Introduction:
Idiopathic nephrotic syndrome (INS) is the most common glomerular disease in children. We performed this study to report the histopathological findings, correlation between the clinical and histopathological features, with the response to steroids and other immunosuppressive drugs and outcome in Syrian children with INS.
Methods:
A single-center retrospective observational cohort study was conducted at Children’s University Hospital in Damascus, included all patients aged 1 – 14 years, admitted from January 2013 to December 2022 with INS and underwent kidney biopsy.
Results:
The study included 109 patients, with a male/female ratio of 1.13:1, and a median age of 5 years with interquartile range (2.8 – 10). The main indication of kidney biopsy was steroid-resistant nephrotic syndrome (SRNS) (57.8%). The main histopathological patterns were minimal change disease (MCD) (45%) and focal segmental glomerulosclerosis (FSGS) (37.6%). FSGS was the most common histopathological pattern in SRNS (44.3%). In SRNS, we used calcineurin inhibitors to induce remission, Tacrolimus was used in 49 patients with response rate (complete remission of proteinuria) of 69.4% and Cyclosporine in 20 patients with response rate of 50%. In steroid-dependent nephrotic syndrome (SDNS), we used MMF and Cyclophosphamide to prevent relapses, MMF was used in 9 patients with response rate (maintaining sustained remission) of 89% and Cyclophosphamide in 3 patients with response rate of 66.7%. Rituximab was used in four patients with FSGS, two SRNS patients and two SDNS patients, with sustained remission rate of 100%. Fifteen patients (13.7%) progressed to chronic kidney disease stage V, of them 7 patients had FSGS and 8 patients had focal and global glomerulosclerosis, 14 patients of them were steroid resistant and one patient was steroid dependent with persistent relapses. The most common outcome was sustained remission (47%) in MCD and frequent relapses (31.7%) in FSGS.
Conclusions:
FSGS was the most common histopathological pattern in idiopathic SRNS and has the worst prognosis. Calcineurin inhibitors could be an effective therapy to induce complete remission in SRNS. Rituximab may be an effective treatment to achieve sustained remission in SDNS and frequently relapsing NS and may have a potential role in SRNS with further studies required.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.