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  • 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: 0764 • ACR Convergence 2021

    Patients with Juvenile Systemic Sclerosis Have a Distinct Pattern of Organ Involvement: Results from the Juvenile Systemic Sclerosis Inception Cohort

    Ivan Foeldvari1, Jens Klotsche2, Ozgur Kasapcopur3, Amra Adrovic4, Kathryn Torok5, Maria Terreri6, Ana Paula Sakamoto7, Flavio Sztajnbok8, Brian Feldman9, Valda Stanevicha10, Jordi Anton11, Raju Khubchandani12, Ekaterina Alexeeva13, Sindhu Johnson14, Maria Martha Katsicas15, Sujata Sawhney16, Vanessa Smith17, Simone Appenzeller18, Tadej Avcin19, Mikhail Kostik20, Thomas Lehman21, Edoardo Marrani22, Dieneke Schonenberg-Meinema23, Walter Alberto Sifuentes-Giraldo24, Natalia Vasquez-Canizares25, Mahesh Janarthanan26, Hana Malcova27, Monika Moll28, Dana Nemcova29, Anjali Patwardhan30, Maria José Santos31, cristina battagliotti32, Lillemor Berntson33, Blanca Elena Rios Gomes Bica34, Jürgen Brunner35, Rolando Cimaz36, Patricia Costa Reis37, Despina Eleftheriou38, Liora Harel39, Gerd Horneff40, Daniela Kaiser41, Tilmann Kallinich42, Dragana Lazarevic43, Kirsten Minden2, Susan Nielsen44, Farzana Nuruzzaman45, Siri Opsahl Hetlevik46, Yosef Uziel47 and Nicola Helmus48, 1Hamburger Zentrum fuer Kinder- und Jugendrheumatologie, Hamburg, Germany, 2German Rheumatism Research Center, Berlin, Germany, 3Istanbul University-Cerrahpasa, Cerrahpasa Medical School, İstanbul, Turkey, 4Cerrahpasa Medical School, Istanbul, Turkey, 5University of Pittsburgh, Pittsburgh, PA, 6UNIFESP, São Paulo, Brazil, 7Federal University of So Paulo (UNIFESP), São Paulo, Brazil, 8UFRJ/UERJ, Rio de Janeiro, Brazil, 9The Hospital for Sick Children, Toronto, ON, Canada, 10Paediatric Rheumatology International Trials Organisation (PRINTO), Riga, Latvia, 11Hospital Sant Joan de Deu, University of Barcelona, Barcelona, Spain, 12Jaslok Hospital and Research Center, Mumbai, India, 13Scientific Center of Children Health of RAMS, Moscow, Russia, 14University of Toronto, Toronto, ON, Canada, 15Hospital de Pediatria J.P Garrahan, Buenos Aires, Argentina, 16Pediatric Rheumatology Department, Institute of Child Health, Sir Gangarm Hospital, New Delhi, India, 17Department of Rheumatology and Internal Medicine, Ghent University Hospital, Ghent, Belgium, 18Hospital das Clínicas da Universidade Estadual de Campinas, Campinas, Brazil, 19University Medical Center Ljubljana, Ljubljana, Slovenia, 20Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, 21Hospital for Special Surgery, New York, NY, 22University of Florence, Florence, Italy, 23Emma Children’s Hospital, Amsterdam, Netherlands, 24Hospital Universitario Ramón y Cajal, Madrid, Spain, 25Children’s Hospital at Montefiore, Bronx, NY, 26Sri Ramachandra University, Chennai, India, 27Motol University Hospital, Prague, Czech Republic, 28University Tuebingen, Tübingen, Germany, 29Charles University, Prague, Czech Republic, 30University of Missouri-Columbia, Columbia, MO, 31Rheumatology Department, Hospital Garcia de Orta, Almada, Portugal, 32Hospital de Niños Dr Orlando Alassia, Santa Fe, Argentina, 33Uppsala University, Uppsala, Sweden, 34Faculdade de Medicina, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, 35Medical University Innsbruck, Innsbruck, Austria, 36ASST Gaetano Pini-CTO, Università degli Studi di Milano, Milan, Italy, 37Hospital de Santa Maria, Lisbon, Portugal, 38Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, 39Scheiders Children Medical Center of Israel, Petah-Tiqva, Israel, 40Paediatric Rheumatology International Trials Organisation (PRINTO), Sankt Augustin, Germany, 41Luzerner Kantonsspital, Kinderspital, Luzern, Switzerland, 42Charité University Medicine, Nuremberg, Germany, 43Dept of Pediatric Rheumatology and Immunology Clinical Center Nis, Nis, Serbia, 44Rigshospitalet, Copenhagen, Denmark, 45Stony Brook Children's Hospital, Stony Brook, NY, 46Oslo University Hospital, Oslo, Norway, 47Meir Medical Center, Kfar Saba, Israel, 48Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany

    Background/Purpose: Juvenile systemic sclerosis (jSSc) is a rare disease with a prevalence of around 3 in 1,000,000 children. To better capture the clinical manifestations of…
  • Abstract Number: 0765 • ACR Convergence 2021

    Male Juvenile Systemic Sclerosis Patients Have More Severe Disease: Results from the International Juvenile Scleroderma Inception Cohort

    Ivan Foeldvari1, Jens Klotsche2, Ozgur Kasapcopur3, Amra Adrovic4, Kathryn Torok5, Maria Terreri6, Ana Paula Sakamoto7, Flavio Sztajnbok8, Brian Feldman9, Valda Stanevicha10, Jordi Anton11, Raju Khubchandani12, Ekaterina Alexeeva13, Sindhu Johnson14, Maria Martha Katsicas15, Sujata Sawhney16, Vanessa Smith17, Simone Appenzeller18, Tadej Avcin19, Mikhail Kostik20, Thomas Lehman21, Edoardo Marrani22, Dieneke Schonenberg-Meinema23, Walter Alberto Sifuentes-Giraldo24, Natalia Vasquez-Canizares25, Mahesh Janarthanan26, Hana Malcova27, Monika Moll28, Dana Nemcova29, Anjali Patwardhan30, Maria José Santos31, cristina battagliotti32, Lillemor Berntson33, Blanca Elena Rios Gomes Bica34, Jürgen Brunner35, Rolando Cimaz36, Patricia Costa Reis37, Despina Eleftheriou38, Liora Harel39, Gerd Horneff40, Daniela Kaiser41, Tilmann Kallinich42, Dragana Lazarevic43, Kirsten Minden2, Susan Nielsen44, Farzana Nuruzzaman45, Siri Opsahl Hetlevik46, Yosef Uziel47 and Nicola Helmus48, 1Hamburger Zentrum fuer Kinder- und Jugendrheumatologie, Hamburg, Germany, 2German Rheumatism Research Center, Berlin, Germany, 3Istanbul University-Cerrahpasa, Cerrahpasa Medical School, İstanbul, Turkey, 4Cerrahpasa Medical School, Istanbul, Turkey, 5University of Pittsburgh, Pittsburgh, PA, 6UNIFESP, São Paulo, Brazil, 7Federal University of So Paulo (UNIFESP), São Paulo, Brazil, 8UFRJ/UERJ, Rio de Janeiro, Brazil, 9The Hospital for Sick Children, Toronto, ON, Canada, 10Paediatric Rheumatology International Trials Organisation (PRINTO), Riga, Latvia, 11Hospital Sant Joan de Deu, University of Barcelona, Barcelona, Spain, 12Jaslok Hospital and Research Center, Mumbai, India, 13Scientific Center of Children Health of RAMS, Moscow, Russia, 14University of Toronto, Toronto, ON, Canada, 15Hospital de Pediatria J.P Garrahan, Buenos Aires, Argentina, 16Pediatric Rheumatology Department, Institute of Child Health, Sir Gangarm Hospital, New Delhi, India, 17Department of Rheumatology and Internal Medicine, Ghent University Hospital, Ghent, Belgium, 18Hospital das Clínicas da Universidade Estadual de Campinas, Campinas, Brazil, 19University Medical Center Ljubljana, Ljubljana, Slovenia, 20Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, 21Hospital for Special Surgery, New York, NY, 22University of Florence, Florence, Italy, 23Emma Children’s Hospital, Amsterdam, Netherlands, 24Hospital Universitario Ramón y Cajal, Madrid, Spain, 25Children’s Hospital at Montefiore, Bronx, NY, 26Sri Ramachandra University, Chennai, India, 27Motol University Hospital, Prague, Czech Republic, 28University Tuebingen, Tübingen, Germany, 29Charles University, Prague, Czech Republic, 30University of Missouri-Columbia, Columbia, MO, 31Rheumatology Department, Hospital Garcia de Orta, Almada, Portugal, 32Hospital de Niños Dr Orlando Alassia, Santa Fe, Argentina, 33Uppsala University, Uppsala, Sweden, 34Faculdade de Medicina, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, 35Medical University Innsbruck, Innsbruck, Austria, 36ASST Gaetano Pini-CTO, Università degli Studi di Milano, Milan, Italy, 37Hospital de Santa Maria, Lisbon, Portugal, 38Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, 39Scheiders Children Medical Center of Israel, Petah-Tiqva, Israel, 40Paediatric Rheumatology International Trials Organisation (PRINTO), Sankt Augustin, Germany, 41Luzerner Kantonsspital, Kinderspital, Luzern, Switzerland, 42Charité University Medicine, Nuremberg, Germany, 43Dept of Pediatric Rheumatology and Immunology Clinical Center Nis, Nis, Serbia, 44Rigshospitalet, Copenhagen, Denmark, 45Stony Brook Children's Hospital, Stony Brook, NY, 46Oslo University Hospital, Oslo, Norway, 47Meir Medical Center, Kfar Saba, Israel, 48Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany

    Background/Purpose: Juvenile systemic sclerosis (jSSc) is a rare disease with a prevalence of around 3 in 1,000,000 children. To better capture the clinical manifestations of…
  • Abstract Number: 1171 • ACR Convergence 2020

    Under Detection of Interstitial Lung Disease in Juvenile Systemic Sclerosis (jSSc) Utilizing Pulmonary Function Tests. Results from the Juvenile Scleroderma Inception Cohort

    Ivan Foeldvari1, Bernd Hinrichs2, Kathryn Torok3, Maria Jose Santos4, Ozgur Kasapcopur5, Amra Adrovic6, Valda Stanevicha7, Flavio Sztajnbok8, Maria Teresa Terreri9, Ana Paula Sakamoto10, Ekaterina Alexeeva11, Jordi Anton12, Maria Katsicas13, Vanessa Smith14, Tadej Avcin15, Edoardo Marrani16, Mikhail Kostik17, Thomas Lehman18, Walter Alberto Sifuentes-Giraldo19, Simone Appenzeller20, Mahesh Janarthanan21, Monika Moll22, Dana Nemcova23, Dieneke Schonenberg-Meinema24, Cristina Battagliotti25, Lillemor Berntson26, Blanca Bica27, Jürgen Brunner28, Patricia Costa Reis29, Despina Eleftheriou30, Liora Harel31, Gerd Horneff32, Tilmann Kallinich33, Dragana Lazarevic34, Kirsten Minden35, Susan Nielsen36, Farzana Nuruzzaman37, Anjali Patwardhan38, Yosef Uziel39 and Nicola Helmus40, 1Head of the Hamburg Centre for Pediatric and Adolescence Rheumatology, Budapest, Hungary, 2Kinderklinik Heidberg Pulmologie, Hamburg, Germany, 3University of Pittsburgh, Pittsburgh, PA, 4Hospital Garcia de Orta, Almada, Portugal, 5PRINTO, Istituto Giannina Gaslini, Genova, Italy, 6Cerrahpaşa Tıp Fakültesi, Istanbul, Turkey, 7Riga Stradins University, Riga, Latvia, 8Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil, 9Federal University of São Paulo, São Paulo, Brazil, 10UNIFESP, Sao Paulo, Brazil, 11Scientific Center of Children’s Health of RAMS, Moscow, Russia, 12Sant Joan de Déu Hospital, Madrid, Spain, 13Hospital de Pediatria, Buenos Aires, Argentina, 14Department of Rheumatology, Ghent University Hospital, Department of Internal Medicine, VIB Inflammation Research Centre Ghent University, Ghent, Belgium, 15Ljubljana University Medical Centre, Ljubljana, Slovenia, 16University of Florence, Firenze, Italy, 17Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, 18Hospital for Special Surgery, New York, 19Hospital Universitario Ramon y Cajal, Madrid, Spain, 20Unicamp, Campinas, Brazil, 21Sri Ramachandra University, Chennai, India, 22University of Tübingen, Tübingen, Germany, 23General University Hospital, Prague, Czech Republic, 24Amsterdam UMC, Amsterdam, Netherlands, 25Children's Hospital Dr. Orlando Alassia, Santa Fee, Argentina, 26Uppsala University Hospital, Uppsala, Sweden, 27Hospital Universitário Clementino Fraga Filho (HUCFF/UFRJ), Rio de Janeiro, Brazil, 28Tirol Kliniken, Innsbruck, Innsbruck, Austria, 29Hospital de Santa Maria, Lisbon, Portugal, 30Great Ormond Street Hospital, London, United Kingdom, 31Clalit Health Services, Nettnja, Israel, 32Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany, 33Charite, Berlin, Germany, 34KC Niš, Nis, Serbia, 35Deutsches Rheuma-Forschungszentrum Berlin, Berlin, Germany, 36Rigshospitalet, Copenhagen, Denmark, 37Stony Brook Children's Hospital, Stony Brook, NY, 38University of Missouri, Columbia, 39Meir Medical Center, Kfar Saba, Israel, 40Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany

    Background/Purpose: Juvenile systemic sclerosis (jSSc) has a prevalence in around 3 in a million children. Pulmonary involvement occurs in approximately 40 % in the international…
  • Abstract Number: 795 • 2019 ACR/ARP Annual Meeting

    Is the Presentation and Severity Different of the Juvenile Diffuse and Limited Subtype Systemic Sclerosis? Results of Juvenile Scleroderma Inception Cohort

    Ivan Foeldvari1, Jens Klotsche 2, Ozgur Kasapcopur 3, Amra Adrovic 4, Kathryn Torok 5, Valda Stanevicha 6, Maria Teresa Terreri 7, Flavio Sztajnbok 8, Ekaterina Alexeeva 9, Jordi Antón 10, Brian Feldman 11, Maria Katsicas 12, Vanessa Smith 13, Tadej Avcin 14, Rolando Cimaz 15, Mikhail Kostik 16, Thomas Lehman 17, Walter Sifuentes-Giraldo 18, Natalia Vasquez-Canizares 19, Simone Appenzeller 20, Mahesh Janarthanan 21, Monika Moll 22, Dana Nemcova 23, Maria José Santos 24, Dieneke Schonenberg 25, Christina Battagliotti 26, Lillemor Berntson 27, Blanca Bica 28, Juergen Brunner 29, Patricia Costa Reis 30, Despina Eleftheriou 31, Liora Harel 32, Gerd Horneff 33, Tilmann Kallinich 34, Dragana Lazarevic 35, Kirsten Minden 36, Susan Nielsen 37, Farzana Nuruzzaman 38, Anjali Patwardhan 39, Yosef Uziel 40, Daniela Kaiser 41 and Nicola Helmus 1, 1Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany, 2German Rheumatism Research Center, Berlin, Germany, 3Department of Pediatric Rheumatology, Istanbul University-Cerrahpasa, Istanbul, Turkey, Istanbul, Turkey, 4Cerrahpaşa Tıp Fakültesi, Istanbul, Turkey, 5UPMC Children's Hospital of Pittsburgh, Pittsburgh, 6Riga Stradins University, Riga, Latvia, 7Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil, 8Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil, 9National Medical Research Center of Children`s Health, Moscow, Russia, 10Hospital Sant Joan de Déu, Barcelona, Spain, 11University of Toronto & The Hospital for Sick Children, Toronto, ON, Canada, 12Hospital de Pediatria, Buenos Aires, Argentina, 13Dept. of Rheumatology, Ghent University Hospital, Ghent, Belgium; Dept. of Internal Medicine, Ghent University, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium, Gent, Belgium, 14Ljubljana University Medical Centre, Ljubljana, Slovenia, 15University Hospital Meyer, Florence, Italy, 16Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia, 17Hospital for Special Surgery, New York, 18Hospital Universitario Ramón y Cajal, Madrid, Spain, 19Children’s Hospital at Montefiore, Bronx NY, 20UNICAMP Universidade Estadual de Campinas, Sao Paulo, Brazil, 21Sri Ramachandra University, Chennai, India, 22University of Tuebingen, Tuebingen, Germany, 23General University Hospital, Prague, Czech Republic, 24Rheumatology department, Hospital Garcia de Orta, Almada, Portugal, 25Amsterdam UMC, Amsterdam, Netherlands, 26Children's Hospital Dr. Orlando Alassia, Santa Fee, Argentina, 27Uppsala University Hospital, Uppsala, Sweden, 28Hospital Universitário Clementino Fraga Filho (HUCFF/UFRJ), Rio de Janeiro, Brazil, 29Tirol Kliniken, Innsbruck, Austria, 30Hospital de Santa Maria, Lisbon, Portugal, 31Great Ormond Street Hospital, London, United Kingdom, 32Tel Aviv University, Tel Aviv, Israel, 33Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany, 34Charite, Berlin, Germany, 35KC Niš, Niš, Serbia, 36German Rheumatism Research Center and Charité University Medicine, Berlin, Germany, 37Department of Pediatrics, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark., Copenhavn, Denmark, 38Stony Brook Children's Hospital, Stony Brook, NY, 39University of Missouri, Columbia, 40Meir Medical Center, Kfar Saba, Israel, 41Luzerner Kantonsspital | Kinderspital, Luzern, Switzerland

    Background/Purpose: Juvenile systemic scleroderma (jSSc) is an orphan disease with an estimated prevalence of 1 in 1 000 000 children. In the adult systemic scleroderma…
  • Abstract Number: 796 • 2019 ACR/ARP Annual Meeting

    Under Detection of Interstitial Lung Disease in Juvenile Systemic Sclerosis (jSSc) Utilizing Pulmonary Function Tests: Results from the Juvenile Scleroderma Inception Cohort

    Ivan Foeldvari1, Bernd Hinrichs 2, Kathryn Torok 3, Maria José Santos 4, Ozgur Kasapcopur 5, Amra Adrovic 6, Valda Stanevicha 7, Flavio Sztajnbok 8, Maria Teresa Terreri 9, Ekaterina Alexeeva 10, Jordi Antón 11, Maria Katsicas 12, Vanessa Smith 13, Tadej Avcin 14, Rolando Cimaz 15, Mikhail Kostik 16, Thomas Lehman 17, Walter Sifuentes-Giraldo 18, Simone Appenzeller 19, Mahesh Janarthanan 20, Monika Moll 21, Dana Nemcova 22, Dieneke Schonenberg 23, Christina Battagliotti 24, Lillemor Berntson 25, Blanca Bica 26, Juergen Brunner 27, Patricia Costa Reis 28, Despina Eleftheriou 29, Liora Harel 30, Gerd Horneff 31, Tilmann Kallinich 32, Dragana Lazarevic 33, Kirsten Minden 34, Susan Nielsen 35, Farzana Nuruzzaman 36, Anjali Patwardhan 37, Yosef Uziel 38 and Nicola Helmus 1, 1Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany, 2Asklepios Klinik Nord - Heidberg, Hamburg, Germany, 3UPMC Children's Hospital of Pittsburgh, Pittsburgh, 4Rheumatology department, Hospital Garcia de Orta, Almada, Portugal, 5Department of Pediatric Rheumatology, Istanbul University-Cerrahpasa, Istanbul, Turkey, Istanbul, Turkey, 6Cerrahpaşa Tıp Fakültesi, Istanbul, Turkey, 7Riga Stradins University, Riga, Latvia, 8Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil, 9Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil, 10National Medical Research Center of Children`s Health, Moscow, Russia, 11Hospital Sant Joan de Déu, Barcelona, Spain, 12Hospital de Pediatria, Buenos Aires, Argentina, 13Dept. of Rheumatology, Ghent University Hospital, Ghent, Belgium; Dept. of Internal Medicine, Ghent University, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium, Gent, Belgium, 14Ljubljana University Medical Centre, Ljubljana, Slovenia, 15University Hospital Meyer, Florence, Italy, 16Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia, 17Hospital for Special Surgery, New York, 18Hospital Universitario Ramón y Cajal, Madrid, Spain, 19UNICAMP Universidade Estadual de Campinas, Sao Paulo, Brazil, 20Sri Ramachandra University, Chennai, India, 21University of Tuebingen, Tuebingen, Germany, 22General University Hospital, Prague, Czech Republic, 23Amsterdam UMC, Amsterdam, Netherlands, 24Children's Hospital Dr. Orlando Alassia, Santa Fee, Argentina, 25Uppsala University Hospital, Uppsala, Sweden, 26Hospital Universitário Clementino Fraga Filho (HUCFF/UFRJ), Rio de Janeiro, Brazil, 27Tirol Kliniken, Innsbruck, Austria, 28Hospital de Santa Maria, Lisbon, Portugal, 29Great Ormond Street Hospital, London, United Kingdom, 30Tel Aviv University, Tel Aviv, Israel, 31Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany, 32Charite, Berlin, Germany, 33KC Niš, Niš, Serbia, 34German Rheumatism Research Center and Charité University Medicine, Berlin, Germany, 35Department of Pediatrics, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark., Copenhavn, Denmark, 36Stony Brook Children's Hospital, Stony Brook, NY, 37University of Missouri, Columbia, 38Meir Medical Center, Kfar Saba, Israel

    Background/Purpose: Juvenile systemic sclerosis(jSSc) is an orphan disease with a prevalence in around 3 in a million children[1]. Pulmonary involvement in jSSc occurs in approximately 40…
  • Abstract Number: 1425 • 2018 ACR/ARHP Annual Meeting

    Update from the Juvenile Scleroderma Inception Cohort

    Ivan Foeldvari1, Jens Klotsche2, Ozgur Kasapcopur3, Amra Adrovic4, Kathryn S. Torok5, Valda Stanevicha6, Flavio Sztajnbok7, Maria Teresa Terreri8, Ekaterina Alexeeva9, Jordi Anton10, Maria M. Katsicas11, Vanessa Smith12, Tadey Avcin13, Rolando Cimaz14, Mikhail Kostik15, Thomas J. A. Lehman16, Walter A. Sifuentes-Giraldo17, Simone Appenzeller18, Mahesh Janarthanan19, Dana Nemkova20, Maria José Santos21, Cristina Battagliotti22, Lillemor Berntson23, Blanca Bica24, Juergen Brunner25, Patrícia Costa Reis26, Despina Eleftheriou27, Liora Harel28, Gerd Horneff29, Tilmann Kallinich30, Dragana Lazarevic31, Kirsten Minden32, Susan Mary Nielsen33, Yosef Uziel34 and Nicola Helmus1, 1Hamburg Center for Pediatric and Adolescent Rheumatology, Hamburg, Germany, 2Program Area Epidemiology, German Rheumatism Research Center, Berlin, Germany, 3Istanbul University, Cerrahpasa Medical School, Department of Pediatric Rheumatology, Istanbul, Turkey, 4Department of Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey, 5Pediatric Rheumatology, Scleroderma Center of Pittsburgh, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, 6Pediatric cathedra, Riga Stradiņš University, Riga, Latvia, 7Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, 8Pediatric Rheumatology Unit, Federal University of São Paulo (UNIFESP - Universidade Federal de São Paulo), São Paulo, Brazil, 9National Medical Research Center of Children's Health and Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation, Moscow, Russian Federation, 10Hospital Sant Joan de Deu, Barcelona, Spain, 11Service of Immunology & Rheumatology., Hospital de Pediatría Prof Dr JP Garrahan, Buenos Aires, Argentina, 12Department of Internal Medicine, Department of Rheumatology, Ghent University Hospital, Department of Internal Medicine, Ghent University, Ghent, Belgium, Ghent, Belgium, 13University Children's Hospital, Ljubljana, Slovenia, 14Pediatrics, Ospedale Pediatrico Anna Meyer, Florence, Italy, 15Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russian Federation, 16Pediatric Rheumatology, Hospital for Special Surgery Weill Cornell Medical College, New York, NY, 17Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain, 18Faculty of Medical Science State University of Campinas (UNICAMP), Sao Paulo, Brazil, 19Pediatric Rheumatology, Chennai, India, 20Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, 21Reuma.pt, Almada, Portugal, Almada, Portugal, 22Hospital de Niños Dr Orlando Alasia, Santa Fé, Argentina, 23Dept. of Pediatrics, Uppsala University Hospital, Uppsala, Sweden, 24"Chefe do Serviço de Reumatologia do Hospital Universitário Clementino Fraga Filho (HUCFF)Universidade Federal do Rio de Janeiro ", Rio de Janeiro, Brazil, 25Division of Pediatric Rheumatology, Medical University Innsbruck, Innsbruck, Austria, 26Pediatrics, Hospital De Santa Maria, Lisbon, Portugal, 27Infection, Inflammation and Rheumatology, UCL Institute of Child Health, London, United Kingdom, 28Schneider Children's Medical Center of Israel, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel, 29Asklepios Kinderklinik St. Augustin GmbH, Sankt Augustin, Germany, 30Charité Berlin Campus Virchow, Berlin, Germany, 31Dept of Pediatric Rheumatology and Immunology Clinical Center Nis, Nis, Serbia, 32Children’s University Hospital Charite/German Rheumatism Research Centre Berlin, Berlin, Germany, 33Rigshospitalet, Copenhagen, Denmark, 34Meir Medical Center, Kfar Saba, Israel

    Background/Purpose: Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children(1). There is limited data published regarding…
  • Abstract Number: 1154 • 2012 ACR/ARHP Annual Meeting

    Impact of FokI VDR and TNFalpha–308 Polymorphism On Disease Severity and Long Term Outcome in JIA Patients On Anti-TNF Treatment

    Jelena Vojinovic1, Jelena Basic2, Gordana Susic3, Dragana Lazarevic4 and Nemanja Damjanov5, 1Dept Pediatric Rheumatology, Clinical Center, School of Medicine University of Nis, Nis, Serbia, 2Institute of Biochemistry, School of Medicine University of Nis, Dr, Nis, Serbia, 3Istituto Giannina Gaslini, Genoa, Italy, 4Dept Pediatric Rheumatology, Clinical Center, School of Medicine University of Nis, Dr, Nis, Serbia, 5Institute for Rheumatology, University of Belgrade, Prof, Belgrade, Serbia

    Background/Purpose: Genetic contribution of SNP (single nucleotide polymorphism) of TNFα–308 promoter and FoxI for VDR (vitamin D receptor) polymorphism on disease severity and outcome in…
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