TY - JOUR T1 - European evidence-based recommendations for diagnosis and treatment of childhood-onset systemic lupus erythematosus: the SHARE initiative JF - Annals of the Rheumatic Diseases JO - Ann Rheum Dis SP - 1788 LP - 1796 DO - 10.1136/annrheumdis-2016-210960 VL - 76 IS - 11 AU - Noortje Groot AU - Nienke de Graeff AU - Tadej Avcin AU - Brigitte Bader-Meunier AU - Paul Brogan AU - Pavla Dolezalova AU - Brian Feldman AU - Isabelle Kone-Paut AU - Pekka Lahdenne AU - Stephen D Marks AU - Liza McCann AU - Seza Ozen AU - Clarissa Pilkington AU - Angelo Ravelli AU - Annet van Royen-Kerkhof AU - Yosef Uziel AU - Bas Vastert AU - Nico Wulffraat AU - Sylvia Kamphuis AU - Michael W Beresford Y1 - 2017/11/01 UR - http://ard.bmj.com/content/76/11/1788.abstract N2 - 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. ER -