European evidence-based recommendations for diagnosis and treatment of childhood-onset systemic lupus erythematosus: the SHARE initiative

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.

European evidence-based recommendations for the diagnosis and treatment of childhood-onset lupus nephritis: the SHARE initiative

“…Lack of adherence to therapy can be as high as 50%, and has been associated with higher persistent disease activity and poorer renal outcomes 78–81. Measuring medication (trough) levels to unmask non-compliance is advisable 15. RCTs in adult LN have shown that time is needed to reach complete renal response for at least 3–6 months 51.…”

“…SHARE recommendations for diagnosis and treatment of cSLE (excluding LN) have also been published 15. Here, the SHARE recommendations for LN are presented.…”

Outcomes following mycophenolate mofetil versus cyclophosphamide induction treatment for proliferative juvenile-onset lupus nephritis

“…Despite distinct differences in the phenotype and pathogenesis of JSLE 7 , international recommendations for the treatment of JSLE 8 The aim of this present study was to use observational data from the UK JSLE Cohort Study 1 , to compare effectiveness of MMF vs. IVCYC induction treatments in patients with juvenile-onset LN, assessing response to treatment, damage accrual, time to achievement of inactive LN, and time to subsequent LN flare.…”

European consensus-based recommendations for the diagnosis and treatment of rare paediatric vasculitides – the SHARE initiative

“…The European initiative SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) aims to optimize care for children and young adults with Paediatric Rheumatic Diseases (6)(7)(8)(9)(10)(11). For rare forms of childhood systemic vasculitis (PAN, ANCA-associated vasculitides (AAV), and TA), the objective was to design cross-cutting and disease-specific evidence-based recommendations for diagnosis and treatment.…”