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European evidence-based recommendations for diagnosis and treatment of paediatric antiphospholipid syndrome: the SHARE initiative
  1. Noortje Groot1,2,
  2. Nienke de Graeff1,
  3. Tadej Avcin3,
  4. Brigitte Bader-Meunier4,
  5. Pavla Dolezalova5,
  6. Brian Feldman6,
  7. Gili Kenet7,
  8. Isabelle Koné-Paut8,
  9. Pekka Lahdenne9,
  10. Stephen D Marks10,
  11. Liza McCann11,12,
  12. Clarissa A Pilkington10,
  13. Angelo Ravelli13,
  14. Annet van Royen-Kerkhof1,
  15. Yosef Uziel14,
  16. Sebastiaan J Vastert1,
  17. Nico M Wulffraat1,
  18. Seza Ozen15,
  19. Paul Brogan10,
  20. Sylvia Kamphuis2,
  21. Michael W Beresford11,12
  1. 1Wilhelmina Children’s Hospital, University Medical Centre, Utrecht, The Netherlands
  2. 2Sophia Children’s Hospital, Erasmus Medical Centre, Rotterdam, The Netherlands
  3. 3University Children’s Hospital, University Medical Centre, Ljubljana, Slovenia
  4. 4Necker Hospital, Assistance Publique-Hôpitaux de Paris, France
  5. 5General University Hospital, 1st Faculty of Medicine, Charles University, Prague, Czech Republic
  6. 6The Hospital for Sick Children, University of Toronto, Canada
  7. 7The Israel National Hemophilia Centre, Sackler Medical School, Sheba Medical Centre, Tel-Hashomer, Tel Aviv, Israel
  8. 8Bicêtre University Hospital, Paris, France
  9. 9Hospital for Children and Adolescents, University of Helsinki, Finland
  10. 10Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK
  11. 11Alder Hey Children’s Hospital NHS Foundation Trust, Liverpool, UK
  12. 12University of Liverpool, Liverpool, UK
  13. 13Università degli Studi di Genova and Istituto Giannina Gaslini, Genova, Italy
  14. 14Meir Medical Center, Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel
  15. 15Department of Pediatrics, Hacettepe University Medical Faculty, Ankara, Turkey
  1. Correspondence to Dr Noortje Groot, Paediatric Immunology, University Medical Centre Utrecht, Lundlaan 6, Utrecht 3584 EA, Netherlands; n.groot{at}


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

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  • Contributors SK and MWB are senior authors. NMW and SJV designed the SHARE initiative. NG and NdG performed the systematic literature review, supervised by MB and SK. Validity assessment of selected papers was done by MWB, SK, TA, AR, IKP, BBM, CAP. Recommendations were formulated by NG, MWB and SK. The expert committee consisted of TA, BBM, PB, PD, IKP, PL, LM, SO, CAP, AR, AvR, YU, NMW, SK, MWB, SM, GK; they completed the online surveys and/or participated in the subsequent consensus meetings. NG, NdG, SK and MWB prepared the consensus meetings, and NG and NdG chaired the meetings and took minutes. AR and BF facilitated the consensus procedure using nominal group technique. NG, SK and MWB wrote the manuscript, with contribution and approval of all co-authors.

  • Competing interests None declared.

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