Focal segmental glomerulosclerosis classification: Difference between revisions

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__NOTOC__
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{{Focal segmental glomerulosclerosis}}
{{Focal segmental glomerulosclerosis}}
{{CMG}} {{AE}} {{MKK}}
{{CMG}} {{AE}} {{MKA}}, {{MKK}}
==Overview==
==Overview==
[[Focal segmental glomerulosclerosis|FSGS]] can be classified as primary and secondary disease depending on etiology, the course of the disease and [[histologic]] pattern
FSGS can be classified as primary and secondary [[disease]] depending on [[etiology]], the course of the [[disease]] and [[histologic]] pattern.


== Classification ==
== Classification ==
[[Focal segmental glomerulosclerosis|FSGS]] can be classified as primary and secondary disease depending on etiology, the course of the disease and [[histologic]] pattern:  
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 [[Focal segmental glomerulosclerosis|FSGS]], presents mostly with the [[nephrotic syndrome]]<ref name="pmidPMID 11423572">{{cite journal| author=Kang DH, Joly AH, Oh SW, Hugo C, Kerjaschki D, Gordon KL et al.| title=Impaired angiogenesis in the remnant kidney model: I. Potential role of vascular endothelial growth factor and thrombospondin-1. | journal=J Am Soc Nephrol | year= 2001 | volume= 12 | issue= 7 | pages= 1434-47 | pmid=PMID 11423572 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11423572  }} </ref>   
*Primary also known as [[idiopathic]] FSGS, presents mostly with [[nephrotic syndrome]]<ref name="pmidPMID 11423572">{{cite journal| author=Kang DH, Joly AH, Oh SW, Hugo C, Kerjaschki D, Gordon KL et al.| title=Impaired angiogenesis in the remnant kidney model: I. Potential role of vascular endothelial growth factor and thrombospondin-1. | journal=J Am Soc Nephrol | year= 2001 | volume= 12 | issue= 7 | pages= 1434-47 | pmid=PMID 11423572 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11423572  }} </ref>   
*Secondary [[Focal segmental glomerulosclerosis|FSGS]], presents mostly without [[nephrotic syndrome]]  
*Secondary FSGS, presents mostly without [[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 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>  


{|
{|
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
|+  
|+  
| colspan="4" |                                                              '''''Pathological Classification of Focal Segmental Glomerulosclerosis'''''
| colspan="4" |                                                              '''''Pathological Classification of Focal Segmental Glomerulosclerosis'''''
|-
|-
| bgcolor="#ececec" |'''Not Otherwise Specified (NOS)''' || Anywhere|| Segmental|| [[Capillary]] [[lumen]] abolished by the segmental increase in the [[matrix]].
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
|Variant
|Location
|Distribution
|Features
|-
|-
| 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.
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Not Otherwise Specified (NOS)'''  
| style="background: #F5F5F5; padding: 5px;" |Anywhere
| style="background: #F5F5F5; padding: 5px;" |Segmental
| style="background: #F5F5F5; padding: 5px;" |[[Capillary]] [[lumen]] abolished by the segmental increase in the [[matrix]].
|-
|-
| 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.
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Perihilar Variant'''
| style="background: #F5F5F5; padding: 5px;" |Perihilar
| style="background: #F5F5F5; padding: 5px;" |Segmental
| style="background: #F5F5F5; padding: 5px;" |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" |'''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.
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Cellular Variant'''
| style="background: #F5F5F5; padding: 5px;" |Anywhere
| style="background: #F5F5F5; padding: 5px;" |Segmental
| style="background: #F5F5F5; padding: 5px;" |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]].
|-
|-
| bgcolor="#ececec" |'''Collapsing Variant''' || Anywhere|| Segmental or global|| One or more glomeruli with collapse with evidence of podocyte hypertrophy and hyperplasia.
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Tip Variant'''
| style="background: #F5F5F5; padding: 5px;" |At tip domain
| style="background: #F5F5F5; padding: 5px;" |Segmental
| style="background: #F5F5F5; padding: 5px;" |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]] [[Tubules|tubule]].  Sclerosing lesions shuld be <25% of tuft, while [[cellular]] [[lesions]] should be < 50% of tuft. No perihilar [[sclerosis]] should be observed.
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Collapsing Variant'''
| style="background: #F5F5F5; padding: 5px;" |Anywhere
| style="background: #F5F5F5; padding: 5px;" |Segmental or global
| style="background: #F5F5F5; padding: 5px;" |One or more [[glomeruli]] with [[collapse]] with evidence of [[podocyte]] [[hypertrophy]] and [[hyperplasia]].
|}
|}


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[[Category:Up-To-Date]]
[[Category:Medicine]]
[[Category:Nephrology]]

Latest revision as of 21:46, 29 July 2020

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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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