Characteristics and survival data of patients with primary focal segmental segmental glomerulosclerosis: tsn-goldmulti-center study

Abstract Background and Aims We aimed to investigate the characteristics and survival data of biopsy-proven primary focal segmental glomerulosclerosis (FSGS) in adult patients across Turkey. Method Patients with primary FSGS were included by retrospectively scanning the database of the Turkish Society of Nephrology Glomerular Diseases Study Group (TSN-GOLD). Demographic and laboratory data of the patients at baseline, sixth month, first year, and third year were recorded. Patients with secondary FSGS, missing data were excluded. Results The study included 1668 patients with primary FSGS who met the criteria. 1386 patients were included. The mean age of the patients was 41.16±13.88 years, and 712 patients (51.4%) were male. The total follow-up period from the biopsy date was 37.63±40.45 (IQR:1-249) months. The mean blood pressure of the patients, respectively; 130.43±17.63/81.47±10.85 mmHg, serum creatinine 1.29±1.28 mg/dl, e-GFR: 86.10±42.70 ml/min/1.73 m2, serum albumin: 3.41±0.92 g/dl and proteinuria amount was 4687±4658 g/day. Microscopic hematuria was detected in 40.2% of the patients. The rate of admission with nephrotic syndrome was .45.7%. In light microscopy, the mean glomeruli count was 17.36±10.58, with 3.32±4.08 global sclerosis and 0.08±065 glomeruli had segmental sclerosis. Mesangial proliferation was found in 53.1% of the patients and interstitial inflammation was found in 69.7% of the patients. Interestingly, the most common immunoglobulin staining was IgM (19.3%) in the immunofluorescent microscope. The rate of receiving immunosuppressive therapy was 36%. A positive correlation was found in terms of serum creatinine, albumin, and proteinuria in the 3-year follow-up (p<0.001). In the univariate analysis, the group with e-GFR<60 ml/min/1.73 m2 was older, hypertensive, uremic, anemic, had more interstitial fibrosis/tubular atrophy and less interstitial inflammation and mesangial proliferation (p<0.001). In terms of quantitative proteinuria, Patients with proteinuria >3.5 g/day were more hypertensive, hyperlipidemic, hypoalbuminemic and anemic (p<0.05). Conclusion Our study presented important data on the status of patients with national primary FSGS. Approximately one-third of patients receive immunosuppressive therapy. The most important factors determining the prognosis of primary FSGS are the initial nephrotic proteinuria and the degree of renal function.