How Have the Clinical, Laboratory, Treatment Features and Outcomes in Children With Lupus Nephritis Progressed Over the Last 30 Years

Abstract

Objectives Management of systemic lupus erythematosus (SLE) through new treatment options has improved lupus nephritis (LN) prognosis. The aim of this study was to compare the changes in the demographic, laboratory, and treatment characteristics, prognosis, and outcomes of paediatric-onset LN patients over 30 years. Methods We retrospectively reviewed the medical records of 103 paediatric-onset LN patients. Patients were divided into two subgroups according to the years of LN diagnosis. Group 1 consisted of patients diagnosed with LN between the years of 1993 and 2005, and group 2 consisted of patients diagnosed with LN between the years of 2006 and 2023. Results The mean age at diagnosis of SLE, age at diagnosis of LN, time to LN development, and mean delay time to diagnosis were significantly higher in group 1 (P < 0.001, P < 0.001, P = 0.049, and P = 0.004, respectively). Baseline Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores were higher and anti-phospholipid antibody positivity was more frequent in group 1 (P = 0.040 and P = 0.025, respectively). Azathioprine in the maintenance phase was given more frequently in group 1 (P = 0.016), while rituximab was more frequently used in group 2 (P = 0.042). In both groups, the majority of the patients had proliferative LN (class III and/or class IV) (53.5% in group 1 vs. 68% in group 2). Complete renal remission was significantly more common in group 2 (P = 0.005), while end-stage kidney disease (ESKD) and death were significantly more common in group 1 (P = 0.005 and P = 0.001, respectively). Proteinuria and SLEDAI scores at the first visit were independent risk factors for progression to ESKD (P = 0.037 and P = 0.024). Conclusion Over the years, there have been significant improvements in the diagnosis and management of children with SLE resulting in an earlier diagnosis, lower disease activity at onset, and improved outcomes.