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  • Abstract Number: 412 • 2015 ACR/ARHP Annual Meeting

    Update on the Juvenile Systemic Sclerosis Inception Cohort Project. Characteristics of the First 50 Patients at First Assessment. Www.Juvenile-Scleroderma.Com

    Ivan Foeldvari1, Jens Klotsche2, Valda Staņeviča3, Ekaterina Alexeeva4, Maria M. Katsicas5, Maria Teresa Terreri6, Ana Paula Sakamoto7, Rolando Cimaz8, Mikhail Kostik9, Tadey Avcin10, Maria Jose Santos11, Monika Moll12, Dana Nemkova13, Flavio Sztajnbok14, Cristina Battagliotti15, Juergen Brunner16, Despina Eleftheriou17, Alberto Sifuentes Giraldo18, Liora Harel19, Mahesh Janarthanan20, Tilmann Kallinich21, Kirsten Minden22, Susan Mary Nielsen23, Kathryn S. Torok24, Yosef Uziel25 and Nicola Helmus26, 1Hamburger Zentrum für Kinder-und Jugendrheumatologie, Hamburg, Germany, 2Epidemiology unit, German Rheumatism Research Center, Berlin, Germany, 3Pediatric cathedra, Riga Stradiņš University, Riga, Latvia, 4Rheumatology, Scientific Center of Children's Health, Moscow, Russia, 5Immunology & Rheumatology., Hospital de Pediatrìa Garrahan, Buenos Aires, Argentina, 6Pediatrics, Universidade Federal de Sao Paulo, São Paulo, Brazil, 7Assistant doctor, Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil, 8Department of Paediatrics, University of Florence and Anna Meyer Children's Hospital, Florence, Italy, Florence, Italy, 9State Pediatric Medical University, Saint-Petersburg, Russia, 10University Children's Hospital, Ljubljana, Slovenia, 11Rheumatology, Hospital Garcia de Orta, Almada, Portugal, 12Pediatric Rheumatology, University Childrenhospital, Tübingen, Germany, 13Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, 14Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, 15Hospital de Niños Dr Orlando Alasia, Santa Fé, Argentina, 16Universitätsklinik für Kinder- u. Jugendheilkunde, Innsbruck, Austria, 17Paediatric Rheumatology Department, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 18Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain, 19Pediatric Rheumatology Unit, Schneider Children's Medical Center,Tel Aviv University, Petach Tikvah, Israel, 20Pediatric Rheumatology, Chennai, India, 21Charite, University Medicine Berlin, Berlin, Germany, 22Chidlrens´ hospital, Charité University Medicine, Berlin, Germany, 23Rigshospitalet, Copenhagen, Denmark, 24Pediatric Rheumatology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, 25Meir Medical Center, Kfar Saba, Israel, 26Hamburg Centre for Pediatric and Adolescent Rheumatology, Hamburg, Germany

    Background/Purpose: Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Currently just retrospective data exist regarding evolvement of organ involvement. In the previous retrospective studies…
  • Abstract Number: 413 • 2015 ACR/ARHP Annual Meeting

    Is There a Difference in the Presentation of Diffuse and Limited Subtype of Juvenile Systemic Sclerosis in Childhood? Results from the Juvenile Scleroderma Inception Cohort Www.Juvenile-Scleroderma.Com

    Ivan Foeldvari1, Jens Klotsche2, Valda Stanevicha3, Maria M. Katsicas4, Maria Teresa Terreri5, Ana Paula Sakamoto6, Rolando Cimaz7, Mikhail Kostik8, Tadey Avcin9, Maria Jose Santos10, Monika Moll11, Dana Nemkova12, Flavio Sztajnbok13, Cristina Battagliotti14, Juergen Brunner15, Despina Eleftheriou16, Alberto Sifuentes Giraldo17, Liora Harel18, Mahesh Janarthanan19, Tilmann Kallinich20, Kirsten Minden21, Susan Mary Nielsen22, Kathryn S. Torok23, Yosef Uziel24 and Nicola Helmus25, 1Hamburger Zentrum für Kinder-und Jugendrheumatologie, Hamburg, Germany, 2Epidemiology unit, German Rheumatism Research Center, Berlin, Germany, 3Pediatric cathedra, University Childrens Hospital, Riga, Latvia, 4Service of Immunology & Rheumatology. Hospital de Pediatrìa Prof Dr.Juan.P. Garrahan, MD, Buenos Aires, Argentina, 5Pediatrics, Universidade Federal de Sao Paulo, São Paulo, Brazil, 6Assistant doctor, Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil, 7Department of Paediatrics, University of Florence and Anna Meyer Children's Hospital, Florence, Italy, Florence, Italy, 8State Pediatric Medical University, Saint-Petersburg, Russia, 9University Children's Hospital, Ljubljana, Slovenia, 10Rheumatology, Hospital Garcia de Orta, Almada, Portugal, 11Pediatric Rheumatology, University Childrenhospital, Tübingen, Germany, 12Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, 13Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, 14Hospital de Niños Dr Orlando Alasia, Santa Fé, Argentina, 15Universitätsklinik für Kinder- u. Jugendheilkunde, Innsbruck, Austria, 16Paediatric Rheumatology Department, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 17Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain, 18Pediatric Rheumatology Unit, Schneider Children's Medical Center,Tel Aviv University, Petach Tikvah, Israel, 19Pediatric Rheumatology, Chennai, India, 20Charite, University Medicine Berlin, Berlin, Germany, 21Chidlrens´ hospital, Charité University Medicine, Berlin, Germany, 22Rigshospitalet, Copenhagen, Denmark, 23Pediatric Rheumatology, Scleroderma Center of Pittsburgh, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, 24Meir Medical Center, Kfar Saba, Israel, 25Hamburg Centre for Pediatric and Adolescent Rheumatology, Hamburg, Germany

    Background/Purpose: Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Several publications in adults looked at the differences between limited and diffuse subtypes. There is…
  • Abstract Number: 414 • 2015 ACR/ARHP Annual Meeting

    Performance of the Adult Systemic Sclerosis Classification in Juvenile Systemic Sclerosis Patients. Results from the Juvenile Systemic Sclerosis Inception Cohorte Www.Juvenile-Scleroderma.Com

    Ivan Foeldvari1, Jens Klotsche2, Valda Stanevicha3, Ekaterina Alexeeva4, Maria M. Katsicas5, Maria Teresa Terreri6, Ana Paula Sakamoto7, Rolando Cimaz8, Mikhail Kostik9, Tadey Avcin10, Maria Jose Santos11, Monika Moll12, Dana Nemkova13, Flavio Sztajnbok14, Cristina Battagliotti15, Juergen Brunner16, Despina Eleftheriou17, Alberto Sifuentes Giraldo18, Liora Harel19, Mahesh Janarthanan20, Tilmann Kallinich21, Kirsten Minden22, Susan Mary Nielsen23, Kathryn S. Torok24, Yosef Uziel25 and Nicola Helmus26, 1Hamburger Zentrum für Kinder-und Jugendrheumatologie, Hamburg, Germany, 2Epidemiology unit, German Rheumatism Research Center, Berlin, Germany, 3University Childrens Hospital, Riga, Latvia, 4Rheumatology, Scientific Center of Children's Health, Moscow, Russia, 5Hospital de Pediatria, Buenos Aires, Argentina, 6Pediatrics, Universidade Federal de Sao Paulo, São Paulo, Brazil, 7Assistant doctor, Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil, 8Pediatric Rheumatology, Anna Meyer Children's Hospital, Florence, Italy, 9State Pediatric Medical University, Saint-Petersburg, Russia, 10University Children's Hospital, Ljubljana, Slovenia, 11Rheumatology, Hospital Garcia de Orta, Almada, Portugal, 12Pediatric Rheumatology, University Childrenhospital, Tübingen, Germany, 13Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, 14Pediatric Rheumatology Division, Adolescent Health Care Unit, Universida de do Estado do Rio de Janeiro., Rio de Janeiro, Brazil, 15Hospital de Niños Dr Orlando Alasia, Santa Fé, Argentina, 16Universitätsklinik für Kinder- u. Jugendheilkunde, Innsbruck, Austria, 17Paediatric Rheumatology Department, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 18Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain, 19Pediatric Rheumatology Unit, Schneider Children's Medical Center,Tel Aviv University, Petach Tikvah, Israel, 20Pediatric Rheumatology, Chennai, India, 21Charite, University Medicine Berlin, Berlin, Germany, 22Epidemiology, Charite, DRFZ, Berlin, Germany, 23Rigshospitalet, Copenhagen, Denmark, 24Pediatric Rheumatology, Scleroderma Center of Pittsburgh, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, 25Meir Medical Center, Kfar Saba, Israel, 26Hamburg Centre for Pediatric and Adolescent Rheumatology, Hamburg, Germany

    Background/Purpose: The new adult classifiction criteria (1) for Systemic Sclerosis (SSc) were recently published and the pediatric criteria were published in 2007 (2). None of…
  • Abstract Number: 415 • 2015 ACR/ARHP Annual Meeting

    Development of a Juvenile Systemic Sclerosis Response Index (JSSRI)

    Ivan Foeldvari1, Gyorgyi Zoltan2, Eileen Baildam3, Ezgi Deniz Batu4, Michael Blakley5, Mustafa Cakan6, Megan Curran7, Antonia Kienast8, Kim Fligelstone9, Dana Nemkova10, Clarissa Pilkington11, Nicolino Ruperto12, Maria Tsinti13, Elena Tsitsami14, Kristina Urbanvica15 and Tamas Constantin16, 1Hamburger Zentrum für Kinder-und Jugendrheumatologie, Hamburg, Germany, 2Semmelweiss University Budapest, Budapest, Hungary, 3Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom, 4Hacettepe University Vasculitis Center (HUVAC), Ankara, Turkey, 5Internal Medicine and Pediatrics, Indiana University School of Medicine and Riley Hospital for Children at IU Health, Indianapolis, IN, 6Pediatric Rheumatology University Childrens Hospital Istanbul, Istanbul, Turkey, 7Pediatric Immunology/Rheum, UCSF, San Francisco, CA, 8Hamburg Centre for Pediatric and Adolescent Rheumatology, Hamburg, Germany, 9FESCA - Scleroderma parents organisation, UK, United Kingdom, 10Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, 11Paediatric Rheumatology Department, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 12Pediatrica II, Instituto Giannina Gaslini, PRINTO, Genoa, Italy, 13Ped rheum univ child hosp athen, Athena, Greece, 14First Department of Pediatrics, Athens University Medical School, Athens, Greece, 15Pediatric Rheumatology, Riga, Latvia, 16Semmelweis University, Budapest, Hungary

    Background/Purpose: Juvenile systemic sclerosis (jSSc) is an orphan disease. We have currently new promising effective medication to treat systemic sclerosis, but no valid outcome measures…
  • Abstract Number: 416 • 2015 ACR/ARHP Annual Meeting

    Prospective, Standardized, Longitudinal Assessment Reveals Higher Prevalence of Extracutaneous Manifestations in a Pediatric Localized Scleroderma Cohort

    Kaveh Ardalan, Christina Kelsey and Kathryn S. Torok, Pediatric Rheumatology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA

    Background/Purpose: Localized scleroderma (LS) is an autoimmune condition whose hallmarks are progressive skin fibrosis and atrophy.  However, LS is unique among skin diseases for its…
  • Abstract Number: 417 • 2015 ACR/ARHP Annual Meeting

    Gender Differences in Pediatric Localized Scleroderma: Clinical and Patient-Reported Outcomes

    Kaveh Ardalan1, Nicole Hershey1, Jonathan Yabes2, Eveline Wu3, Suzanne C. Li4, Robert C. Fuhlbrigge5, C. Egla Rabinovich6, Kathryn S. Torok1 and The CARRA Registry Investigators, 1Pediatric Rheumatology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, 2Department of Medicine, University of Pittsburgh, Pittsburgh, PA, 3Pediatrics, University of North Carolina - Chapel Hill, Chapel Hill, NC, 4Pediatrics, Joseph M Sanzari Children’s Hospital, Hackensack University Medical Center, Hackensack, NJ, 5Program in Rheumatology, Division of Immunology, Boston Children’s Hospital, Boston, MA, 6Pediatric Rheumatology, Duke University Medical Center, Durham, NC

    Background/Purpose: Localized scleroderma (LS) is an autoimmune disease of the skin and underlying tissue that leads to progressive fibrosis and disability in growing children.  Pediatric…
  • Abstract Number: 418 • 2015 ACR/ARHP Annual Meeting

    Relationship of Race, Ethnicity, and Outcomes in Pediatric Localized Scleroderma: Possible Differences in Disease Activity

    Kaveh Ardalan1, Nicole Hershey1, Jonathan Yabes2, Kathryn S. Torok1 and The CARRA Registry Investigators, 1Pediatric Rheumatology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, 2Department of Medicine, University of Pittsburgh, Pittsburgh, PA

    Background/Purpose: Pediatric localized scleroderma (LS) is an autoimmune skin and soft tissue disease that causes morbidity via progressive skin fibrosis and extracutaneous manifestations (ECMs), such…
  • Abstract Number: 419 • 2015 ACR/ARHP Annual Meeting

    Development and Preliminary Validation of a New Composite Disease Activity Measure for Juvenile Dermatomyositis

    Alessandro Consolaro1, Giulia Camilla Varnier1, Cristina Ferrari1, Jaime De Inocencio2, Adele Civino3, Marija Jelusic-Drazic4, Elena Tsitsami5, Jelena Vojinovic6, Balahan Makay7, Graciela Espada8, Clara Malattia1, Susan Maillard9, Alberto Martini1, Clarissa Pilkington10, Angelo Ravelli1,11 and Kiran Nistala12, 1Pediatria II, Istituto Giannina Gaslini, Genova, Italy, 2Hospital 12 de Octubre, Madrid, Spain, 3Ospedale Cardinale G. Panico, Tricase, Italy, 4University Hospital Center, Zagreb, Croatia, 5First Department of Pediatrics, Athens University Medical School, Athens, Greece, 6Faculty of Medicine, University of Nis, Nis, Serbia, 7Pediatric Rheumatology, Eylul University, Izmir, Turkey, 8Rheumatology Section, Childrens Hosp Ricardo Gutierrez, Buenos Aires, Argentina, 9Paediatric Rheumatology, Great Ormond Street Hospital NHS Foundation Trust for Children, London, United Kingdom, 10Rheumatology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 11University of Genova, Genova, Italy, 12Centre for Rheumatology, University College London, London, United Kingdom

    Background/Purpose: Evaluation of the level of disease activity is a fundamental component of the clinical assessment of children with JDM. The global tools that are currently available for the assessment of the overall disease activity in JDM are centered on physician’s evaluation, neglecting parent’s or child’s perception. Furthermore, these instruments are lengthy and complex. There remains the need for a concise and easily administered tool that provides an absolute measure of disease activity for use in future trials in JDM. Aim of…
  • 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: 421 • 2015 ACR/ARHP Annual Meeting

    Clinical Analysis and Outcome of Interstitial Lung Disease with Juvenile Dermatomyositis

    Satoshi Sato, Yoji Uejima, Eisuke Suganuma, Tadamasa Takano and Yutaka Kawano, Division of Infectious Diseases and Immunology, Saitama Children's Medical Center, Saitama, Japan

    Background/Purpose: Juvenile dermatomyositis (JDM) is the most common idiopathic inflammatory myositis in children. It is a heterogeneous disease and clinical manifestations range from a relatively…
  • Abstract Number: 422 • 2015 ACR/ARHP Annual Meeting

    Mood Disorder Is Highly Prevalent in a Multi-Ethnic Urban Pediatric Lupus Cohort

    Tamar Rubinstein1,2, Dawn Wahezi1,2, Jay Mehta3,4, Norman Ilowite3,5, Debbie Rybak6, Jordan Brodsky6, Nicole Jordan6, Ruth Stein5,7 and Chaim Putterman6, 1Pediatric Rheumatology, Albert Einstein College of Medicine, Bronx, NY, 2Pediatric Rheumatology, Children's Hospital at Montefiore, Bronx, NY, 3Division of Pediatric Rheumatology, Children's Hospital at Montefiore, Bronx, NY, 4Division of Pediatric Rheumatology, Albert Einstein College of Medicine, Bronx, NY, 5Pediatrics, Albert Einstein College of Medicine, Bronx, NY, 6Division of Rheumatology, Albert Einstein College of Medicine, Bronx, NY, 7Pediatrics, Children's Hospital at Montefiore, Bronx, NY

    Background/Purpose: While mood disorder, most commonly manifesting as depression and anxiety symptoms, is often reported in pediatric lupus patients, prevalence rates vary widely. Many published…
  • Abstract Number: 423 • 2015 ACR/ARHP Annual Meeting

    Institutional and Regional Variation in Childhood SLE 30-Day Hospital Readmission Rates: A Comparative Effectiveness Research Using the Pediatric Health Information System Database

    Breanna Beltz1, Mohammad Shah2, Mary Toth3 and Moussa El-Hallak4, 1Hiram College, Akron, OH, 2Patient Safety and Quality Services, Akron Children's Hospital, Akron, OH, 3Rheumatology, Akron Children's Hospital, Akron, OH, 4Dept of Pediatric Rheumatology, Akron Children's Hospital, Akron, OH

    Background/Purpose: Early hospital readmission is emerging as an indicator of care quality. The reported 30-day hospital readmission (30-DHR) rate for pediatric patients is 6.5%. However,…
  • 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: 425 • 2015 ACR/ARHP Annual Meeting

    Impact of in Utero Hydroxychloroquine Exposure on Age of Onset of Cutaneous Neonatal Lupus

    Julie Barsalou1, Nathalie Costedoat-Chalumeau2, Cesar Fors-Nieves3, Ummara Shah4, Patrick Brown5, Carl Laskin6, Nathalie Morel2, Kateri Levesque7, Jill P. Buyon8, Earl Silverman9 and Peter M. Izmirly10, 1Pediatric Rheumatology, CHU Sainte-Justine, Université de Montréal, Montréal, QC, Canada, 2Internal Medicine Department, Cochin Hospital, “René-Descartes Paris V” University, Paris, France, 3Division of Rheumatology, NYU School of Medicine, New York, NY, 4Medicine, Strong Memorial Hospital, University of Rochester, Rochester, NY, 5University of Toronto, Toronto, ON, Canada, 6Medicine, Rheumatology and Obstetrics and Gynecology, University of Toronto and LifeQuest Centre for Reproductive Medicine, Toronto, ON, Canada, 7Internal Medicine, CHU Ste-Justine, Montréal, QC, Canada, 8NYU School of Medicine, New York, NY, 9Division of Rheumatology, Hospital for Sick Children and University of Toronto, Toronto, ON, Canada, 10Rheumatology, NYU School of Medicine, New York, NY

    Background/Purpose: Biopsy specimens of cutaneous neonatal lupus (cNL) lesions usually show interface dermatitis. Hydroxychloroquine (HCQ) is an effective treatment for interface dermatitis seen in connective…
  • Abstract Number: 426 • 2015 ACR/ARHP Annual Meeting

    The Risk of Hospitalized Infection Following Initiation of Biologic Agents Versus Methotrexate in the Treatment of Juvenile Idiopathic Arthritis

    Timothy Beukelman1, Fenglong Xie2, John Baddley3, Lang Chen4, Melissa L. Mannion5, Kenneth G. Saag4, Jie Zhang6 and Jeffrey R. Curtis4, 1Rheumatology, University of Alabama at Birmingham, Birmingham, AL, 2Rheumatology & Immunology, University of Alabama at Birmingham, Birmingham, AL, 3Medicine, University of Alabama at Birmingham, Birmingham, AL, 4University of Alabama at Birmingham, Birmingham, AL, 5Pediatrics, University of Alabama at Birmingham, Birmingham, AL, 6Epidemilogy, University of Alabama at Birmingham, Birmingham, AL

    Background/Purpose: Biologic agents are highly effective for the treatment of juvenile idiopathic arthritis (JIA) but have the potential risk of increased serious infections. Using observational…
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