Clinical course and outcome of adult patients with primary focal segmental glomerulosclerosis with kidney function...

Kidney function loss or renal insufficiency indicated by elevated creatinine levels and/or an estimated glomerular filtration rate (eGFR) < 60 mL/minute/1.73 m² at presentation in patients with primary focal segmental glomerulosclerosis (FSGS) is commonly seen as a poor prognostic marker for kidney survival. However, a pre>vious study from our center suggested this may be due to hemodynamic factors.

To observe the clinical and biochemical parameters, treatment response, kidney survival, and overall outcomes of adult patients with primary FSGS presenting with kidney function insufficiency.

This retrospective observational study was conducted at the Department of Nephrology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan, from January 1995 to December 2017. During this period, 401 biopsy-proven primary FSGS patients were identified, of which 98 (24.4%) presented with kidney function loss or renal insufficiency defined as eGFR < 60 mL/minute/1.73 m² at presentation and were studied in detail.

Among the 98 patients with renal function loss on presentation, the mean age was 30.9 years ± 13.6 years with a male-to-female ratio of 2.5:1. The mean serum creatinine level was 2.2 mg/dL ± 1.3 mg/dL and mean eGFR 37.1 mL/minute/1.73 m² ± 12.8 mL/minute/1.73 m². The mean 24-hour urinary protein excretion was 5.9 g/day ± 4.0 g/day, and the mean serum albumin was 2.1 g/dL ± 1.0 g/dL (median: 1.5 g/dL). The mean systolic blood pressure (BP) was 132.7 mmHg ± 19.8 mmHg, and the mean diastolic BP was 87.4 mmHg ± 12.7 mmHg. Steroid treatment was given to 81 (82.6%) of 98 patients for an average duration of 19.9 weeks ± 14.4 weeks, with a mean total steroid dose of 4.4 g ± 1.5 g. Treatment response showed that 20 (24.6%) patients achieved complete remission, 9 (11.1%) achieved partial remission, and 52 (64.1%) did not respond. The baseline eGFR was significantly lower in the non-responsive group (P = 0.006). The distribution of FSGS variants was also significantly different among steroid-responsive and non-responsive groups (P = 0.012).

Renal function loss in FSGS patients at presentation does not necessarily indicate irreversible kidney function loss and a significant number of patients respond to appropriate treatment of the underlying disease.