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.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Alexopoulos E, Stangou M, Papagianni A, Pantzaki A, Papadimitriou M. Factors influencing the course and the response to treatment in primary focal segmental glomerulosclerosis. Nephrol Dial Transplant. 2000;15:1348–56.
D'Agati VD, Fogo AB, Bruijn JA, Jennette JC. Pathologic classification of focal segmental glomerulosclerosis: a working proposal. Am J Kidney Dis. 2004;43(2):368–82.
Laurinavicius A, Hurwitz S, Rennke HG. Collapsing glomerulopathy in HIV and non-HIV patients: a clinicopathological and follow-up study. Kidney Int. 1999;56:2203–13.
Valeri A, Barisoni L, Appel GB, Seigle R, D’Agati V. Idiopathic collapsing focal segmental glomerulosclerosis: a clinicopathologic study. Kidney Int. 1996;50:1734–46.
Schwartz GJ, Haycock GB, Edelman CM, Spitzer A. A simple measure of glomerular filtration rate in children derived from body length and plasma creatinine. Pediatrics. 1976;58:259–63.
Myers GL, Miller WG, Coresh J, Fleming J, Greenberg N, Greene T, Hostetter T, Levey AS, Panteghini M, Welch M, Eckfeldt JH, National Kidney Disease Education Program Laboratory Working Group. Recommendations for improving serum creatinine measurement: a report from the laboratory working group of the national kidney disease education program. Clin Chem. 2006;52:5–18.
Cattran DC, Alexopoulos E, Heering P, Hoyer PF, Johnston A, Meyrier A, Ponticelli C, Saito T, Choukroun G, Nachman P, Praga M, Yoshikawa N. Cyclosporin in idiopathic glomerular disease associated with the nephrotic syndrome: workshop recommendations. Kidney Int. 2007;72:1429–47.
Chesney R. The changing face of childhood nephrotic syndrome. Kidney Int. 2004;66:1294–302.
Ponticelli C. Focal segmental glomerular sclerosis. To treat or not to treat? It is worthwhile to give the adult patient with nephrotic syndrome the benefit of an adequate therapeutic trial? Nephrol Dial Transplant. 1995;10:2351–4.
Passerini P, Ponticelli C. Treatment of focal segmental glomerulosclerosis. Curr Opin Nephrol Hypertens. 2001;10:189–93.
Korbet SM. Primary focal segmental glomerulosclerosis. J Am Soc Nephrol. 1998;9:1333–40.
Singh HK, Baldree LA, Mc Kenney DW, Hogan SL, Jennette JC. Idiopathic collapsing glomerulopathy in children. Pediatr Nephrol. 2000;14:132–7.
El-Rafaey AM, Kapur G, Jain A, Hidalgo G, Imam A, Valentini RP, Mattoo TK. Idiopathic collapsing focal segmental glomerulosclerosis in pediatric patients. Pediatr Nephrol. 2007;22:396–402.
Detwiler RK, Falk RJ, Hogan SL, Jennette JC. Collapsing glomerulopathy: a clinically and pathologically distinct variant of focal segmental glomerulosclerosis. Kidney Int. 1994;45:1416–24.
Grcevska L, Polenakovik M. Collapsing glomerulopathy: clinical characteristics and follow-up. Am J Kidney Dis. 1999;33:652–7.
Korbet SM. Treatment of primary focal segmental glomerulosclerosis. Kidney Int. 2002;62:2301–10.
Casado MC, Alarcon GV. Familial collapsing glomerulopathy: clinical, pathological and immunogenetic features. Kidney Int. 2003;63:233–9.
Chesney R. The changing face of childhood nephrotic syndrome. Kidney Int. 2004;66:1294–302.
Wiggins RC. The spectrum of podocytopathies: a unifying view of glomerular diseases. Kidney Int. 2007;71:1205–14.
Author information
Authors and Affiliations
Corresponding author
About this article
Cite this article
Gulati, A., Sharma, A., Hari, P. et al. Idiopathic collapsing glomerulopathy in children. Clin Exp Nephrol 12, 348–353 (2008). https://doi.org/10.1007/s10157-008-0053-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10157-008-0053-6