Response to Steroids Is Not Completely Protective Against the Development of Progressive Kidney Disease in Collapsing Focal Segmental Glomerulosclerosis

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.

Cite this article as: Jafry NH, Mubarak M, Khan AS, Akhtar F. Response to steroids is not completely protective against the development of progressive kidney disease in collapsing focal segmental glomerulosclerosis. Turk J Nephrol. 2023;32(4):324-331.