Renal tubular and glomerular function was studied in patients under 18 years of age with childhood-onset systemic lupus erythematosus (SLE) in relation to disease activity in two groups: patients with clinical or laboratory evidence of lupus nephritis and those without (lupus non-nephritis). We reviewed 11 patients with lupus non-nephritis and 10 patients with lupus nephritis over a 12-month period. The measured glomerular filtration rates had a tendency to be lower in the lupus nephritis group. Glomerular dysfunction was manifest in the lupus nephritis group with elevated urinary albumin/creatinine ratios (P <0.001). Markers of tubular function were measured and compared with data from 94 controls. The lupus nephritis group had elevated urinary NAG [N-acetyl-beta-D-glucosaminidase (P =0.001)] and RBP [retinol-binding protein (P =0.03)] levels. Tubular dysfunction with elevated urinary NAG levels was present in 2 lupus non-nephritis patients with no evidence of glomerular disease. The cohort of patients in this study was followed and 2 lupus non-nephritis patients with the highest urinary RBP levels developed evidence of glomerular dysfunction and biopsy-proven lupus nephritis. Evidence of tubular dysfunction in lupus non-nephritis patients may help to identify lupus nephritis prior to the onset of albuminuria.