43 Egyptian evidence-based consensus recommendations for diagnosis and targeted management of childhood-onset systemic lupus erythematosus. An initiative by the Egyptian College of Pediatric Rheumatology

Abstract

Abstract Background Childhood-onset systemic lupus erythematosus (cSLE) is a rare prototype of a multi-systemic, inflammatory, heterogeneous and potentially life-threatening autoimmune condition with quite significant linked morbidity. To date, there are no Egyptian nationally agreed, evidence-based guidelines concerning the diagnosis and management of kDa in children and treatment is often based on clinical expertise. Consequently, treatment regimens vary broadly. This work was an initiative by the Egyptian College of Paediatric Rheumatology aiming at optimising the management approaches for children and young adults with cSLE. Objective To provide evidence-based consensus recommendations for diagnosis and management of cSLE Methods This study was carried out to achieve an Egyptian expert consensus on a management strategy for cSLE using Delphi technique. A multistep process strategy was adopted, which started by developing 18 key clinical questions by scientific committee according to the Patient/Population, Intervention, Comparison, Outcomes and Time (PICOT) approach. The core leadership team identified clinicians and researchers with expertise in pediatric rheumatology all over Egypt. An evidence-based, systematic, literature review was conducted to compile evidence for the cSLE management. Delphi process was implemented (3-rounds) to reach a consensus. Results Twenty-five expert panel participated in the 3 rounds with response rate 100%. A total of 24 recommendations, categorized into 14 domains (Targeted population, criteria for classification, definition of disease activity status, definition of flare up, lab tests, treatment targets, monitoring, treatment, recommendations for specific organ system, prognostic markers, refractory condition, vaccination and transition program. The Agreement with the recommendations (rank 7–9) ranged from 84.8–94.8%. The Consensus was reached (i.e. ≥75% of respondents strongly agreed or agreed) on all the clinical standards. Algorithm for management has also been developed. Conclusion This work provided an updated management approach for cSLE patients. This evidence-based informed consensus process is expected to support uniform, high quality standards of care for children with cSLE in Egypt The implication to policy, practice, research and advocacy: to provide updated recommendations for better management of cSLE.