Focal segmental glomerulosclerosis other diagnostic studies

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief:’’’ Cafer Zorkun, M.D., Ph.D. [2]

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Renal Biopsy

A kidney biopsy with electron microscopy is recommended for all patients with FSGS for prognostic and therapeutic purposes. The following table shows the pathological classification of FSGS based on characteristic features. Electron microscopy generally shows podocyte foot process effacement.

Based on the proposed Columbia classification by D’Agati and colleagues in 2004, the classification of focal segmental glomerulosclerosis (FSGS) based on the morphology is as follows:

Pathological Classification of Focal Segmental Glomerulosclerosis

Variant	Location of Lesion	Distribution of Lesion	Characteristic Features
Not Otherwise Specified (NOS)	Anywhere	Segmental	Capillary lumen abolished by the segmental increase in matrix.
Perihilar Variant	Perihilar	Segmental	Presence of one or more glomeruli containing hyalinosis in the perihilar regions with or without sclerosis. Within each glomerulus, the segmental lesions must contain > 50% perihilar hyalinosis and/or sclerosis.
Cellular Variant	Anywhere	Segmental	Presence of one or more glomerulus with segmental hypercellularity of the capillary endothelium that blocks the capillary lumen, with or without foam cells and/or karryohexis.
Tip Variant	At tip domain	Segmental	One or more segmental lesions, that include tip domains. Lesions must have adhesions/confluence of podocytes with parietal or tubular cells. Tip domains are defined as 25% of tuft adjacent to the origin of the proximal tubule. Sclerosing lesions shuld be <25% of tuft, while cellular lesions should be < 50% of tuft. No perihilar sclerosis should be observed.
Collapsing Variant	Anywhere	Segmental or global	One or more glomeruli with collapse with evidence of podocyte hypertrophy and hyperplasia.

^{Adapted from D’Agati VD, Fogo AB, Bruijn JA, and Jennette JC. Pathological classification of focal segmental glomerulosclerosis. A working proposal. Am J of Kidney Dis. 2004; 43(2):368-382.}