gms | German Medical Science

45. Kongress der Deutschen Gesellschaft für Rheumatologie, 31. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie, 27. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie

06.09. - 09.09.2017, Stuttgart

Is there a Difference in the Presentation of male and female Patients with diffuse subtype of juvenile systemic Sclerosis? Results from the juvenile Scleroderma Inception Cohort www.juvenile-scleroderma.com

Meeting Abstract

  • Ivan Foeldvari - Schön Klinik Hamburg-Eilbek, 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
  • Valda Stanevicha - University Childrens Hospital, Riga, Latvia
  • M. T. Terreri - Universidade Federal de São Paulo, Pediatric Rheumatology, Sao Paulo, Brasil
  • Ekaterina Alexeeva - Russian Academy of Medical Sciences, Rheumatology Department, Scientific Center for Children’s Health, Moskau, Russland
  • Maria Katsicas - Hospital de Pediatria, Buenos Aires, Argentine
  • Vanessa Smith - Gent University Hospital, Rheumatology, Gent, Belgium
  • 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
  • Jordi Anton - University Children´s Hospital, Pediatric Rheumatology, Barcelona, Spain
  • W. Alberto Sifuentes-Giraldo - University Hospital Ramón y Cajal, Madrid, Spain
  • Flavio Sztajnbok - Hospital Universitario Pedro Ernesto, Rio de Janeiro, Brasilien
  • Tadey Avcin - University Childrens Hospital, Pediatric Rheumatology, Ljubljana, Slovenia
  • Mahesh Janarthanan - Pediatric Rheumatology, Chennai, India
  • Maria Jose Santos - Serviço de Reumatologia, Hospital Garcia de Orta, Almada, Portugal
  • Monika Moll - Universitätsklinikum Tübingen, Klinik für Kinder- und Jugendmedizin, Tübingen
  • Dana Nemcova - University Childrens Hospital, Pediatric Rheumatology, Prague, Czech
  • Cristina Battagliotti - hospital den Ninos Dr. Orlando Alassia, Santa Fe, Argentine
  • Jürgen Brunner - Medizinische Universität Innsbruck, Department für Kinder- und Jugendheilkunde, Innsbruck, Österreich
  • Despina Eleftheriou - Great Ormond Street Childrens Hospital, London, United Kingdom
  • Liora Harel - Pediatric Rheumatology, Nettnja, Israel
  • 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
  • Susan Nielsen - Juliane Marie Centret, Rigshospitalet, Pediatric Rheumatology, Copenhagen, Danmark
  • Kathryn Torok - University Childrens Hospital, Pittsburgh, United States of America
  • Josef Uziel - University Children´s Hospital, Pediatric Rheumatology, Karfa Saba, Israel
  • Ann Stevens - Seattle Children's, Seattle, USA
  • Clarissa Pilkington - Great Ormond Street Hospital, London, Vereinigtes Königreich
  • 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. 45. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 31. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), 27. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR). Stuttgart, 06.-09.09.2017. Düsseldorf: German Medical Science GMS Publishing House; 2017. DocKR.31

doi: 10.3205/17dgrh148, urn:nbn:de:0183-17dgrh1484

Published: September 4, 2017

© 2017 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 sclerosis (jSSc) is an orphan autoimmune disease. Several adult publications looked at the differences between the clinical presentation of male and female patients with Systemic Sclerosis. There is a rarity of data regarding this topic in pediatric jSSc. The juvenile scleroderma inception cohort (http://www.juvenile-scleroderma.com/) is a prospective standardized register for patients with jSSc.

Methods: Patients with jSSc were included worldwide to the juvenile scleroderma inception cohort. We compared the demographics and clinical characteristics of male and female patients.

Results: Up till now 88 patients were enrolled, 62 (70%) with djSSc 11/62 (18%) of the patients were male (M) and 51/62 female (F) (82%). The mean disease duration at the time of inclusion was 3.5 in M and 3.6 in F patients. The mean age of the onset of Raynaud symptoms was 8.0 in M and 9.4 years in the F patients and the non-Raynaud symptoms was 8.2 in M and 10.0 in F patients. At the time of the inclusion the mean modified Rodnan Skin Score was 24.3 in M and 17.3 in F patients. Anti-Scl 70 positivity was found in 4/11 (36.4%) in M and 14/49 (28.6%) in F patients. Anticentromere positivity occurred in 2/11 (18.2%) (p=0.035) in M and 0/23 (0%) in F patients. Capillary changes were present in 8/11(73%) of the M and 30/51 (59%) of F patients, but 36% of M and F had already history of ulcerations. 7/11 (64%) of the M and 21/51 (41%) of the F patients had cardiopulmonary involvement. Only 6 F patients had pulmonary hypertension. 7/11 (64%) of M and 11/51 (22%) of F patients had signs of interstitial lung disease (p=0.005). Renal involvement was 2/11 (18%) in M and 3/51 (6%) in F patients. 37% in both sexes had gastrointestinal involvement. 9/11 (82%) of M and 26/50 (52%) in F patients had musculoskeletal involvement. Patient global disease damage was on a VAS (0-100) 56.9 in M and in 38.4 in F (p=0.014) and patient global disease activity was 58.8 in M and 41.9 in M (p=0.024). Physician global of disease activity on a VAS was 58.9 in M and 36.9 in F (p=0.004) and physician global disease damage was 60.2 in M and 31.2 in F (p=0.001).

Conclusion: We present the data of the first 62 diffuse subtype patients with jSSc included in our cohort. Male Patients presented a significantly more severe disease similar to adult male patients.