- Poster presentation
- Open Access
SHARE – workpackage 5: evidence based recommendations for diagnosis and treatment of juvenile dermatomyositis
Pediatric Rheumatology volume 12, Article number: P89 (2014)
Juvenile Dermatomyositis is a rare Pediatric Rheumatic Disease, associated with significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physician’s experience. Consequently, treatment regimens differ throughout Europe. In 2012, a European initiative called SHARE (S ingle H ub and A ccess point for pediatric R heumatology in E urope) was launched to optimize and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases.
One of the aims of SHARE was to provide evidence-based recommendations for diagnosis and treatment of JDM.
Evidence based recommendations were developed using the European League Against Rheumatism (EULAR) standard operating procedure (1). An expert committee was instituted, consisting of pediatric rheumatologists and experts in pediatric exercise physiology and physical therapy. The expert committee defined search terms for the systematic literature review. Two independent experts scored articles for validity and level of evidence. Recommendations derived from the literature were evaluated by an online survey. Those with less than 80% agreement during the online survey were reformulated. Subsequently, all recommendations were discussed at a consensus meeting using nominal group technique . Recommendations were accepted if more than 80% agreement was reached.
The literature search yielded 3280 articles, of which 108 (63 for diagnosis and 44 for treatment) were considered relevant and therefore scored for validity and level of evidence. 77 articles (55 for diagnosis and 22 for treatment) were scored valid and used in the formulation of the recommendations. 21 recommendations for diagnosis and 7 for treatment were suggested in the online survey. During the consensus meeting, recommendations were discussed and re-formulated where applicable. It was agreed that all children with suspected inflammatory myopathies should be referred to a specialist centre, with immediate referral for defined high-risk patients. In addition to this, 20 evidence-based recommendations for diagnosis and 9 for treatment were accepted with more than 80% agreement. Topics covered for diagnosis, included assessment of skin involvement, muscle involvement including muscle biopsy, MRI and muscle testing, lung involvement, auto-antibodies, and biomarkers. Treatment recommendations included initial treatment of newly diagnosed patients and therapy for severe disease.
The SHARE initiative provides recommendations for diagnosis and treatment for JDM and thereby facilitates improvement and uniformity of care throughout Europe.
Disclosure of interest
Rights and permissions
Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made.
The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder.
To view a copy of this licence, visit https://creativecommons.org/licenses/by/4.0/.
The Creative Commons Public Domain Dedication waiver (https://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
About this article
Cite this article
Enders, F.B., Bader-Meunier, B., Baildam, E. et al. SHARE – workpackage 5: evidence based recommendations for diagnosis and treatment of juvenile dermatomyositis. Pediatr Rheumatol 12 (Suppl 1), P89 (2014). https://doi.org/10.1186/1546-0096-12-S1-P89
- Rheumatic Disease
- Online Survey
- Systematic Literature Review