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European evidence-based recommendations for diagnosis and treatment of childhood-onset systemic lupus erythematosus: the SHARE initiative
  1. Noortje Groot1,2,
  2. Nienke de Graeff1,
  3. Tadej Avcin3,
  4. Brigitte Bader-Meunier4,
  5. Paul Brogan5,
  6. Pavla Dolezalova6,
  7. Brian Feldman7,
  8. Isabelle Kone-Paut8,
  9. Pekka Lahdenne9,
  10. Stephen D Marks5,
  11. Liza McCann10,
  12. Seza Ozen11,
  13. Clarissa Pilkington5,
  14. Angelo Ravelli12,
  15. Annet van Royen-Kerkhof1,
  16. Yosef Uziel13,
  17. Bas Vastert1,
  18. Nico Wulffraat1,
  19. Sylvia Kamphuis2,
  20. Michael W Beresford10,14
  1. 1Wilhelmina Children's Hospital, Utrecht, Netherlands
  2. 2Sophia Children's Hospital, Erasmus University Medical Centre, Rotterdam, Netherlands
  3. 3University Children's Hospital Ljubljana, Ljubljana, Slovenia
  4. 4Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
  5. 5Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK
  6. 6General University Hospital, First Faculty of Medicine, Charles University, Prague, Czech Republic
  7. 7The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
  8. 8Bicêtre Hospital, APHP, University of Paris Sud, Paris, France
  9. 9Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland
  10. 10Alder Hey Children's NHS Foundation Trust, Liverpool, UK
  11. 11Department of Pediatrics, Hacettepe University, Ankara, Turkey
  12. 12Università degli Studi di Genova and Istituto Giannina Gaslini, Genoa, Italy
  13. 13Meir Medical Center, Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel
  14. 14Institute of Translational Medicine, University of Liverpool, Liverpool, UK
  1. Correspondence to Dr Noortje Groot, Department of Paediatric Immunology, University Medical Centre Utrecht, Lundlaan 6, Utrecht 3584 EA, The Netherlands; n.groot{at}erasmusmc.nl

Abstract

Childhood-onset systemic lupus erythematosus (cSLE) is a rare, multisystem and potentially life-threatening autoimmune disorder with significant associated morbidity. Evidence-based guidelines are sparse and management is often based on clinical expertise. SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) was launched to optimise and disseminate management regimens for children and young adults with rheumatic diseases like cSLE. Here, we provide evidence-based recommendations for diagnosis and treatment of cSLE. In view of extent and complexity of cSLE and its various manifestations, recommendations for lupus nephritis and antiphospholipid syndrome will be published separately. Recommendations were generated using the EULAR (European League Against Rheumatism) standard operating procedure. An expert committee consisting of paediatric rheumatologists and representation of paediatric nephrology from across Europe discussed evidence-based recommendations during two consensus meetings. Recommendations were accepted if >80% agreement was reached. A total of 25 recommendations regarding key approaches to diagnosis and treatment of cSLE were made. The recommendations include 11 on diagnosis, 9 on disease monitoring and 5 on general treatment. Topics included: appropriate use of SLE classification criteria, disease activity and damage indices; adequate assessment of autoantibody profiles; secondary macrophage activation syndrome; use of hydroxychloroquine and corticosteroid-sparing regimens; and the importance of addressing poor adherence. Ten recommendations were accepted regarding general diagnostic strategies and treatment indications of neuropsychiatric cSLE. The SHARE recommendations for cSLE and neuropsychiatric manifestations of cSLE have been formulated by an evidence-based consensus process to support uniform, high-quality standards of care for children with cSLE.

  • Systemic Lupus Erythematosus
  • Treatment
  • Corticosteroids
  • Disease Activity
  • Autoantibodies

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Footnotes

  • Contributors MWB and SK are senior authors. NW and BV designed the SHARE initiative. NG and NdG performed the systematic literature review, supervised by MWB and SK. Validity assessment of selected papers was done by MWB, SK, TA, AR, IKP, BBM and CP. Recommendations were formulated by NG, MWB and SK. The expert committee consisted of TA, BBM, PB, PD, IKP, PL, LM, SO, CP, AR, AvR, YU, NW, SK, MWB and SDM; 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 coauthors.

  • Competing interests None declared.

  • Patient consent The study did not involve human subjects.

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

  • Correction notice This paper has been amended since it was published Online First. Owing to a scripting error, some of the publisher names in the references were replaced with 'BMJ Publishing Group'. This only affected the full text version, not the PDF. We have since corrected these errors and the correct publishers have been inserted into the references.

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