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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

Proposal for a Juvenile Systemic Sclerosis Response Index(JSScRI): Result of the Consensus Meeting in Hamburg. Germany 11th of December 2016

Meeting Abstract

  • Ivan Foeldvari - Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg
  • D. Furst - University of California at Los Angeles, Los Angeles, United States of America
  • Jordi Anton - University Children´s Hospital, Pediatric Rheumatology, Barcelona, Spain
  • Eileen Baildem - Alder Hey Children’s Foundation NHS Trust, Liverpool, UK
  • Michael Blakley - Indianapolis University, Indianapolis, USA
  • Tamas Constantin - Semmelweis University, Faculty of Medicine, Budapest, Hungary
  • Patrícia Costa Reis - Hospital de Santa Maria, Lisbon, Portugal
  • Megan Curran - Northwestern University Feinberg School of Medicine, Chicago, USA
  • Maurizio Cutolo - Universita degli Studi di Genova, Genova, Italy
  • Christopher P. Denton - Royal Free and University College, Centre for Rheumatology, London, United Kingdom
  • Kim Fligelstone - FESCA, London, UK
  • Bernd Hinrichs - Practice Buchholz, AMS, Hamburg
  • Francesca Ingegnoli - University of Milan, Milan, Italy
  • Antonia Kienast - Praxis Dr. Foeldvari Hamburger Zentrum für Kinder- und Jugendrheumatologie, Kinder- und Jugendrheumatologie, Hamburg
  • Dana Nemcova - University Childrens Hospital, Pediatric Rheumatology, Prague, Czech
  • Clare Pain - University Childrens Hospital, Pediatric Rheumatology, Liverpool, United Kingdom
  • Clarissa Pilkington - Great Ormond Street Hospital, London, Vereinigtes Königreich
  • Vanessa Smith - Gent University Hospital, Rheumatology, Gent, Belgium
  • D. Khanna - University of Michigan, Ann Arbor, United States of America

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.42

doi: 10.3205/17dgrh153, urn:nbn:de:0183-17dgrh1533

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



Background: Juvenile Systemic Sclerosis (jSSc) is an orphan disease. There is increaseing interest to test novel therapies in management of fibrotic diseases. Therefore, is very important to develop a Response Index for jSSc (JSScRI) to separate effective therapies from placebo. In 2014 at the First Consensus Meeting in Hamburg, following two rounds of Delphi, a proposal for domains and items for a JSScRI were made. In 2016, the 2nd JSScRI Consensus Meeting was held in Hamburg, Germany.

Methods: Before the 2nD JSScRI Consensus Meeting, the items from the first JSScRI Consensus Meeting (2014) were scored in a Delphi by the participants to the current meeting. Participants included 14 experts in adult and juvenile SSc and a patient partner. During the subsequent face to face NGT meeting, moderated by DEF, the items were scored anonymously by the participants after a nominal group discussion moderated by DEF. The domains and items were scored regarding their importance for 1 year clinical trial. The items were scored 1(not relevant at all) to 9(most relevant). A Priori, it was agreed by the participants that the goal oft he NGT was to exclude items that are 1. Not feasible and 2. Don’t represent an outcome measure but may represent the impact of disease on quality of life and vocational and avocational activity. Items that scored a median score of <4 or where greater than 1/3 of participants scored 1-3 [despite the item have a median score > 3] were excluded.

Results: 71 items in 13 domains were scored. 6 Items were not scored as they were felt not to represent an outcome measure or non-feasible and and 6 items received a median score less then 4.

Table 1 provides an example of musculoskeletal involvement with Median score for different outcome measures. ll).

Conclusion: In a rigorous, NGT consensus meeting, some item reduction for the JSScRI was achieved. These will be tested in a prospective,inception cohort of juvenile SSc patients. clerosis inception cohort (