What is the significance of a histologic finding of focal segmental glomerulosclerosis (FSGS) in pediatric nephrotic syndrome?

Updated: Mar 04, 2020
  • Author: Jerome C Lane, MD; Chief Editor: Craig B Langman, MD  more...
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Answer

FSGS describes a lesion in which, as seen on light microscopy (LM), discrete segments of the glomerular tuft reveal sclerosis (segmental); some glomeruli are involved, and others are spared (focal).

Adhesion of the glomerular tuft to the Bowman capsule (synechiae) is observed. Glomerular hypertrophy is common. Interstitial fibrosis and tubular atrophy are often present and correlate with the severity of disease.

IF reveals IgM and C3 trapped in the sclerotic areas. As in MCNS, EM reveals effacement of the podocyte foot processes. Additionally, EM reveals obliteration of capillary lumens by fine granular and lipid deposits.

A subtype of FSGS, in which the glomerular tufts demonstrate collapse of capillaries (collapsing glomerulopathy) on LM, has a poorer prognosis and high rate of progression to end-stage kidney disease (ESKD).

FSGS is not a specific disease but a histopathologic finding that can be associated with INS but can also be found in a wide variety of other conditions, including HIV nephropathy, heroin nephropathy, reflux nephropathy, obstructive uropathy, renal hypoplasia, hypertension, obesity, and Alport syndrome.

As always, clinical and histopathologic correlations must be made when considering the findings evident on kidney biopsy. [60]


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