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.

Alder Hey Children's NHS Foundation Trust Liverpool UK

Bicêtre Hospital APHP University of Paris Sud Paris France

Department of Pediatrics Hacettepe University Ankara Turkey

General University Hospital 1st Faculty of Medicine Charles University Prague Czech Republic

Great Ormond Street Hospital for Children NHS Foundation Trust London UK

Hospital for Children and Adolescents University of Helsinki Helsinki Finland

Institute of Translational Medicine University of Liverpool Liverpool UK

Meir Medical Center Sackler School of Medicine Tel Aviv University Tel Aviv Israel

Necker Hospital Assistance Publique Hôpitaux de Paris Paris France

Sophia Children's Hospital Erasmus University Medical Centre Rotterdam Netherlands

The Hospital for Sick Children University of Toronto Toronto Ontario Canada

Università degli Studi di Genova and Istituto Giannina Gaslini Genoa Italy

University Children's Hospital Ljubljana Ljubljana Slovenia

Wilhelmina Children's Hospital Utrecht Netherlands

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