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Ann Rheum Dis. 2017 Oct;76(10):1637-1641. doi: 10.1136/annrheumdis-2016-211001. Epub 2017 May 4.

European evidence-based recommendations for diagnosis and treatment of paediatric antiphospholipid syndrome: the SHARE initiative.

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Wilhelmina Children's Hospital, University Medical Centre, Utrecht, The Netherlands.
Sophia Children's Hospital, Erasmus Medical Centre, Rotterdam, The Netherlands.
University Children's Hospital, University Medical Centre, Ljubljana, Slovenia.
Necker Hospital, Assistance Publique-Hôpitaux de Paris, France.
General University Hospital, 1st Faculty of Medicine, Charles University, Prague, Czech Republic.
The Hospital for Sick Children, University of Toronto, Canada.
The Israel National Hemophilia Centre, Sackler Medical School, Sheba Medical Centre, Tel-Hashomer, Tel Aviv, Israel.
Bicêtre University Hospital, Paris, France.
Hospital for Children and Adolescents, University of Helsinki, Finland.
Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Alder Hey Children's Hospital NHS Foundation Trust, Liverpool, UK.
University of Liverpool, Liverpool, UK.
Università degli Studi di Genova and Istituto Giannina Gaslini, Genova, Italy.
Meir Medical Center, Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
Department of Pediatrics, Hacettepe University Medical Faculty, Ankara, Turkey.


Antiphospholipid syndrome (APS) is rare in children, and evidence-based guidelines are sparse. Consequently, management is mostly based on observational studies and physician's experience, and treatment regimens differ widely. The Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) initiative was launched to develop diagnostic and management regimens for children and young adults with rheumatic diseases. Here, we developed evidence-based recommendations for diagnosis and treatment of paediatric APS. Evidence-based recommendations were developed using the European League Against Rheumatism standard operating procedure. Following a detailed systematic review of the literature, a committee of paediatric rheumatologists and representation of paediatric haematology with expertise in paediatric APS developed recommendations. The literature review yielded 1473 articles, of which 15 were valid and relevant. In total, four recommendations for diagnosis and eight for treatment of paediatric APS (including paediatric Catastrophic Antiphospholipid Syndrome) were accepted. Additionally, two recommendations for children born to mothers with APS were accepted. It was agreed that new classification criteria for paediatric APS are necessary, and APS in association with childhood-onset systemic lupus erythematosus should be identified by performing antiphospholipid antibody screening. Treatment recommendations included prevention of thrombotic events, and treatment recommendations for venous and/or arterial thrombotic events. Notably, due to the paucity of studies on paediatric APS, level of evidence and strength of the recommendations is relatively low. The SHARE initiative provides international, evidence-based recommendations for diagnosis and treatment for paediatric APS, facilitating improvement and uniformity of care.


Antiphospholipid Antibodies; Antiphospholipid Syndrome; Autoantibodies; Systemic Lupus Erythematosus; Treatment

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