Focal segmental glomerulosclerosis classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: M. Khurram Afzal, MD [2], Manpreet Kaur, MD [3]

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:

The Columbia classification of focal segmental glomerulosclerosis (FSGS) based on morphology by D’Agati[2]

Pathological Classification of Focal Segmental Glomerulosclerosis
Variant Location Distribution Features
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 karyorrhexis.
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.

References

  1. 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).
  2. 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.


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