BACKGROUND: There is no information in international literature on the mode of presentation, laboratory and clinical features, treatment and prognosis of primary focal segmental glomerulosclerosis (FSGS) in adults in Pakistan. METHODS: This is a retrospective review of 124 adults (≥17 years) with a diagnosis of primary FSGS who presented to the adult nephrology clinic at Sindh Institute of Urology and Transplantation between January 1995 and June 2006. The clinical records and biopsy reports were reviewed to determine the demographic, clinical, laboratory and pathologic features, treatment responses and outcome of the disease. Renal biopsies were performed and evaluated by light microscopy, immunofluorescence and electron microscopy. Standard therapeutic regimens and response to therapy definitions were used. Outcome data were obtained at last follow-up. RESULTS: Mean age of all patients was 30.9 ± 13.6 years (range: 17-85 years). Of these, 86 (69%) were males and 38 (31%) females, with a male to female ratio of 2.2:1. Of 124 patients, 79 (63.7%) were treated with steroids for a median duration of 5 months. Remission was achieved in 40 (50.6%) patients. Relapse occurred in 14 (17.7%) steroid-treated patients. These achieved sustained remission with retreatment. None of the steroid-treated patients went into end-stage renal disease over a mean follow-up of 2.3 years. CONCLUSIONS: Results from this study show that half of adults with primary FSGS achieve sustained remission with prolonged steroid treatment and consequently exhibit an excellent prognosis for long-term outcome. Moreover, raised serum creatinine at presentation does not in itself adversely affect steroid response in adults with primary FSGS.
BACKGROUND: There is no information in international literature on the mode of presentation, laboratory and clinical features, treatment and prognosis of primary focal segmental glomerulosclerosis (FSGS) in adults in Pakistan. METHODS: This is a retrospective review of 124 adults (≥17 years) with a diagnosis of primary FSGS who presented to the adult nephrology clinic at Sindh Institute of Urology and Transplantation between January 1995 and June 2006. The clinical records and biopsy reports were reviewed to determine the demographic, clinical, laboratory and pathologic features, treatment responses and outcome of the disease. Renal biopsies were performed and evaluated by light microscopy, immunofluorescence and electron microscopy. Standard therapeutic regimens and response to therapy definitions were used. Outcome data were obtained at last follow-up. RESULTS: Mean age of all patients was 30.9 ± 13.6 years (range: 17-85 years). Of these, 86 (69%) were males and 38 (31%) females, with a male to female ratio of 2.2:1. Of 124 patients, 79 (63.7%) were treated with steroids for a median duration of 5 months. Remission was achieved in 40 (50.6%) patients. Relapse occurred in 14 (17.7%) steroid-treated patients. These achieved sustained remission with retreatment. None of the steroid-treated patients went into end-stage renal disease over a mean follow-up of 2.3 years. CONCLUSIONS: Results from this study show that half of adults with primary FSGS achieve sustained remission with prolonged steroid treatment and consequently exhibit an excellent prognosis for long-term outcome. Moreover, raised serum creatinine at presentation does not in itself adversely affect steroid response in adults with primary FSGS.
Authors: Dawn J Caster; Barbara Magalhaes; Natali Pennese; Andrea Zaffalon; Marina Faiella; Kirk N Campbell; Jai Radhakrishnan; Vladmir Tesar; Howard Trachtman Journal: Kidney Med Date: 2022-06-11