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SHARE – workpackage 5 : evidence based recommendations for diagnosis and treatment of childhood-onset systemic lupus erythematosus

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Pediatric Rheumatology201412 (Suppl 1) :P109

  • Published:


  • Systemic Lupus Erythematosus
  • Access Point
  • Rheumatic Disease
  • Online Survey
  • Standard Operating Procedure


Childhood-onset systemic lupus erythematosus (cSLE) is a rare multisystem autoimmune disease that often leads to 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 (Single Hub and Access point for paediatric Rheumatology in Europe) was launched to optimize and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases such as cSLE.


To develop evidence-based recommendations for diagnosis and treatment of cSLE.


Evidence based recommendations were developed using the European League Against Rheumatism (EULAR) standard operating procedure. An expert committee, consisting of paediatric rheumatologists from across Europe with expertise in cSLE, defined search terms for the systematic literature review. Two independent experts scored articles for validity and level of evidence. Through an online survey, experts evaluated recommendations derived from the literature. The recommendations were discussed at a consensus meeting using the nominal group technique1. Recommendations were accepted if more than 80% agreement was reached.


The literature search yielded 9341 articles, of which 128 (75 for diagnosis; 53 for treatment) were considered relevant and therefore scored for validity and level of evidence. Only sixteen articles (4 for diagnosis; 12 for treatment) were deemed valid; a larger number of articles were scored moderately valid (62 for diagnosis, 25 for treatment). Both were used in the formulation of the recommendations. Eighteen recommendations for diagnosis and 24 for treatment were suggested in the online survey. Thirteen recommendations for diagnosis and 14 for treatment were accepted with more than 80% agreement during the consensus meeting. Table 1 summarizes the categories of recommendations.

Table 1

Recommendations regarding:






Laboratory features


Access to other specialities


Comorbidities (growth/MAS/ heri-ditary conditions)




Acknowledgement of necessary research








Haematological involvement


Lupus Nephritis (Class III/IV/V)







The SHARE initiative provides recommendations for diagnosis and treatment for cSLE and thereby facilitates improvement and uniformity of care throughout Europe. Currently, a similar process is going on to add further guidelines including those on treatment and holistic care for PRD patients. As a final result, SHARE will provide standards of minimal care for different PRDs, including cSLE.

Disclosure of interest

None declared.

Authors’ Affiliations

Alder Hey Children's Hospital, Liverpool, UK
Erasmus MC-Sophia, Rotterdam, Netherlands
University Medical Centre Utrecht, Utrecht, Netherlands
University Medical Centre, Ljubljana, Slovenia
Necker Hospital, Paris, France
Great Ormond Street Hospital, London, UK
General University Hospital, Prague, Czech Republic
Sick Kids Hospital, Toronto, Canada
CHU de Bigetre, Paris, France
Children's Hospital of Helsinki and Uusimaa, Helsinki, Finland
Gaslini Children's Hospital, Genova, Italy
Hacettepe University Children's Hospital, Ankara, Turkey


© Groot et al; licensee BioMed Central Ltd. 2014

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver ( applies to the data made available in this article, unless otherwise stated.