i047 SLE: science in paediatric and adolescent patients

Abstract

Introduction: Childhood-onset, or juvenile-onset SLE (JSLE) is rare, complex and can lead to significant associated morbidity and mortality. Evidence-based guidelines are sparse and management is mostly based on physician experience. Consequently, treatment regimens differ widely between centres and throughout Europe and beyond. Objectives: To provide an update on UK and European initiatives and progress in optimising the care and understanding of JSLE. Specific focus will be on key challenges impacting on young people with lupus, accumulation of damage as they prepare to transition to adults, and European recommendations for the diagnosis and management of childhood-onset SLE. Methods: Since 2006, the UK JSLE Study Group has fostered a UKwide translational programme of research directed towards future clinical trials in JSLE. This UK-wide multi-disciplinary, multi-centre collaborative group comprises all UK tertiary paediatric rheumatology and nephrology centres. At the heart of this has been developing from inception the UK JSLE Cohort Study & Repository. Recruiting from 21 UK-sites over 500 patients, it forms one of the largest national cohorts of JSLE in the world. It enables exploration of the clinical and immunopathological characteristics of this archetypal systemic autoimmune disease, and fosters a robust translational programme of research directed towards clinical trials in JSLE. It has set a consensus UK-wide standard of care for the assessment, diagnosis, monitoring and follow-up of patients with JSLE. In 2013, a European initiative called SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) was launched to optimise and disseminate guidelines for diagnosis and management for children and young adults with paediatric rheumatic diseases within Europe, including childhood-onset SLE. Results: An overview of the work of the UK JSLE Study Group will be presented, focussing on disease accumulation, medication burden (including corticosteroid usage), and organ damage (both disease- and medication-related) already in adolescence, that becomes a significant burden as they transition to adult care. A summary of the final consensus SHARE recommendations will be presented for SLE. These provide evidence based, internationally agreed standards of minimal care for children and young people with lupus. They provide a critical platform for improving the care and minimising the future impact and damage of this complex disease. Conclusions: Significant progress has been made, nationally and internationally, in providing an evidence-base for improving our understanding, diagnosis and management of SLE in young people. This provides a key opportunity to improve the outcomes of children as they transition to adult care and throughout their adult lives.