Approximately one in five patients with systemic lupus erythematosus (SLE) has disease-onset during childhood (cSLE). Lupus nephritis is more common in cSLE than adult-onset SLE and is associated with significant and increased morbidity and mortality. In this article, we review lupus nephritis in cSLE, including pathogenesis, diagnosis, biomarkers, and management through PUBMED search between July and December 2024. Diagnosis of lupus nephritis is made in 93% of cSLE patients during the first 2 years of disease. The majority of patients have active disease in other organs, and nephrotic range proteinuria and hypertension is frequently observed at diagnosis. Class III and IV are observed in over 50% of renal biopsies and progression to end-stage renal disease varies across cohorts. Major progress made in recent years includes adjustment of the proportion of fibrous crescents when scoring nephritis in cSLE to better discriminate kidney disease outcomes, and development of non-invasive biomarkers to identify renal disease activity and damage. It is anticipated that accurate non-invasive biomarkers will foster multicenter studies and help identify new treatment approaches to improve outcomes in cSLE nephritis.
