSSc-…
Abstract Number: 1169 • ACR Convergence 2022
Downregulated Fli1 in Systemic Sclerosis Myeloid Cells Contributes to Enhanced Transendothelial Migration
Pablo Weilg Espejo¹, Taha Dinc², Fatima El Adili³, Maria Trojanowska⁴ and Andreea Bujor⁴, ¹Boston Medical Center, Chelsea, MA, ²Boston University School of Medicine, Boston, MA, ³Boston University School of Medicine, Revere, MA, ⁴Boston University, Boston, MA
Background/Purpose: The mononuclear phagocytic system in Systemic Sclerosis (SSc) is central to fibrogenesis and may contribute to fibrosis via enhanced monocyte migration into injured tissues…
Abstract Number: 1531 • ACR Convergence 2022
Continued Treatment with Nintedanib in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD): Three-Year Data from SENSCIS-ON
Yannick Allanore¹, Madelon Vonk², Oliver Distler³, Arata Azuma⁴, Maureen Mayes⁵, Alexandra James⁶, Veronika Kohlbrenner⁷, Margarida Alves⁸, Dinesh Khanna⁹ and Kristin Highland¹⁰, ¹Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France, ²Department of Rheumatology, Radboud University Medical Center, Nijmegen, Netherlands, ³Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ⁴Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan, ⁵Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, TX, ⁶Elderbrook solutions GmbH, Bietigheim-Bissingen, Germany, Bietigheim-Bissingen, Germany, ⁷Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, Connecticut, USA, Ridgefield, CT, ⁸Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, ⁹Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI, ¹⁰Cleveland Clinic, Cleveland, Ohio, USA, Cleveland, OH
Background/Purpose: In the randomized placebo-controlled SENSCIS trial in patients with SSc-ILD, nintedanib reduced the rate of decline in forced vital capacity (FVC) (mL/year) over 52…

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