A Clinical Analysis of Primary Focal and Segmental Glomerulosclerosis in Adults / 대한신장학회잡지

ABSTRACT

PURPOSE: Primary focal and segmental glomerulosclerosis(FSGS) is a clinicopathological entity defined by the segmental sclerosis involving glomeruli in a focal distribution with poor prognosis. Approximately 50% to 70% of adults with FSGS manifests nephrotic syndrome, and the others show mild to moderate proteinuria. In this study, we intended to figure out the clinical course, treatments and possible prognostic factors of FSGS in adults. METHODS: We retrospectively reviewed the clinical characteristics of 54 adult patients diagnosed as primary focal segmental glomerulo-sclerosis(FSGS) by renal biopsy in Chungnam National University Hospital. from Dec. 1989 to Jan. 2000. RESULTS: Mean age of 54 patients was 36.8+/-24.6 (15-75) years. Male to female ratio of them was 1.08 1. Thirty one patients(57%) of them were manifested as nephrotic syndrome and their male to female ratio was 1.4 1. In all of 54 primary FSGS patients, generalized edema(59%), microscopic hematuria (44%), hypertension(39%) and azotemia(15%) were noted as clinical manifestations at the time of diagnosis. Median duration of follow-up was 48.7(6-123) months. Except generalized edema, serum cholesterol and albumin level, and 24 hour urine protein excretion, there was no significant difference in hypertension, hematuria, serum creatinine, duration of follow- up and progression to chronic renal failure between 31 nephrotic and 23 non-nephrotic patients. Sixteen (52%) of 31 nephrotic patients with primary FSGS showed complete remission. Seven(22%) of them showed partial remission and eight(26%) of them showed no response after 8 weeks of first steroid treatment. No response group of primary FSGS nephrotic patients progressed significantly more to chronic renal failure than remission group including complete or partial remission patients. No response groups showed significantly higher serum creatinine, lower creatinine clearance, higher degree of glomerular global sclerosis and interstitial fibrosis than remission group at the time of diagnosis. Eleven patients(8 nephrotic and 3 non-nephrotic patients) of 54 primary FSGS patients progressed to CRF during follow-up period, and they showed significantly lower creatinine clearance and higher degree of global glomerular sclerosis at the time of diagnosis than normal renal function maintaining group. CONCLUSION: It is speculated that initial renal function and degree of global glomerular sclerosis at the time of diagnosis in primary FSGS patients, and additively response to initial steroid therapy and the degree of interstitial fibrosis at the time of diagnosis in nephrotic primary FSGS patients are thought to be significant long-term prognostic factors.