Abstracts tagged "Juvenile scleroderma"

Abstract Number: 078 • 2020 Pediatric Rheumatology Symposium
Reliability and Validity of a New Skin Activity Measure for Localized Scleroderma
Suzanne Li¹, Mara Becker ², Sandy Hong ³, Polly Ferguson ⁴, Themba Nyrienda ⁵, Tracy Andrews ⁶, Katie Stewart ⁷, C. Egla Rabinovich ⁸, Robert Fuhlbrigge ⁹, Thomas Mason ¹⁰, Elena Pope ¹¹, Maria Ibarra ¹², Fatma Dedeoglu ¹³, Gloria Higgins ¹⁴, Ronald Laxer ¹⁵, Marilynn Punaro ¹⁶ and Kathryn Torok ¹⁷ for the CARRA investigators, ¹Joseph M. Sanzari Children's Hospital Hackensack Meridian Health, Hackensack, ²Duke University Medical Center/Duke Clinical Research Institute, Chapel Hill, ³University of Iowa Stead Family Children's Hospital, Iowa City, ⁴University of Iowa Carver College of Medicine, Iowa City, ⁵Hackensack Univesity Medical Center, Hackensack, ⁶Rutgers University, Newark, ⁷UT Southwestern, Dallas, ⁸Duke University Hospital, Durham, ⁹University of Colorado, Aurora, ¹⁰Rochester, ¹¹Hospital For Sick Kids, Toronto, Canada, ¹²Children's Mercy Kansas City, Kansas City, ¹³Boston Children's Hospital, Boston, ¹⁴Nationwide Childrens Hospital/ The Ohio State University, Columbus, ¹⁵The Hospital for Sick Children and University of Toronto, Toronto, Canada, ¹⁶Dallas, Texas, ¹⁷Pediatric Rheumatology, Univ of Pittsburgh Med Ctr, Pittsburgh
Background/Purpose: Juvenile localized scleroderma (jLS) is a chronic inflammatory and fibrosing disease. Treatment is directed towards controlling disease activity to minimize risk for functional impairment…
Abstract Number: 023 • 2020 Pediatric Rheumatology Symposium
Capturing the Range of Disease Involvement in Localized Scleroderma: The Total Morbidity Score
Suzanne Li¹, Aashka Patel ², Elena Pope ³, Thomas Mason ⁴, Vidya Sivaraman ⁵, Fatma Dedeoglu ⁶, Kathryn Torok ⁷, Katie Stewart ⁸, Gloria Higgins ⁹, C. Egla Rabinovich ¹⁰, Robert Fuhlbrigge ¹¹, Maria Ibarra ¹², Sandy Hong ¹³, Polly Ferguson ¹⁴, Mara Becker ¹⁵, Brian Feldman ¹⁶ and Ronald Laxer ¹⁷ for the CARRA investigators, ¹Joseph M. Sanzari Children's Hospital Hackensack Meridian Health, Hackensack, ²Hackensack University Medical Center, Hackensack, ³Hospital For Sick Kids, Toronto, Canada, ⁴Rochester, ⁵Division of Rheumatology, Nationwide Children's Hospital, Bexley, ⁶Boston Children's Hospital, Boston, ⁷Pediatric Rheumatology, Univ of Pittsburgh Med Ctr, Pittsburgh, ⁸UT Southwestern, Dallas, ⁹Nationwide Childrens Hospital/ The Ohio State University, Columbus, ¹⁰Duke University Hospital, Durham, ¹¹University of Colorado, Aurora, ¹²Children's Mercy Kansas City, Kansas City, ¹³University of Iowa Stead Family Children's Hospital, Iowa City, ¹⁴University of Iowa Carver College of Medicine, Iowa City, ¹⁵Duke University Medical Center/Duke Clinical Research Institute, Chapel Hill, ¹⁶The Hospital for Sick Children, Toronto, Canada, ¹⁷The Hospital for Sick Children and University of Toronto, Toronto, Canada
Background/Purpose: Localized scleroderma (LS) is a chronic inflammatory and fibrosing disease that causes both cutaneous and extracutaneous (EC) damage. EC involvement (ECI) is common in…
Abstract Number: 024 • 2020 Pediatric Rheumatology Symposium
Abatacept Treatment Reduces Cutaneous and Joint Activity in Juvenile Localized Scleroderma
Suzanne Li¹, Sarah Ishaq ², Mary Buckley ³, Kathryn Torok ⁴, Barbara Edelheit ⁵, Kaleo Ede ⁶ and C. Egla Rabinovich ⁷, ¹Joseph M. Sanzari Children's Hospital Hackensack Meridian Health, Hackensack, ²Montclair State University, montclair, ³Duke University, Durham, ⁴Pediatric Rheumatology, Univ of Pittsburgh Med Ctr, Pittsburgh, ⁵CT Children's Medical Center, Hartford, ⁶Phoenix Children's Hospital, Phoenix, ⁷Duke University Hospital, Durham
Background/Purpose: Juvenile localized scleroderma (jLS) is an autoimmune disease commonly associated with damage. Damage includes dyspigmentation, tissue atrophy, arthropathy, hemiatrophy, vision loss, and seizures. To…
Abstract Number: 025 • 2020 Pediatric Rheumatology Symposium
HLA Genetic Signatures Associated with Inflammatory Sub-type in Juvenile Localized Scleroderma
Christina Schutt¹, Emily Mirizio ², Kaila Schollaert-Fitch ² and Kathryn Torok ², ¹UPMC Children's Hospital of Pittsburgh, Pittsburgh, ²Pediatric Rheumatology, Univ of Pittsburgh Med Ctr, Pittsburgh
Background/Purpose: Juvenile localized scleroderma (jLS) is an autoimmune disease of the skin and underlying tissue that is characterized by an earlier inflammatory infiltrate, followed by…
Abstract Number: 771 • 2019 ACR/ARP Annual Meeting
Von Willebrand Factor Is Localized in the Extravascular Tissue of Patients with Juvenile Scleroderma
Natalia Vasquez-Canizares¹, Beamon Agarwal ², Tamar Rubinstein ³, Dawn Wahezi ⁴ and Morayma Reyes Gil ⁵, ¹Children's Hospital at Montefiore, Bronx, NY, ²GenomeRxUS LLC, Providence, PA, ³Children's Hospital at Montefiore/Albert Einstein College of Medicine, Bronx, NY, ⁴Children’s Hospital at Montefiore, New York, ⁵Montefiore Medical Center/Albert Einstein College of Medicine, Bronx
Background/Purpose: Von Willebrand Factor (VWF) is a glycoprotein synthesized in endothelial cells and megakaryocytes that has an essential role in primary hemostasis. There is increasing…
Abstract Number: 776 • 2019 ACR/ARP Annual Meeting
Genetic Signatures Support Inflammation Driven Fibrosis in Localized Scleroderma
Christina Schutt¹, Emily Mirizio ², Claudia Salgado ³, Miguel Reyes-Mugica ³, Kaila L. Schollaert ² and Kathryn Torok ³, ¹UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, ²University of Pittsburgh, Pittsburgh, ³UPMC Children's Hospital of Pittsburgh, Pittsburgh
Background/Purpose: Localized scleroderma (LS) is a progressive autoimmune disease of the skin and underlying tissue that is characterized by an initial inflammatory infiltration which is…
Abstract Number: 791 • 2019 ACR/ARP Annual Meeting
Closing the Seronegative Gap in Pediatric Localized Scleroderma and Systemic Sclerosis
May Choi¹, Emily Mirizio ², Fernanda Quinteros ³, Katherine Buhler ⁴, Marvin Fritzler ¹ and Kathryn Torok ⁵, ¹Cumming School of Medicine, University of Calgary, Calgary, AB, Canada, ²University of Pittsburgh, Pittsburgh, ³University of Calgary, Calgary, ⁴University of Calgary, Calgary, Canada, ⁵UPMC Children's Hospital of Pittsburgh, Pittsburgh
Background/Purpose: It has become increasingly recognized that extra-cutaneous manifestations, such as musculoskeletal and neurologic involvement, are common in pediatric patients with localized scleroderma (LS). We…
Abstract Number: 792 • 2019 ACR/ARP Annual Meeting
Speckle Tracking Echocardiography, a Sensitive Tool to Detect Early Cardiac Dysfunctions in Juvenile Systemic Sclerosis
Giovanni Civieri ¹, Biagio Castaldi ¹, Giorgia Martini ¹, Alessandra Meneghel ¹, Ornella Milanesi ¹ and Francesco Zulian², ¹Dept. of Woman's and Child's Health, university of Padua, Italy, Padua, Italy, ²Department of Woman and Child Health, University of Padua, Italy, Padova, Italy
Background/Purpose: Juvenile Systemic Sclerosis (JSSc) is a rare connective tissue disease in which cardiac involvement is burdened by high morbidity and mortality. The traditional cardiac…
Abstract Number: 1281 • 2017 ACR/ARHP Annual Meeting
Plasma CXCL4 As a Biomarker in Juvenile Systemic Sclerosis
Katharine Moore^1,2, Marvin J. Fritzler³, Marisa S. Klein-Gitelman⁴, Ann M Reed⁵, Tzielan Lee⁶ and Anne Stevens^7,8, ¹Pediatrics, University of Colorado, Aurora, CO, ²Pediatric Rheumatology, Children's Hospital Colorado, Aurora, CO, ³Medicine, University of Calgary, Calgary, AB, Canada, ⁴Division of Pediatric Rheumatology/PDD PTD, Lurie Children's Hospital of Chicago/Northwestern University, Chicago, IL, ⁵Rheumatology, Duke University, Durham, NC, ⁶Pediatric Rheumatology, Stanford University, Palo Alto, CA, ⁷University of Washington, Department of Pediatrics, Seattle, WA, ⁸Center for Immunity and Immunotherapies, Seattle Children's Research Institute, Seattle, WA
Background/Purpose: Juvenile systemic sclerosis (jSSc) is a severe and heterogeneous autoimmune vasculopathy. Pulmonary fibrosis is the highest independent predictor of mortality, yet currently there are…
Abstract Number: 2390 • 2016 ACR/ARHP Annual Meeting
Proposal of Assessment of the Activity of Juvenile Localised Scleroderma. Results of the Consensus Meeting in Hamburg, Germany December 2015
Ivan Foeldvari¹, Eileen Baildam², Michael Blakley³, Christina Boros⁴, Kim Fligelstone⁵, Antonia Kienast¹, Dana Nemkova⁶, Clare Pain², Amanda Saracino⁴, Gabriele Simonini⁷, Kathryn S. Torok⁸, Lisa Weibel⁹ and Nicola Helmus¹, ¹Hamburg Center for Pediatric and Adolescent Rheumatology, Hamburg, Germany, ²Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom, ³Internal Medicine and Pediatrics, Indiana University School of Medicine and Riley Hospital for Children at IU Health, Indianapolis, IN, ⁴Pediatric Rheumatology, London, United Kingdom, ⁵Manchester Academic Health Science Centre, Centre for Musculoskeletal Research, Institute of Inflammation and Repair, The University of Manchester, Manchester, United Kingdom, ⁶Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, ⁷Pediatric Rheumatology, Anna Meyer Children's Hospital, Florence, Italy, ⁸Pediatric Rheumatology, Univ of Pittsburgh Med Ctr, Pittsburgh, PA, ⁹Pediatric Dermatology, University Childrenxs Hospital, Zurich, Switzerland
Background/Purpose: Juvenile Localised Scleroderma (JLS) is an orphan disease which is complicated by difficulties in robust measurement of disease activity. Several outcome measures to assess…
Abstract Number: 420 • 2015 ACR/ARHP Annual Meeting
Description of the Juvenile Localized Scleroderma Subgroup of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry
Eveline Y. Wu¹, Suzanne C. Li², Kathryn S. Torok³, Yamini Virkud⁴, Robert C. Fuhlbrigge⁵, C. Egla Rabinovich⁶ and CARRA Registry Investigators, ¹Pediatrics, University of North Carolina at Chapel Hill, Chapel Hill, NC, ²Pediatrics, Hackensack University Medical Center, Hackensack, NJ, ³Pediatric Rheumatology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, ⁴Pediatrics, Massachusetts General Hospital for Children, Boston, MA, ⁵Pediatric Rheumatology, Boston Children's Hospital, Boston, MA, ⁶Pediatric Rheumatology, Duke University Medical Center, Durham, NC
Background/Purpose: Localized scleroderma (LS) is a chronic inflammatory and fibrosing skin disease. We present baseline data on the juvenile LS (jLS) cohort from the Childhood…
Abstract Number: 424 • 2015 ACR/ARHP Annual Meeting
Magnetic Resonance and Echocardiographic Strain Rate Imaging for the Early Detection of Cardiac Involvement in Juvenile Systemic Sclerosis
Francesco Zulian¹, Marta Balzarin¹ and Elena Reffo, Biagio Castaldi, Giorgia Martini, Alessandra Meneghel, Ornella Milanesi, ¹Department of Pediatrics, University of Padua, Padua, Italy
Background/Purpose: Cardiac involvement is one of the worst prognostic factors in JSSc. The diagnosis is usually based on clinical symptoms, EKG and conventional echocardiography, but…
Abstract Number: 2178 • 2013 ACR/ARHP Annual Meeting
Juvenile Onset Systemic Sclerosis: Clinical and Serological Features, and Mortality In Comparison With Adult Onset Disease
Juan G. Ovalles-Bonilla¹, Francisco Javier López-Longo², Indalecio Monteagudo¹, Esperanza Naredo¹, Carlos Gonzalez Fernandez¹, María Montoro Alvarez¹, Lina Martínez-Estupiñán¹, Juan C. Nieto³, Julia Martínez-Barrio¹, Michelle Hinojosa¹, Natalia Bello¹, Belen Serrano¹, Carmen Mata¹ and Luis Carreño¹, ¹Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain, ²Hospital General Universitario Gregorio Marañón, Department of Rheumatology, Madrid, Spain, ³Hospital General Universitario Gregorio Marañón, Madrid, Spain
Background/Purpose: Currently data regarding long-term outcome of juvenile systemic sclerosis (jSSc) is scarce. To describe the differences between patients with jSSc versus adult onset evaluated at a single medical…

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