성인 원발성 초점성 사구체 경화증의 예후인자

Abstract

Background : Primary focal segmental glomerulosclerosis (FSGS) is a cause of nephrotic syndrome in adult. Although primary FSGS has been known to be refractory to treatment, recent studies reveal higher remission rate and better prognosis. And it has been reported that some clinical and histopathologic paramenters are significant to prognosis. But, confirmative prognostic indices remain to be defined. In order to further clarify the prognostic factors for therapeutic response and risk factors for progression to chronic renal failure (CRF), we reviewed the medical records of primary adult FSGS patients. Methods : Forty-adult patients diagnosed as primary FSGS between 1991 to 2002 were enrolled. We retrospectively analyzed the clinical and histopathological parameters of all patinents at the time of renal biopsy. In addition, the therapeutic responses to immunosuppressants and the renal survival were analyzed. Results : At the time of renal biopsy, 26 patients (65%) had proteinuria of the nephrotic range and 14 patients (35%) had proteinuria of the non-nephrotic range. The serum creatinine level was higher in nephrotic-ranged patients than that in non nephroticranged patients (p＜0.05). The other parameters were not significantly different between two groups. Twenty-seven patients were treated with immunosuppressants and 15 patients (55.6%) responded to the treatment. There was no significant difference in clinical or histopathological variables between the responders and the non-responders. High serum creatinine level at diagnosis and responsiveness to treatment appeared to be significant as risk factors for progression to CRF (p＜0.05). The paticnts treated with immunosuppressants had longer survival period, compared with those without treatment. And the responders had significantly longer survival period compared with the non-responders (p＜0.05). Conclusion : The patients with initial impairment of renal function or poor response to therapy may have worse prognosis, and the intense treatment with regular follow-up of renal function should be recommended for these patients.