Focal segmental glomerulosclerosis classification: Difference between revisions
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*Secondary FSGS, presents mostly with non nephrotic syndrome | *Secondary FSGS, presents mostly with non nephrotic syndrome | ||
The Columbia classification of focal segmental glomerulosclerosis (FSGS) based on the morphology by D’Agati<ref name="pmid14750104">{{cite journal| author=D'Agati VD, Fogo AB, Bruijn JA, Jennette JC| title=Pathologic classification of focal segmental glomerulosclerosis: a working proposal. | journal=Am J Kidney Dis | year= 2004| volume= 43 | issue= 2 | pages= 368-82 | pmid=14750104 | doi= | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14750104 }} </ref> | '''The Columbia classification of focal segmental glomerulosclerosis (FSGS) based on the morphology by D’Agati'''<ref name="pmid14750104">{{cite journal| author=D'Agati VD, Fogo AB, Bruijn JA, Jennette JC| title=Pathologic classification of focal segmental glomerulosclerosis: a working proposal. | journal=Am J Kidney Dis | year= 2004| volume= 43 | issue= 2 | pages= 368-82 | pmid=14750104 | doi= | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14750104 }} </ref> | ||
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| | | colspan="4" | '''''Pathological Classification of Focal Segmental Glomerulosclerosis''''' | ||
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| bgcolor="#ececec" |'''Not Otherwise Specified (NOS)''' || Anywhere|| Segmental|| Capillary lumen abolished by the segmental increase in matrix. | | bgcolor="#ececec" |'''Not Otherwise Specified (NOS)''' || Anywhere|| Segmental|| Capillary lumen abolished by the segmental increase in matrix. | ||
|- | |- | ||
| bgcolor="#ececec"|'''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. | | bgcolor="#ececec" |'''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. | ||
|- | |- | ||
| bgcolor="#ececec"|'''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. | | bgcolor="#ececec" |'''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. | ||
|- | |- | ||
| bgcolor="#ececec"|'''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. | | bgcolor="#ececec" |'''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. | ||
|- | |- | ||
|bgcolor="#ececec"|'''Collapsing Variant''' || Anywhere|| Segmental or global|| One or more glomeruli with collapse with evidence of podocyte hypertrophy and hyperplasia. | | bgcolor="#ececec" |'''Collapsing Variant''' || Anywhere|| Segmental or global|| One or more glomeruli with collapse with evidence of podocyte hypertrophy and hyperplasia. | ||
|} | |} | ||
<sup><center>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.</center | <sup><center>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.</center> | ||
==References== | ==References== | ||
Revision as of 19:07, 31 May 2018
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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 with non 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 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.