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
FSGS can be classified as primary and secondary disease depending on etiology, the course of the disease and histologic pattern
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.