성인 국소성 분절성 사구체 경화증의 임상경과 및 치료반응 = The Response to Therapy and Outcome in Adult Focal Segmental Glomerulosclerosis

다국어 초록 (Multilingual Abstract)

Background: The response to therapy in adult patients with nephrotic syndrome caused by primary focal segmental glomerulosclerosis (FSGS) was discouraging, but recent reports have shown high remission rate. Of these patients in remission, few progressed to end stage renal disease. Methods : we analyzed author's experiences in 42 Korean adults with primary FSGS between January 1986 and December 1995 in attempt to know the response to therapy and outcome. Results: 1) Twenty-six of 42 patients(62%) had nephrotir range proteinuria, and renal insufficiency (serum creatinine>1.4 mg/dL ) was present in 14 patients(34%). Nephrotic patients had higher serum level of creatinine than that of nonnephrotic patients, and there was no difference between the two groups in blood pressure, age, and hematuria. 2) Twenty-six patients with nephrotic range proteinuria received combined treatment with prednisolone and cyclophosphamide. Complete remission (proteinuria≤300 mg/d) was observed in 10 cases(39%), and partial remission(≤2.50g/d) in 10 cases(42%), with mean remission time of 4.1±2.2 months after initiation of treatment. They had remission within 6 months of therapy except one. Relapse was noticed in 11 cases (52%) during follow-up. 3) None of responder group progressed to ESRD, while two of 5 non-responders progressed to ESBD, but there was no significant difference in slope of inverse creatinine between two groups. 4) The degree of proteinuria, initial serum creatinine, age, and sex did not affect the patient's response to therapy 5) The risk factors related to progression of renal failure were the initial serum creatinine level and degree of proteinuria. Conclusion: Nephrotic adult patients with primary FSGS may benefit from a more prolonged course of therapy with prednisolone and cyclophosphamide.