Histopathological Spectrum and Short-Term Outcome of Treatment with Cyclophosphamide in Relapsing Steroid-Sensitive Nephrotic Syndrome.

Abstract

To determine the short-term outcome of cyclophosphamide (CPO) course in children with relapsing steroid sensitive nephrotic syndrome (SSNS) with different histopathological lesions. Descriptive, observational study. Pediatric Nephrology Department, Sindh Institute of Urology and Transplantation, Karachi, from January 2012 to December 2014. All children with relapsing steroid-sensitive nephrotic syndrome, who underwent renal biopsy and received cyclophosphamide therapy, were included and followed up for 2 years. Histopathological features in renal biopsy, duration of treatment, duration of complete remission and complication frequency was noted. Of the total 74 patients, 47 (63.5%) were males and 27 (36.5%) females. Median age with Interquartile range (IQR) at presentation was 5 years (4-7 years). Minimal change disease (MCD) was the most common histopathological diagnosis (n=54, 73%) followed by focal segmental glomerulosclerosis (FSGS) (n=13, 17.5%), mesangioproliferative glomerulonephritis(MesPGN) (n=6, 8.1%), IgA nephropathy (n=1, 1.4%). The median number of glomeruli included in each biopsy sample was 15. The median duration of treatment with CPO was 11 weeks (9 to 13 weeks), whereas the median duration of complete remission post-therapy was 13 months (7-23 months). A median timeframe of 17 months (13-24.2 months) lapsed between establishing the diagnosis of NS and initiating CPO treatment. Leucopenia was noted in six (8.1%) patients. The short-term outcome of relapsing SSNS can be improved with CPO and steroids, with minimum short-term side effects.