Focal segmental glomerulosclerosis classification: Difference between revisions

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__NOTOC__
__NOTOC__
{{Focal segmental glomerulosclerosis}}
{{Focal segmental glomerulosclerosis}}
{{CMG}}{{APM}}'''Associate Editor-In-Chief:’’’ {{OO}}
{{CMG}} {{AE}} {{MKA}}, {{MKK}}
==Overview==
==Overview==
FSGS can be classified as primary and secondary [[disease]] depending on [[etiology]], the course of the [[disease]] and [[histologic]] pattern.


== Classification ==
== 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 [[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 FSGS, presents mostly without [[nephrotic syndrome]]


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'''<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 primary cause of FSGS is unknown or idiopathic, but there are several postulations as probable causes. 80% of FSGS cases are idiopathic. Primary or Idiopathic FSGS often presents with features of Nephrotic Syndrome with associated hematuria (microscopic), hypertension and renal insufficiency.
Some genetic mutations had been associated with familial idiopathic FSGS. The role of over-expression of inflammatory markers like Tumor Necrosis Factor (TNF), Interleukins had also been associated with the extent of glomerular sclerosis.<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 FSGS is better described. It often occurs from some glomerular injury from previous glomeruli injury and hypertrophy. Secondary FSGS presents with non-nephrotic proteinuria and with some renal insufficiency. Etiologies of Secondary FSGS include:
{|
*Infections with HIV, HBV;
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
*Drugs like anabolic steroids, heroin, lithium, pamidronate, analgesics
|+
*Conditions like sickle cell disease and obesity.
| colspan="4" |                                                             '''''Pathological Classification of Focal Segmental Glomerulosclerosis'''''
*Systemic diseases like SLE, Lupus Nephritis, IgA Nephropathy that can cause glomerular scarring can cause Secondary FSGS.
|-
*Genetic mutations with familial mode of inheritance had also been implicated in Secondary FSGS.
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
 
|Variant
 
|Location
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:
|Distribution
 
|Features
{| class="wikitable"
|+ '''''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>'''''
| bgcolor="#ececec" style="text-align:center"|'''Variant''' || bgcolor="#ececec" style="text-align:center"|'''Location of Lesion'''||bgcolor="#ececec" style="text-align:center"|'''Distribution of Lesion'''|| bgcolor="#ececec" style="text-align:center"|'''Characteristic Features'''
|-
|-
| bgcolor="#ececec" |'''Not Otherwise Specified (NOS)''' || Anywhere|| Segmental|| Capillary lumen abolished by the segmental increase in matrix.
| 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"|'''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;" |'''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"|'''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;" |'''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"|'''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;" |'''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.
|-
|-
|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;" |'''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]].
|}
|}


<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>
<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==
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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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