Treatment of Primary Focal and Segmental Glomerulosclerosis

Document Type : Original Article

Authors

1 Chronic Renal Failure Research Center, Department of Pediatric Nephrology, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran

2 Chronic Renal Failure Research Center, Department of Nephrology, Faculty of Medicine, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.

Abstract

Focal segmental glomerulosclerosis (FSGS) is a common cause of nephrotic syndrome in adults and children. It may occur either without an identifiable cause (idiopathic or primary) or in response to previous glomerular injury (secondary). In the treatment of FSGS distinguishing between primary and secondary is important because immunosuppressive therapy is indicated in most patients with primary FSGS but not in secondary FSGS. Untreated patients with primary FSGS who present with nephrotic range proteinuria (proteinuria>3.5 g/day) and hypoalbuminemia typically have a progressive course to end stage renal disease. It appears that primary FSGS is responded to prednisone at a dose of 1 mg/kg per day (maximum dose 60 to 80 mg/day) in 40 to 80 % of patients, however prolonged glucocorticoid therapy for a minimum of 12 to 16 weeks is generally required to induce remission among these patients. For the treatment of steroid-resistant or relapsing disease, cyclosporine is most commonly used at a dose of approximately 2 to 4 mg/kg per day (given in two divided doses) in conjunction with low dose of prednisone (maximum 15 mg/day). In addition, other agents, including cyclophosphamide and mycophenolatemofetile have also been tried among steroid-resistant or relapsing disease with various degrees of response.

Keywords


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