Cyclosporine therapy in primary steroid-dependent and steroid-resistant focal segmental glomerulosclerosis in adults

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.

Cite this article as: Hassan Jafry N, Qureshi S, Mubarak M, Ahmed E. Cyclosporine therapy in steroid-dependent and steroid-resistant focal segmental glomerulosclerosis in adults. Turk J Nephrol. 2023;32(2):120-127.