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47. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 33. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), 29. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)

04.09. - 07.09.2019, Dresden

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Update from the juvenile scleroderma Inception Cohort

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  • Ivan Foeldvari - Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg
  • Jens Klotsche - Deutsches Rheuma-Forschungszentrum (DRFZ), Programmbereich Epidemiologie, Berlin
  • Ozgur Kasapcopur - Department of Pediatric Nephrology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul
  • Amra Adrovic - Department of Pediatric Nephrology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey
  • Kathryn Torok - University Childrens Hospital, Pittsburgh, United States of America
  • Valda Stanevicha - University Childrens Hospital, Riga, Latvia
  • Flavio Sztajnbok - Hospital Universitario Pedro Ernesto, Rio de Janeiro, Brasilien
  • M. T. Terreri - Universidade Federal de São Paulo, Pediatric Rheumatology, Sao Paulo, Brazil
  • Ekaterina Alexeeva - Russian Academy of Medical Sciences, Rheumatology Department, Scientific Center for Children’s Health, Moskau, Russland
  • Jordi Anton - University Children’s Hospital, Pediatric Rheumatology, Barcelona, Spain
  • Maria Katsicas - Hospital de Pediatria, Buenos Aires, Argentina
  • Vanessa Smith - Gent University Hospital, Rheumatology, Gent, Belgium
  • Tadey Avcin - University Childrens Hospital, Pediatric Rheumatology, Ljubljana, Slovenia
  • Rolando Cimaz - University of Florence, Florence, Italy
  • Mikhail Kostik - Saint-Petersburg State Pediatric Medical University, St. Petersburg, Russia
  • Thomas Lehman - Hospital for Special Surgery, New York, USA
  • W. Alberto Sifuentes-Giraldo - University Hospital Ramón y Cajal, Madrid, Spain
  • Simone Appenzeller - Faculty of Medical Science, State University of Campinas (UNICAMP), Sao Paulo, Brazil
  • Mahesh Janarthanan - Pediatric Rheumatology, Chennai, India
  • Monika Moll - Universitätsklinikum Tübingen, Klinik für Kinder- und Jugendmedizin, Tübingen
  • Dana Nemcova - University Childrens Hospital, Pediatric Rheumatology, Prague, Czech
  • Maria Jose Santos - Serviço de Reumatologia, Hospital Garcia de Orta, Almada, Portugal
  • Dieneke Schonenberg - Amsterdam UMC, Amsterdam, Netherland
  • Cristina Battagliotti - Hospital den Ninos Dr. Orlando Alassia, Santa Fe, Argentina
  • Lillemor Berntson - Dept. of Pediatrics, Uppsala University Hospital, Uppsala, Sweden
  • Blanca Bica - Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil
  • Jürgen Brunner - Medizinische Universität Innsbruck, Department für Kinder- und Jugendheilkunde, Innsbruck, Österreich
  • Patrícia Costa Reis - Hospital de Santa Maria, Lisbon, Portugal
  • Despina Eleftheriou - Great Ormond Street Childrens Hospital, London, United Kingdom
  • Liora Harel - Pediatric Rheumatology, Nettnja, Israel
  • Gerd Horneff - Asklepios Klinik Sankt Augustin, Sankt Augustin
  • Tilmann Kallinich - Charité - Universitätsmedizin Berlin, Klinik für Pädiatrie mit Schwerpunkt Pneumologie und Immunologie, Sektion Rheumatologie, Berlin
  • Dragana Lazarevic - KC Niš, Nis, Serbia
  • Kirsten Minden - Deutsches Rheuma-Forschungszentrum (DRFZ) und Charité Universitätsmedizin Berlin, Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie, Berlin
  • Susan Nielsen - Juliane Marie Centret, Rigshospitalet, Pediatric Rheumatology, Copenhagen, Denmark
  • Farzana Nuruzzaman - Stony Brook Children’s Hospital, Stony Brook, NY, USA
  • Anjali Patwardhan - University of Missouri, Columbia, USA
  • Yosef Uziel - Pediatric Rheumatology, Sapir Medical Center, Kfar Saba, Israel
  • Nicola Helmus - Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg

Deutsche Gesellschaft für Rheumatologie. Deutsche Gesellschaft für Orthopädische Rheumatologie. Gesellschaft für Kinder- und Jugendrheumatologie. 47. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 33. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), 29. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR). Dresden, 04.-07.09.2019. Düsseldorf: German Medical Science GMS Publishing House; 2019. DocKR.01

doi: 10.3205/19dgrh164, urn:nbn:de:0183-19dgrh1641

Published: October 8, 2019

© 2019 Foeldvari et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. See license information at http://creativecommons.org/licenses/by/4.0/.

Outline

Text

Background: Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. There are limited data regarding the clinical presentation of jSSc. The Juvenile Systemic Scleroderma Inception Cohort (JSSIC) is a multinational registry that prospectively collects information regarding patients with this disease in a standardized manner.

Methods: Patients were included in the JSSIC if they fulfilled the adult classification criteria, if they presented the first non Raynaud symptom before 16 years old and if they were younger than 18 years of age at the time of inclusion. Patients’ characteristics at time of inclusion were evaluated.

Results: Currently, the cohort includes 120 patients, being 89% Caucasian and 80% female. The majority had diffuse subtype (74%) and 18% had overlap features. The mean age of onset of Raynaud phenomenon was 9.7 years in the diffuse subtype (djSSc) and

10.7 years in the limited subtype (ljSSc). The mean age of non-Raynaud’s symptoms was 10.0 years in the djSSc and 11.4 years in the ljSSc (p=0.041). Mean disease duration at time of inclusion was 3.4 years in the djSSc and 2.4 years in the ljSSc group.

Mean Modified Rodnan skin score was 17.5 in the djSSc and 7.3 in the ljSSc (p=0.002). Gottron papulae were significantly more common in the djSSc compared to ljSSc group (29% vs 6%, respectively) (p=0.011). History of ulceration was significantly more common in the djSSc than in the ljSSc group (57% vs 30%, respectively) (p=0.004). FVC<80% occurred in 31% in the djSSc and 24% in the ljSSc group (p=0.55). Pulmonary hypertension assessed by echocardiogram occurred around 7% in both groups. No systemic hypertension or renal crisis was reported. Gastrointestinal involvement occurred in 39% in the djSSc and in 26% in the ljSSc (p=0.176). Number of joints with decreased range of motion was observed in approximately half of patients in both groups. Muscle weakness with joint contractures was present in 18% in the djSSc and 38% in the ljSSc group (p=0.271). Tendon friction rub was present in 11 % in djSSc and 4% in the ljSSc group. djSSc patients had significantly worse scores for physician global disease activity (VAS 0-100) (41vs 30) (p=0.020) and for physician global disease damage (VAS 0-100) (37 vs 18) (p=0.001). Patient judgment of disease activity and damage was similar in both subtypes. ANA positivity was 88% in both groups. Anti-Scl70 was positive in 33% in djSSc and 37% in the ljSSc group. Anticentromere positivity occurred in 3% in the djSSc and 10% in the ljSSc group. ESR was elevated in 30% in djSSc compared to 18% in the ljSSc group. DMARDs were used in 86% of the patients.

Conclusion: In this large cohort of jSSc patients there were surprisingly not many significant differences between djSSc and ljSSc. According to the physician global scores the djSSc patients have a significantly more severe disease.

Supported by the "Joachim Herz Stiftung"