The clinical course and long-term outcome of primary focal segmental glomerulosclerosis in Chinese adults

Abstract

To explore the long-term outcome and prognostic factors of primary focal segmental glomerulosclerosis (FSGS) in Chinese adults. A retrospective study was conducted in 104 adults with primary FSGS. Clinical records and renal biopsies were reviewed. The primary endpoint was end-stage renal disease (ESRD) and death. The most frequent FSGS variant was not otherwise specified (45.2%), followed by tip lesion (20.2%). 57 patients presented nephrotic syndrome. Among the nephrotic patients with first episode, the total remission rate was 94.75% by prednisone treatment and 50% by Tripterygium wilfordii (TW) multiglycoside treatment. Steroid-resistant patients treated by TW multiglycoside achieved a total remission rate of 80%. Over a median follow-up of 72 months, 25 patients developed ESRD. The median renal survival was 116 months. The renal survival rates were 83.5% and 43.8% at 5 and 10 years after the biopsy, respectively. By multivariate Cox proportional analysis, the degree of proteinuria, acute kidney injury (AKI) with chronic kidney disease (CKD) Stage 3, chronic tubulointerstitial injury, and complete and partial remission were independent predictors of ESRD. TW is a new potential treatment for steroid-resistant nephrotic FSGS. The 5-year renal outcome in adults with primary FSGS in China is better in comparison to the west. Severe proteinuria, CKD with AKI, chronic tubulointerstitial injury, and no response were independent risk factors of prognosis.