Idiopathic collapsing glomerulopathy in children

Abstract

Background: Collapsing glomerulopathy (CG) is a clinically and pathologically distinct variant of focal segmental glomerulosclerosis (FSGS). Pathologically similar lesions have been reported in adults and children with human immuno-deficiency virus (HIV) infection. However, there is a recent interest in the recognition of this variant in the absence of HIV infection.

Aim: To evaluate the clinical presentation and outcome of our pediatric patients with idiopathic CG.

Methods: A sum of six children with idiopathic CG, aged 1-7 years at presentation, were retrospectively identified. Clinical data and renal biopsy were reviewed for all patients. Serum creatinine and estimated GFRs at presentation and last follow-up were compared using the Wilcoxon signed rank test and the risk factors for occurrence of ESRD analyzed using the Cox proportional hazard models.

Results: Steroid-resistant nephrotic syndrome with or without azotemia was the presenting clinical finding in all the cases. The median serum creatinine values at onset and last follow-up were 1.05 and 1.25 mg/dl, respectively (p = 0.128). Following immunosuppressive therapy one patient achieved complete remission of proteinuria, and four were in partial remission. The remaining one patient did not show any change in proteinuria at 6 months of therapy. Two of the six patients progressed to end-stage renal disease within a median follow-up period of 27 months (range 14-96 months).

Conclusions: Collapsing glomerulopathy is an aggressive variant of focal segmental glomerulosclerosis. All patients with CG should be screened for the underlying etiology, and patients with idiopathic CG should be offered a trial of immunosuppressive therapy.