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Objective: Collapsing focal segmental glomerulosclerosis, a recently described condition, has an increased risk of ending up in kidney failure with replacement therapy. Data are limited on clinical presentation, treatment responses, and outcomes. This study sought to elucidate the clinicopathological profile, therapeutic responses, and outcome of idiopathic collapsing focal segmental glomerulosclerosis in adults. Methods: The study was conducted at the Department of Nephrology, Sindh Institute of Urology and Transplantation (SIUT), Karachi, Pakistan on patients with biopsy-proven collapsing focal segmental glomerulosclerosis between January 1995 and December 2017. The clinical charts were scrutinized to retrieve relevant data items. Data were entered and analyzed in Statistical Package for the Social Sciences version 20. Results: Among 401 patients with idiopathic focal segmental glomerulosclerosis, 58 (14.4%) belonged to collapsing focal segmental glomerulosclerosis category; 34 (58.6%) were males and 24 (41.4%) females with a mean age of 30.1 ± 12.4 years. The mean value of 24-hour proteinuria was 3.81 ± 1.85 g/day and the median time to kidney biopsy was 2.5 weeks (interquartile range = 1-5). Among 58, 53 (91.4%) were treated with steroids; 2 (3.8%) achieved complete remission and 11 (20.8%) achieved partial remission. Steroid responsive (n = 13) and non-responsive (n = 40) groups showed no significant differences in all characteristics. Calcineurin inhibitors were also used in 17/53 (32%) patients. Kidney biopsy showed the lesions to be mild in most cases. Overall, progressive kidney failure was observed in 37/53 (69.8%) patients; 27 (50.9%) showed variable degrees of kidney failure, while 10 (18.8%) required replacement therapy (kidney failure with replacement therapy). The median time to kidney failure was 22 months (interquartile range = 6-33). The steroid responsive group also developed progressive kidney failure in 8/13 (61.5%) cases. Conclusion: The results show that the response to steroids and calcineurin inhibitors is poor in collapsing focal segmental glomerulosclerosis and does not completely protect from progression to kidney failure. [ABSTRACT FROM AUTHOR]

Objective: There is scant data on treatment responses to cyclosporine and outcomes of idiopathic focal segmental glomerulosclerosis. The aim of this study was to share our experience on the use of cyclosporine in adults with primary focal segmental glomerulosclerosis at a single center in Pakistan. Methods: This retrospective study was carried out in the Nephrology Department, Sindh Institute of Urology and Transplantation (SIUT), Karachi between January 1995 and June 2018. Clinical records of adult patients (≥16 years) with biopsy-proven idiopathic focal segmental glomerulosclerosis were reviewed to determine the relevant data items. Data were analyzed using Statistical Package for the Social Sciences version 22. Results: Over a period of 22.5 years, 102/426 (23.9%) patients with a mean age of 26.3 ± 9.7 years with focal segmental glomerulosclerosis failed to achieve remission with steroids or relapsed during tapering and were treated with cyclosporine, including 33 (32.3%) steroid-dependent and 69 (67.6%) steroid-resistant patients. Among these, 44 (43.1%) patients achieved remission: 25 (24.5%) complete remission and 19 (18.6%) partial remission, while 58 (56.8%) showed no response. Among steroid-dependent focal segmental glomerulosclerosis, remission was excellent; 25 (75.7%) patients achieved remission. At the last follow-up, 32 (31.3%) patients had abnormal kidney function, 29 (42.0%) in steroid-resistant group versus only 3 (9.1%) in steroid-dependent group (P = .001). In the steroid-resistant group, 14 (22.6%) patients developed kidney failure and 7 (11.3%) required kidney failure with replacement therapy, while 4 (6%) died. None in steroid-dependent focal segmental glomerulosclerosis required kidney failure with replacement therapy or expired till the last follow-up. Conclusion: In conclusion, this study shows that cyclosporine is effective as second-line therapy in a sizable number of adults with idiopathic focal segmental glomerulosclerosis, particularly in steroid-dependent cases. [ABSTRACT FROM AUTHOR]