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SHARE – workpackage 5: evidence based recommendations for diagnosis and treatment of the antiphospholipid syndrome

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Pediatric Rheumatology201412 (Suppl 1) :P111

  • Published:


  • Rheumatic Disease
  • Online Survey
  • Systematic Literature Review
  • Expert Committee
  • Consensus Meeting


Antiphospholipid syndrome (APS), either primary or secondary to other paediatric rheumatic diseases, is rare in children, but it can lead 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 APS.


To provide evidence based recommendations for diagnosis and treatment of APS.


Evidence based recommendations were developed using the European League Against Rheumatism (EULAR) standard operating procedure. An expert committee was instituted, consisting of paediatric rheumatologists from across Europe with expertise in APS. The expert committee defined search terms for the systematic literature review, which was performed in summer 2013. 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 the nominal group technique[1]. Recommendations were accepted if more than 80% agreement was reached.


The literature search yielded 1463 articles, of which 15 ( all relating to diagnosis only, none were relevant for treatment) were considered relevant and therefore scored for validity and level of evidence. Only 8 articles were deemed valid and were used in the formulation of the recommendations. In view of paucity of paediatric-specific data, the majority of proposed recommendations were developed based on adult-derived literature or expert opinion. Four recommendations for diagnosis and 2 for treatment were suggested in the online survey. During the consensus meeting, recommendations based on expert opinion were added. Three recommendations for diagnosis and 6 for treatment were accepted with more than 80% agreement after the consensus meeting. Table 1 summarizes the categories of recommendations.

Table 1

Recommendations regarding:






Laboratory features






For venous thrombotic events


For arterial thrombotic events



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

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.