Correlation between Prognosis and Response to Treatment in Children with Focal Segmental Glumerulosclerosis

To determine the prognostic value of response to treatment in patients with focal segmental glomerulo- sclerosis. FSGS includes 10-15% of idiopathic Nephrotic syndrome in children. Bulk of evidence supports disease relationship with immune system. Unfortunately, responses to immunosuppressive drugs are not desirable and progression to end-stage renal disease is common. We analyzed 62 out of 99 cases of biopsy proven idiopathic FSGS who were followed for at least 5-years or until renal failure occurred during study. Study design was historical cohort and patients were divided into two groups: exposed (resistant to treatment) and non-exposed (responsive to treatment). Correlation between prognosis and response to treatment was statistically evaluated. P-value (0.05 and relative risk ( 1 was considered significant. In 3 out of 25 steroid responsive patients (12%) and 22 out of 37 steroid resistant patients (59.5%), disease progressed to renal failure. Disease progressed to renal failure in 2 out of 11 cyclophosphamide responsive patients (18.1%), 17 out of 23 cyclophosphamide resistant patients (74.3%), and 8 out of 14 cyclosporine resistant patients (57.1%). 2 patients who responded to cyclosporine had normal renal function at the time of the last follow up. We concluded that favorable response to steroid and cyclophosphamide treatment is a protective factor against disease progression to end stage renal disease and resistance to these drugs imply a poor prognosis. For making any definite conclusion concerning response to cyclosporine treatment and prognosis, similar studies with a larger sample are required.