dc.description.abstract | Focal segmental glomerulosclerosis(FSGS) is a clinical pathological syndrome clinically manifested by nephrotic range proteinuria and histologically characterized by focal and segmental glomerulosclerotic lesions and loss of podocyte foot processes. Focal segmental glomerulosclerosis accounts for approximately 20% of cases of the nephrotic syndrome in children and 40% of such cases in adults, with an estimated incidence of 7 per 1 million. Unfortunately, so far there are no reliable clinical or histological features at presentation that allow the nephrologist to predict which patients will respond to therapy. The aim of this study was to analyze our experience in adult patients with primary FSGS. Furthermore, an attempt was also made to identify clinical and pathological features at presentation that might help to identify patients who will have progressive disease and who might benefit from more aggressive and/or prolonged treatment.
Material & Methods
It was a cross-sectional study of biopsy proven primary FSGS patients who presented over a period of three years from 2015 to 2018, with a one year of follow-up. Treatment results were interpreted with respect to remission reached after 1-year follow-up of each patient. The information collected included age, gender, blood pressure, response to therapy (ACEI /ARB,Corticosteroids / Immunosuppressants), laboratory data such as urea, creatinine, 24-h urine protein excretion, hematuria, lipid profile, The above data were collected at the time of presentation, at the time of biopsy, after attaining remission, at every three-month intervals and at the last followup. The reduction of proteinuria to <400 mg/day was considered a complete remission. Partial remission was defined as reduction of proteinuria by 50% from baseline.
Results
Of 50 patients with primary FSGS, 15 (30%) had complete remission; also 23 patient(46%) had partial remission and 12 patient(24%) was non-responsive. Chi-Square and T-test analysis showed that the factors predictive of remission were: (1) disease duration (p=0.014); (2) serum creatinine (Cr) at onset (p=0.001); (3) presence of interstitial fibrosis (>25%) at initial biopsy (p=0.001) and; presence of glomerulosclerosis(>25%)(P=0.013) at initial biopsy. the degree of interstitial fibrosis and serum cratinine at onset has been the most significant predictor of disease progression. | en_US |