Minimal change disease classification: Difference between revisions
(8 intermediate revisions by 2 users not shown) | |||
Line 7: | Line 7: | ||
==Classification== | ==Classification== | ||
* [[Minimal change disease]] may be classified into the following variants with only minor changes on [[light microscopy]] and these variants may represent MCD or [[focal segmental glomerulosclerosis]] (FSGS) | * [[Minimal change disease]] may be classified into the following variants with only minor changes on [[light microscopy]] and these variants may represent MCD or [[focal segmental glomerulosclerosis]] ([[Focal segmental glomerulosclerosis|FSGS]]) | ||
** [[Idiopathic]] [[mesangial proliferative glomerulonephritis]]. | ** [[Idiopathic]] [[mesangial proliferative glomerulonephritis]]. | ||
** [[Immunoglobulin]] M ([[IgM]]) [[nephropathy]]. | ** [[Immunoglobulin]] M ([[IgM]]) [[nephropathy]]. | ||
Line 19: | Line 19: | ||
** [[Post-streptococcal glomerulonephritis|Postinfectious glomerulonephritis]] | ** [[Post-streptococcal glomerulonephritis|Postinfectious glomerulonephritis]] | ||
* No immune deposits are found in idiopathic mesangial proliferative glomerulonephritis. | * No [[immune]] deposits are found in [[idiopathic]] [[mesangial proliferative glomerulonephritis]]. | ||
==== IGM NEPHROPATHY ==== | ==== IGM NEPHROPATHY ==== | ||
* In IGM nephropathy patients present with deposits of IgM and complement with electron dense deposits in the mesangium.<ref name="pmid1852859">{{cite journal |vauthors=O'Donoghue DJ, Lawler W, Hunt LP, Acheson EJ, Mallick NP |title=IgM-associated primary diffuse mesangial proliferative glomerulonephritis: natural history and prognostic indicators |journal=Q. J. Med. |volume=79 |issue=288 |pages=333–50 |date=April 1991 |pmid=1852859 |doi= |url=}}</ref> | * In IGM [[nephropathy]] patients present with deposits of [[IgM]] and [[complement]] with [[electron]] dense deposits in the [[mesangium]].<ref name="pmid1852859">{{cite journal |vauthors=O'Donoghue DJ, Lawler W, Hunt LP, Acheson EJ, Mallick NP |title=IgM-associated primary diffuse mesangial proliferative glomerulonephritis: natural history and prognostic indicators |journal=Q. J. Med. |volume=79 |issue=288 |pages=333–50 |date=April 1991 |pmid=1852859 |doi= |url=}}</ref> | ||
* Patients who are presenting with IgM nephropathy are less much likely to respond to immunosuppressive agents than those with MCD. | * Patients who are presenting with [[IgM]] [[nephropathy]] are less much likely to respond to [[Immunosuppression|immunosuppressive]] agents than those with MCD. | ||
==== C1Q NEPHROPATHY ==== | ==== C1Q NEPHROPATHY ==== | ||
* Mesangial deposits on electron microscopy and C1q deposits on immunofluorescence microscopy are noed in patients with C1Q nephropathy.<ref name="pmid16247648">{{cite journal |vauthors=Kersnik Levart T, Kenda RB, Avgustin Cavić M, Ferluga D, Hvala A, Vizjak A |title=C1Q nephropathy in children |journal=Pediatr. Nephrol. |volume=20 |issue=12 |pages=1756–61 |date=December 2005 |pmid=16247648 |doi=10.1007/s00467-005-2040-4 |url=}}</ref><ref name="pmid3875286">{{cite journal |vauthors=Jennette JC, Hipp CG |title=C1q nephropathy: a distinct pathologic entity usually causing nephrotic syndrome |journal=Am. J. Kidney Dis. |volume=6 |issue=2 |pages=103–10 |date=August 1985 |pmid=3875286 |doi= |url=}}</ref> | * [[Mesangial cell|Mesangial]] deposits on [[electron microscopy]] and C1q deposits on [[immunofluorescence]] microscopy are noed in patients with C1Q [[nephropathy]].<ref name="pmid16247648">{{cite journal |vauthors=Kersnik Levart T, Kenda RB, Avgustin Cavić M, Ferluga D, Hvala A, Vizjak A |title=C1Q nephropathy in children |journal=Pediatr. Nephrol. |volume=20 |issue=12 |pages=1756–61 |date=December 2005 |pmid=16247648 |doi=10.1007/s00467-005-2040-4 |url=}}</ref><ref name="pmid3875286">{{cite journal |vauthors=Jennette JC, Hipp CG |title=C1q nephropathy: a distinct pathologic entity usually causing nephrotic syndrome |journal=Am. J. Kidney Dis. |volume=6 |issue=2 |pages=103–10 |date=August 1985 |pmid=3875286 |doi= |url=}}</ref> | ||
* C1Q nephropathy is a subgroup of primary focal segmental glomerulosclerosis.<ref name="pmid1928065">{{cite journal |vauthors=Iskandar SS, Browning MC, Lorentz WB |title=C1q nephropathy: a pediatric clinicopathologic study |journal=Am. J. Kidney Dis. |volume=18 |issue=4 |pages=459–65 |date=October 1991 |pmid=1928065 |doi= |url=}}</ref> | * C1Q [[nephropathy]] is a subgroup of primary [[focal segmental glomerulosclerosis]].<ref name="pmid1928065">{{cite journal |vauthors=Iskandar SS, Browning MC, Lorentz WB |title=C1q nephropathy: a pediatric clinicopathologic study |journal=Am. J. Kidney Dis. |volume=18 |issue=4 |pages=459–65 |date=October 1991 |pmid=1928065 |doi= |url=}}</ref> | ||
===Clinical Classification=== | ===Clinical Classification=== | ||
The clinical classification of minimal change disease is based on the underlying etiology of the disease. | * The clinical classification of [[minimal change disease]] is based on the underlying [[etiology]] of the disease. | ||
====Primary==== | ====Primary==== | ||
In primary (idiopathic) cases, the underlying cause is not known. | * In primary ([[idiopathic]]) cases, the underlying cause is not known. | ||
====Secondary==== | ====Secondary==== | ||
Secondary forms of minimal change disease are associated with certain environmental exposures, such as [[allergies]] ([[bee sting]]), [[malignancies]] ([[lymphoma]]s and [[leukemia]]s), [[medication]]s ([[NSAID]], [[penicillamine]], [[ampicillin]]), and other toxins (gold, mercury) | * Secondary forms of [[minimal change disease]] are associated with certain environmental exposures, such as [[allergies]] ([[bee sting]]), [[malignancies]] ([[lymphoma]]s and [[leukemia]]s), [[medication]]s ([[NSAID]], [[penicillamine]], [[ampicillin]]), and other [[Toxin|toxins]] ([[gold]], [[Mercury (element)|mercury]]).<ref name="pmid16864010">{{cite journal |vauthors=Habib GS, Saliba W, Nashashibi M, Armali Z |title=Penicillamine and nephrotic syndrome |journal=Eur. J. Intern. Med. |volume=17 |issue=5 |pages=343–8 |date=August 2006 |pmid=16864010 |doi=10.1016/j.ejim.2006.03.001 |url=}}</ref> | ||
===Pathological Classification=== | ===Pathological Classification=== | ||
* Minimal change disease currently has no classification system. | * [[Minimal change disease]] currently has no classification system. | ||
* Early observations noted that a small number of patients with minimal change disease have focal tip lesions.<ref name="pmid12046027">{{cite journal| author=Haas M, Yousefzadeh N| title=Glomerular tip lesion in minimal change nephropathy: a study of autopsies before 1950. | journal=Am J Kidney Dis | year= 2002 | volume= 39 | issue= 6 | pages= 1168-75 | pmid=12046027 | doi=10.1053/ajkd.2002.33386 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12046027 }} </ref> | * Early observations noted that a small number of patients with minimal change disease have focal tip lesions.<ref name="pmid12046027">{{cite journal| author=Haas M, Yousefzadeh N| title=Glomerular tip lesion in minimal change nephropathy: a study of autopsies before 1950. | journal=Am J Kidney Dis | year= 2002 | volume= 39 | issue= 6 | pages= 1168-75 | pmid=12046027 | doi=10.1053/ajkd.2002.33386 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12046027 }} </ref> | ||
* 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> | * Based on the proposed Columbia classification by D’Agati and colleagues in 2004, minimal change disease was considered an entity within the spectrum of [[focal segmental glomerulonephritis]] ([[FSGS]]) and may have a clinical course similar to those with “tip lesion” subtype of [[Focal segmental glomerulosclerosis|FSGS]].<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> | ||
{| border="1" style="border-collapse:collapse; text-align:left;" cellpadding="5" align="center" | {| border="1" style="border-collapse:collapse; text-align:left;" cellpadding="5" align="center" | ||
|+ '''''Pathological Classification of Focal Segmental Glomerulosclerosis<ref name="pmid14750104">{{cite journal| | |+ '''''Pathological Classification of Focal Segmental Glomerulosclerosis<ref name="pmid14750104">{{cite journal |vauthors=D'Agati VD, Fogo AB, Bruijn JA, Jennette JC |title=Pathologic classification of focal segmental glomerulosclerosis: a working proposal |journal=Am. J. Kidney Dis. |volume=43 |issue=2 |pages=368–82 |date=February 2004 |pmid=14750104 |doi= |url=}}</ref>''''' | ||
| bgcolor="#d9ff54" |'''Variant''' || bgcolor="#d9ff54" |'''Location of Lesion'''|| bgcolor="#d9ff54" |'''Distribution of Lesion'''|| bgcolor="#d9ff54" |'''Characteristic Features''' | | bgcolor="#d9ff54" |'''Variant''' || bgcolor="#d9ff54" |'''Location of Lesion'''|| bgcolor="#d9ff54" |'''Distribution of Lesion'''|| bgcolor="#d9ff54" |'''Characteristic Features''' | ||
|- | |- | ||
| 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 | | 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. | ||
Line 55: | Line 55: | ||
|- | |- | ||
| 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> | ||
<sup | |||
==References== | ==References== |
Latest revision as of 14:23, 13 June 2018
Minimal Change Disease Microchapters |
Diagnosis |
---|
Treatment |
Case Studies |
Minimal change disease classification On the Web |
American Roentgen Ray Society Images of Minimal change disease classification |
Risk calculators and risk factors for Minimal change disease classification |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Yazan Daaboul, Serge Korjian; Vamsikrishna Gunnam M.B.B.S [2]
Overview
Minimal change disease can be classified based on the underlying clinical etiology of disease into primary and secondary. Minimal change disease currently has no pathological classification system. Based on the proposed Columbia classification, minimal change disease was considered an entity within the spectrum of focal segmental glomerulonephritis (FSGS).
Classification
- Minimal change disease may be classified into the following variants with only minor changes on light microscopy and these variants may represent MCD or focal segmental glomerulosclerosis (FSGS)
IDIOPATHIC MESANGIAL PROLIFERATIVE GLOMERULONEPHRITIS
- Focal(<50% of glomeruli are involved) or diffuse(>50% of glomeruli are involved) is an non specific sign for glomerular injury.
- IDIOPATHIC MESANGIAL PROLIFERATIVE GLOMERULONEPHRITIS is seen in [1]
- No immune deposits are found in idiopathic mesangial proliferative glomerulonephritis.
IGM NEPHROPATHY
- In IGM nephropathy patients present with deposits of IgM and complement with electron dense deposits in the mesangium.[2]
- Patients who are presenting with IgM nephropathy are less much likely to respond to immunosuppressive agents than those with MCD.
C1Q NEPHROPATHY
- Mesangial deposits on electron microscopy and C1q deposits on immunofluorescence microscopy are noed in patients with C1Q nephropathy.[3][4]
- C1Q nephropathy is a subgroup of primary focal segmental glomerulosclerosis.[5]
Clinical Classification
- The clinical classification of minimal change disease is based on the underlying etiology of the disease.
Primary
- In primary (idiopathic) cases, the underlying cause is not known.
Secondary
- Secondary forms of minimal change disease are associated with certain environmental exposures, such as allergies (bee sting), malignancies (lymphomas and leukemias), medications (NSAID, penicillamine, ampicillin), and other toxins (gold, mercury).[6]
Pathological Classification
- Minimal change disease currently has no classification system.
- Early observations noted that a small number of patients with minimal change disease have focal tip lesions.[7]
- Based on the proposed Columbia classification by D’Agati and colleagues in 2004, minimal change disease was considered an entity within the spectrum of focal segmental glomerulonephritis (FSGS) and may have a clinical course similar to those with “tip lesion” subtype of FSGS.[8]
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. |
References
- ↑ Sagel I, Treser G, Ty A, Yoshizawa N, Kleinberger H, Yuceoglu AM, Wasserman E, Lange K (October 1973). "Occurrence and nature of glomerular lesions after group A streptococci infections in children". Ann. Intern. Med. 79 (4): 492–9. PMID 4795879.
- ↑ O'Donoghue DJ, Lawler W, Hunt LP, Acheson EJ, Mallick NP (April 1991). "IgM-associated primary diffuse mesangial proliferative glomerulonephritis: natural history and prognostic indicators". Q. J. Med. 79 (288): 333–50. PMID 1852859.
- ↑ Kersnik Levart T, Kenda RB, Avgustin Cavić M, Ferluga D, Hvala A, Vizjak A (December 2005). "C1Q nephropathy in children". Pediatr. Nephrol. 20 (12): 1756–61. doi:10.1007/s00467-005-2040-4. PMID 16247648.
- ↑ Jennette JC, Hipp CG (August 1985). "C1q nephropathy: a distinct pathologic entity usually causing nephrotic syndrome". Am. J. Kidney Dis. 6 (2): 103–10. PMID 3875286.
- ↑ Iskandar SS, Browning MC, Lorentz WB (October 1991). "C1q nephropathy: a pediatric clinicopathologic study". Am. J. Kidney Dis. 18 (4): 459–65. PMID 1928065.
- ↑ Habib GS, Saliba W, Nashashibi M, Armali Z (August 2006). "Penicillamine and nephrotic syndrome". Eur. J. Intern. Med. 17 (5): 343–8. doi:10.1016/j.ejim.2006.03.001. PMID 16864010.
- ↑ Haas M, Yousefzadeh N (2002). "Glomerular tip lesion in minimal change nephropathy: a study of autopsies before 1950". Am J Kidney Dis. 39 (6): 1168–75. doi:10.1053/ajkd.2002.33386. PMID 12046027.
- ↑ 8.0 8.1 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.