NEPHROLOGY: Edited by Michel BaumRenal manifestations of paediatric systemic lupus erythematosusDowsett, Thomasa; Oni, Louiseb,c Author Information aDepartment of Paediatric Nephrology, Royal Manchester Children's NHS Trust Hospital, Manchester bDepartment of Paediatric Nephrology, Alder Hey Children's NHS Foundation Trust Hospital cDepartment of Women's and Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, UK Correspondence to Louise Oni, Senior Lecturer in Paediatric Nephrology and Honorary Consultant Paediatric Nephrologist, Institute in the Park Building, University of Liverpool, Alder Hey Children's Hospital, Eaton Road, Liverpool L12 2AP, UK. Tel: +44 0151 252 5441; e-mail: [email protected] Current Opinion in Pediatrics: April 2022 - Volume 34 - Issue 2 - p 203-208 doi: 10.1097/MOP.0000000000001101 Buy Metrics Abstract Purpose of review Systemic lupus erythematosus is a lifelong, multisystemic disease. Around a fifth of patients present during childhood. Children are recognized to have a more active disease course with more renal involvement (lupus nephritis) when compared with adults. This review article summarizes the current literature surrounding the management of paediatric lupus nephritis. Recent findings International recommendations agree that active, proliferative forms of lupus nephritis are treated with a period of intense induction therapy aimed at inducing remission followed by maintenance immunosuppressive therapy for at least 3 years. Complete response rates in lupus nephritis remain inadequate, in the region of 40–60%, and disease flares are common. Revised histological classification have been proposed but they are yet to be adopted in clinical practice. Lupus nephritis progresses to chronic kidney disease (CKD) stage 5 (kidney failure) in more than 10% of patients within 10 years however the rates of CKD stages 1–4 remain largely unknown. Current trials are focused on the use of biologic agents as adjuncts to current therapy. Summary There is an urgent need for better outcomes in paediatric lupus nephritis. The use of biomarkers to monitor lupus nephritis and scientific studies to improve our understanding of the pathogenesis offer hope of improved outcomes. Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.