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Consensus-based recommendations for the management of juvenile localised scleroderma
  1. Francesco Zulian1,
  2. Roberta Culpo1,
  3. Francesca Sperotto1,
  4. Jordi Anton2,
  5. Tadej Avcin3,
  6. Eileen M Baildam4,
  7. Christina Boros5,
  8. Jeffrey Chaitow6,
  9. Tamàs Constantin7,
  10. Ozgur Kasapcopur8,
  11. Sheila Knupp Feitosa de Oliveira9,
  12. Clarissa A Pilkington10,
  13. Ricardo Russo11,
  14. Natasa Toplak3,
  15. Annet van Royen12,
  16. Claudia Saad Magalhães13,
  17. Sebastiaan J Vastert12,
  18. Nico M Wulffraat12,
  19. Ivan Foeldvari14
  1. 1 Pediatric Rheumatology, University of Padova, Padua, Italy
  2. 2 Pediatric Rheumatology, Hospital and Institut de Recerca Sant Joan de Déu, Barcelona, Spain
  3. 3 Pediatric Rheumatology, University Children's Hospital Ljubljana, Ljubljana, Slovenia
  4. 4 Paediatric Rheumatology, Alder Hey Children’s NHS Foundation Trust, Liverpool, UK
  5. 5 Discipline of Paediatrics, Women's and Children’s Hospital, University of Adelaide, North Adelaide, New South Wales, Australia
  6. 6 Pediatrics, Sydney Children's Hospital Network Randwick and Westmead, Sydney, New South Wales, Australia
  7. 7 Unit of Paediatric Rheumatology, Semmelweis University, Budapest, Hungary
  8. 8 Pediatric Rheumatology, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey
  9. 9 Pediatrics, Instituto de Puericultura e Pediatria Martagao Gesteira, Rio de Janeiro, Brazil
  10. 10 Rheumatology, Great Ormond Street Hospital NHS Trust, London, UK
  11. 11 Servicio de Inmunología/Reumatología, Hospital de Pediatria Juan P Garrahan, Buenos Aires, Argentina
  12. 12 Department of Paediatric Immunology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands
  13. 13 Departamento de Pediatria, Faculdade de Medicina de Botucatu, Hospital das Clínicas, UNESP, Botucatu, Brazil
  14. 14 Klinikum Eilbek, Hamburger Zentrum für Kinder-und Jugendrheumatologie, Hamburg, Germany
  1. Correspondence to Professor Francesco Zulian, Pediatric Rheumatology, University of Padova, Padua 35128, Italy; francescozulian58{at}


In 2012, a European initiative called Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile localised scleroderma (JLS) is a rare disease within the group of paediatric rheumatic diseases (PRD) and can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physicians’ experience. This study aims to provide recommendations for assessment and treatment of JLS. Recommendations were developed by an evidence-informed consensus process using the European League Against Rheumatism standard operating procedures. A committee was formed, mainly from Europe, and consisted of 15 experienced paediatric rheumatologists and two young fellows. Recommendations derived from a validated systematic literature review were evaluated by an online survey and subsequently discussed at two consensus meetings using a nominal group technique. Recommendations were accepted if ≥80% agreement was reached. In total, 1 overarching principle, 10 recommendations on assessment and 6 recommendations on therapy were accepted with ≥80% agreement among experts. Topics covered include assessment of skin and extracutaneous involvement and suggested treatment pathways. The SHARE initiative aims to identify best practices for treatment of patients suffering from PRDs. Within this remit, recommendations for the assessment and treatment of JLS have been formulated by an evidence-informed consensus process to produce a standard of care for patients with JLS throughout Europe.

  • methotrexate
  • systemic sclerosis
  • DMARDs (biologic)

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  • Handling editor Josef S Smolen

  • Contributors FZ and IF are senior authors. NMW and SJV designed the SHARE initiative. RC, FS and NT performed the systematic literature review, supervised by FZ and IF. Validity assessment of selected papers was done by NMW, CB, RR, OK, TC, CSM, EMB, JA, SKFdeO and JC. Recommendations were formulated by FZ, RC and IF. The expert committee consisted of FZ, IF, JA, TC, EMB, CB, JC, TA and RR; they completed the online surveys and/or participated in the subsequent consensus meetings. SJV facilitated the consensus procedure using a nominal group technique. FZ, RC and FS wrote the manuscript, with contribution and approval of all coauthors.

  • Funding This project was supported by a grant from the European Agency for Health and Consumers (EAHC), grant number 20111202.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Ethics approval This study did not involve human participants, therefore the ethical approval was not needed.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data sharing statement Additional unpublished data from the study are available by just sending an email to RC ( or FZ (

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