Focal segmental glomerulosclerosis classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Manpreet Kaur, MD [2]
Overview
Classification
FSGS can be classified as primary and secondary disease depending on etiology, the course of the disease and histologic pattern:
- Primary also known as idiopathic FSGS, presents mostly with the nephrotic syndrome[1]
- Secondary FSGS, presents mostly without nephrotic syndrome
The Columbia classification of focal segmental glomerulosclerosis (FSGS) based on the morphology by D’Agati[2]
| Pathological Classification of Focal Segmental Glomerulosclerosis | |||
| Not Otherwise Specified (NOS) | Anywhere | Segmental | Capillary lumen abolished by the segmental increase in the 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. |
References
- ↑ Kang DH, Joly AH, Oh SW, Hugo C, Kerjaschki D, Gordon KL; et al. (2001). "Impaired angiogenesis in the remnant kidney model: I. Potential role of vascular endothelial growth factor and thrombospondin-1". J Am Soc Nephrol. 12 (7): 1434–47. PMID 11423572 PMID 11423572 Check
|pmid=value (help). - ↑ D'Agati VD, Fogo AB, Bruijn JA, Jennette JC (2004). "Pathologic classification of focal segmental glomerulosclerosis: a working proposal". Am J Kidney Dis. 43 (2): 368–82. PMID 14750104.