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Recommendations
Extended report
Consensus-based recommendations for the management of juvenile dermatomyositis
  1. Felicitas Bellutti Enders1,2,
  2. Brigitte Bader-Meunier3,
  3. Eileen Baildam4,
  4. Tamas Constantin5,
  5. Pavla Dolezalova6,
  6. Brian M Feldman7,
  7. Pekka Lahdenne8,
  8. Bo Magnusson9,
  9. Kiran Nistala10,
  10. Seza Ozen11,
  11. Clarissa Pilkington10,
  12. Angelo Ravelli12,
  13. Ricardo Russo13,
  14. Yosef Uziel14,
  15. Marco van Brussel15,
  16. Janjaap van der Net15,
  17. Sebastiaan Vastert1,
  18. Lucy R Wedderburn10,
  19. Nicolaas Wulffraat1,
  20. Liza J McCann4,
  21. Annet van Royen-Kerkhof1
  1. 1Department of Pediatric Immunology, Wilhelmina Children's Hospital, University Medical Centre Utrecht, Utrecht, The Netherlands
  2. 2Division of Allergology, Immunology and Rheumatology, Department of Pediatrics, University Hospital, Lausanne, Switzerland
  3. 3Department for Immunology, Hematology and Pediatric Rheumatology, Necker Hospital, APHP, Institut IMAGINE, Paris, France
  4. 4Alder Hey Children's NHS Foundation Trust, Liverpool, UK
  5. 52nd Department of Pediatrics, Semmelweis Hospital, Budapest, Hungary
  6. 6Paediatric Rheumatology Unit, Department of Paediatrics and Adolescent Medicine, General University Hospital and 1st Faculty of Medicine, Charles University in Prague, Praha, Czech Republic
  7. 7Division of Rheumatology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
  8. 8Department of Pediatric Rheumatology, Children's Hospital, Helsinki University Central Hospital, Helsinki, Finland
  9. 9Paediatric Rheumatology Unit, Astrid Lindgren Children's Hospital, Karolinska University Hospital Stockholm, Sweden
  10. 10Centre for Adolescent Rheumatology, Institute of Child Health University College London, London, UK
  11. 11Department of Paediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey
  12. 12Università degli Studi di Genova and Istituto Giannina Gaslini, Genoa, Italy
  13. 13Service of Immunology and Rheumatology, Hospital de Pediatría Garrahan, Buenos Aires, Argentina
  14. 14Department of Paediatrics, Meir Medical Center, Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel
  15. 15Division of Pediatrics, Child Development and Exercise Center, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands
  1. Correspondence to Dr Annet van Royen-Kerkhof, Department of Paediatric Immunology, and Rheumatology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Lundlaan 6, 3584 EA Utrecht, Netherlands; a.vanroyen{at}umcutrecht.nl

Abstract

Background In 2012, a European initiative called Single Hub and Access point for pediatric 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 dermatomyositis (JDM) is a rare disease within the group of paediatric rheumatic diseases (PRDs) and can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physicians' experience. Consequently, treatment regimens differ throughout Europe.

Objectives To provide recommendations for diagnosis and treatment of JDM.

Methods Recommendations were developed by an evidence-informed consensus process using the European League Against Rheumatism standard operating procedures. A committee was constituted, consisting of 19 experienced paediatric rheumatologists and 2 experts in paediatric exercise physiology and physical therapy, mainly from Europe. Recommendations derived from a validated systematic literature review were evaluated by an online survey and subsequently discussed at two consensus meetings using nominal group technique. Recommendations were accepted if >80% agreement was reached.

Results In total, 7 overarching principles, 33 recommendations on diagnosis and 19 recommendations on therapy were accepted with >80% agreement among experts. Topics covered include assessment of skin, muscle and major organ involvement and suggested treatment pathways.

Conclusions The SHARE initiative aims to identify best practices for treatment of patients suffering from PRD. Within this remit, recommendations for the diagnosis and treatment of JDM have been formulated by an evidence-informed consensus process to produce a standard of care for patients with JDM throughout Europe.

  • Autoimmune Diseases
  • Dermatomyositis
  • Treatment

This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

https://doi.org/10.1136/annrheumdis-2016-209247

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    Footnotes

    • Handling editor Tore K Kvien

    • Twitter Follow Ricardo Russo at @el_reumatologo

    • Contributors LJM and AvR-K are senior authors. NW and SV designed the SHARE initiative. FBE performed the systematic literature review, supervised by LMC and ARK. Validity assessment of selected papers was done by FBE, BB-M, BMF, CP, AR, MvB, JvdN, SV, LRW, NW, LJMC and AvR-K. Recommendations were formulated by FBE, LJMC and AvR-K. The expert committee consisted of FBE, BB-M, EB, TC, PD, BMF, PL, BM, KN, CP, SO, AvR-K, RR, YU, JvdN, NW, LRW, MvB, SV, LJMC and AvR-K; they completed the online surveys and/or participated in the subsequent consensus meetings. AR assisted in the preparation of the two live consensus meetings with FBE, LJMC, AvR-K and facilitated the consensus procedure using nominal group technique. FBE, AvR-K and LJM wrote the manuscript, with contribution and approval of all co-authors.

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

    • Competing interests FBE—Valeria e Ettore Bossi Foundation. EB—speaker bureau for Roche/ Chugai, Ad Board for Abbvie and Pfizer. BMF—consultant for Novartis, Pfizer, BMS. PL—consultant for BMS, Pfizer. SO—consultant for Novartis, speaker bureau of SOBI. PD—consultant for Roche, speaker bureau for Pfizer, Novartis, grant support from SOBI, Novartis, Abbvie, Roche, Pfizer, Medac. AR—grant/research support from Pfizer and The Myositis Association; consultant for Novartis, Roche; speaker bureau of Abbvie, Novartis, Pfizer, Roche. YU—consultant for Novartis, speaker bureau of Abbvie, Neopharm, Novartis, Roche. NW—grant/research support from EAHC, Abbvie, GSK, Roche, consultant for Genzyme, Novartis, Pfizer, Roche

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