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43. Kongress der Deutschen Gesellschaft für Rheumatologie, 29. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie, 25. Wissenschaftliche Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie

02.-05. September 2015, Bremen

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Update on the juvenile systemic sclerosis inception cohort WWW.JUVENILE-SCLERODERMA.COM

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  • Ivan Foeldvari - Schön Klinik Hamburg-Eilbek, Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg
  • Maria Katsicas - Hospital de Pediatria, Buenos Aires, Argentine
  • M. T. Terreri - Universidade Federal de São Paulo, Pediatric Rheumatology, Sao Paulo, Brasil
  • Rolando Cimaz - University of Florence, Florence, Italy
  • Mikhail Kostik - Saint-Petersburg State Pediatric Medical University, St. Petersburg, Russia
  • Flavio Sztajnbok - Hospital Universitario Pedro Ernesto, Rio de Janeiro, Brasilien
  • Dana Nemcova - University Childrens Hospital, Pediatric Rheumatology, Prague, Czech
  • Monika Moll - Universitätsklinikum Tübingen, Klinik für Kinder- und Jugendmedizin, Tübingen
  • Maria Jose Santos - Serviço de Reumatologia, Hospital Garcia de Orta, Almada, Portugal
  • Tadey Avcin - University Childrens Hospital, Pediatric Rheumatology, Ljubljana, Slovenia
  • Jürgen Brunner - Medizinische Universität Innsbruck, Department für Kinder- und Jugendheilkunde, Innsbruck, Österreich
  • Susan Nielsen - Juliane Marie Centret, Rigshospitalet, Pediatric Rheumatology, Copenhagen, Danmark
  • Tilmann Kallinich - Charité - Universitätsmedizin Berlin, Klinik für Pädiatrie mit Schwerpunkt Pneumologie und Immunologie, Sektion Rheumatologie, Berlin
  • Kirsten Minden - Deutsches Rheuma-Forschungszentrum (DRFZ) und Charité Universitätsmedizin Berlin, Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie, Berlin
  • Jörg Müller - St. Elisabeth Krankenhaus, Zentrum für Kinder- und Jugendmedizin, Lörrach
  • Mahesh Janarthanan - Pediatric Rheumatology, Chennai, India
  • Yosef Uziel - Pediatric Rheumatology, Sapir Medical Center, Kfar Saba, Israel
  • W. Alberto Sifuentes-Giraldo - University Hospital Ramón y Cajal, Madrid, Spain
  • Despina Eleftheriou - Great Ormond Street Childrens Hospital, London, United Kingdom
  • Kathryn Torok - University Childrens Hospital, Pittsburgh, United States of America
  • Nicola Helmus - Schön Klinik Hamburg-Eilbek, Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg

Deutsche Gesellschaft für Rheumatologie. Deutsche Gesellschaft für Orthopädische Rheumatologie. Gesellschaft für Kinder- und Jugendrheumatologie. 43. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh); 29. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh); 25. wissenschaftliche Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR). Bremen, 02.-05.09.2015. Düsseldorf: German Medical Science GMS Publishing House; 2015. DocKR.05

doi: 10.3205/15dgrh129, urn:nbn:de:0183-15dgrh1296

Published: September 1, 2015

© 2015 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

Introduction: Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Currently just retrospective data exist regarding evolvement of organ involvement. In the previous retrospective studies assessment of the organ involvement was not standardized. Our project is the first one, where data of jSSc patientes were collected prospectively and with a standardized assessment.

Methods: Patients with jSSc were recruited worldwide and were prospectively assessed, using the proposed standardized patient assessment protocol. Data of the juvenile systemic sclerosis inception cohort have been contributed to the DeSScipher project which was funded by a grant of theEuropean Community's Framework Programme 7 under grant agreement N° 305495.”

Results: 44 centers from 24 countries aggreed to participate on the project. The assent and consent forms were translated into the local native languages. Untill now 39 patients have been enrolled with a mean disease duration of 6.1 years. Thirty (77%) of the 39 patients were females. The mean age of the onset of Raynaud´s phenomenon was 9.7 years (2-16 years), the youngest 2 years old. The mean age at the onset of the non-Raynaud presentation of jSSc was 10.3 years (3-16 years). 29 (74%) of the 39 have diffuse subtype. 5 in the diffuse (17%) and 3 in the limited subtype (30%) had an overlap feature.

At the time of the inclusion the mean modified Rodnan Skin Score was 16.5. 26/37 had already capillary changes and 22/37 already history of ulcerations, 9/37 had active ulcerations at the time of the inclusion. 26/39 had cardiopulmonary involvement, 11/39 presented with signs of interstitial lung disease on imaging. Two patients had pulmonary hypertension. Three had renal involvement, but no renal crisis. 16/39 had gastrointestinal involvement and 11 of them esophageal involvement. 30/38 had musculoskeletal involvement. ANA positivity occurred in 30/36 and 12/30 of them were anti-Scl 70 positive. 1/23 had anticentromere positivity.

Conclusion: We present the data on the first 39 patients with jSSc included in our cohort. The current recruitment data confirms that pediatric patients are different from the adult patients, with a higher proportion of diffuse subset patients with 74% and of patients with overlap features.