Focal segmental glomerulosclerosis classification: Difference between revisions

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== Classification ==
== Classification ==
Depending on the cause it is broadly classified as  
Based on the proposed Columbia classification by D’Agati and colleagues<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> in 2004, the classification of focal segmental glomerulosclerosis (FSGS) based on the morphology is as follows:
*Primary, when no underlying cause is found; usually presents as [[nephrotic syndrome]]
 
*Secondary, when an underlying cause is identified; usually presents with [[kidney failure]] and [[proteinuria]]. This is actually a heterogeneous group including a myriad causes such as
{| border="1" style="border-collapse:collapse; text-align:left;" cellpadding="5" align="center"
**Infections such as [[HIV]] (known as HIV-Associated Nephropathy)
|+ '''''Pathological Classification of Focal Segmental Glomerulosclerosis<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>'''''
**Toxins and drugs such as [[heroin]] and [[pamidronate]]
| bgcolor="#d9ff54"|'''Variant''' || bgcolor="#d9ff54"|'''Location of Lesion'''||bgcolor="#d9ff54"|'''Distribution of Lesion'''|| bgcolor="#d9ff54"|'''Characteristic Features'''
**Familial forms
|-
**Secondary to [[nephron]] loss and hyperfiltration, such as with chronic [[pyelonephritis]] and [[reflux]], morbid [[obesity]], [[diabetes mellitus]]
| 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"|'''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"|'''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>


==References==
==References==

Revision as of 18:00, 3 December 2013

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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]

Overview

Classification

Based on the proposed Columbia classification by D’Agati and colleagues[1] in 2004, the classification of focal segmental glomerulosclerosis (FSGS) based on the morphology is as follows:

Pathological Classification of Focal Segmental Glomerulosclerosis[1]
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.

References

  1. 1.0 1.1 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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