Therapies for steroid-sensitive nephrotic syndrome

Abstract

Nephrotic syndrome (NS), a common childhood kidney disease, is associated with significant morbidity and mortality due to disease complications. Most patients who respond to corticosteroids show a relapsing course that requires repeated courses of therapy, and frequent relapses or steroid dependence are common. Most children with steroid-sensitive relapses show minimal change disease upon biopsy. Focal segmental glomerulosclerosis is the predominant histology in patients with steroid-resistant NS where renal biopsy is recommended, and a complicated disease course is anticipated. Patients with frequent relapses are at risk of severe infections, thrombosis, and hypovolemia and receive repeated and prolonged courses of prednisolone that often result in corticosteroid toxicity. These challenges have led to the use of numerous corticosteroid-sparing agents or regimens to reduce the risk of relapses as well as cumulative corticosteroid burden. This review discusses therapy-related aspects of steroid-sensitive NS and compares different regimens of corticosteroid and other immunosuppressive medications that are used in managing this condition.