Pathogenesis, Treatment Advances, and Renal Outcomes in Childhood-Onset Lupus Nephritis

Systemic lupus erythematosus is defined as an autoimmune and inflammatory disease that is distinguished by the involvement of diversified multisystem and a persistent course with unpredictable flares. Immune complex-mediated glomerulonephritis is the main presentation of renal involvement and is commonly regarded as lupus nephritis (LN). Although the long-term renal prognosis has been considerably improved, a significant morbidity and mortality is still experienced by children and adolescents with LN. Furthermore, present literature is insufficient with regard to data related to pediatrics, resulting in clinicians to depend entirely on therapeutic approaches employed for adults. Treatment protocols have been produced by various international groups that recommend an intense duration of immunosuppression to stop the process of acute renal inflammation, with subsequent maintenance therapy and close monitoring for disease improvement and rapid assessment of disease flares. Prevalence of renal remission is suboptimal indicating only 40% to 60% of patients attaining complete remission. The rate of chronic kidney disease (CKD) stage V has been observed to be at least 15% and the presence of LN has an association with high mortality rates. In ascertained renal failure, renal transplantation is implied to be the most favorable modality of renal replacement therapy in this specific patient group. This review aims to describe childhood-onset LN and provide an overview on the pathogenesis, therapeutic approaches, emerging therapies and present renal outcomes for this disease with the objective of recognizing the key features that might aid in reducing the risk of long-term CKD.