Difference between revisions of "Medical kidney diseases"

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Medical kidney diseases (view source)

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This article describes '''medical renal disease''' or the '''medical kidney'''.  Much in medical kidney depends on the clinical information.  Most of the disease seen by pathologists is... glomerular disease.  If in doubt... the answer to most questions is ''[[diabetes mellitus]]'' or ''[[systemic lupus erythematosus]]''.  Medical kidney is niche area in pathology.  It is one of the few areas that routinely requires [[electron microscopy]].
[[Image:Acute thrombotic microangiopathy - pas - very high mag.jpg|250px|thumb|[[Micrograph]] showing a glomerulus with an [[thrombotic microangiopathy|acute thrombotic microangiopathy]]. [[PAS stain]].]]
This article describes '''medical renal disease''' or the '''medical kidney'''.  Much in medical kidney depends on the clinical information.  Most of the disease seen by pathologists is... glomerular disease. It is one of the few areas that routinely requires [[electron microscopy]].
   
Medical kidney is niche area in pathology. In the context of exams for general pathologists, if in doubt... the answer to most questions is ''[[diabetes mellitus]]'' or ''[[systemic lupus erythematosus]]''.   


Kidney tumours are dealt with in the ''[[kidney tumours]]'' article, and ''[[pediatric kidney tumours]]'' article.
Kidney tumours are dealt with in the ''[[kidney tumours]]'' article, and ''[[pediatric kidney tumours]]'' article.  


=Clinical=
=Clinical=
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Notes:
Notes:
*Dinosaurs use the units mg/dL; normal with these units is: 0.8 to 1.4 mg/dL.<ref>URL: [http://www.nlm.nih.gov/medlineplus/ency/article/003475.htm http://www.nlm.nih.gov/medlineplus/ency/article/003475.htm]. Accessed on: 8 November 2010.</ref>
*In some parts of the world they use the units mg/dL; normal with these units is: 0.8 to 1.4 mg/dL.<ref>URL: [http://www.nlm.nih.gov/medlineplus/ency/article/003475.htm http://www.nlm.nih.gov/medlineplus/ency/article/003475.htm]. Accessed on: 8 November 2010.</ref>
*Conversion: 1.0 mg/dL = 88.4 umol/L.<ref>URL: [http://www.sydpath.stvincents.com.au/other/Conversions/ConversionMasterF3.htm http://www.sydpath.stvincents.com.au/other/Conversions/ConversionMasterF3.htm]. Accessed on: 8 November 2010.</ref><ref>URL: [http://www.unc.edu/~rowlett/units/scales/clinical_data.html http://www.unc.edu/~rowlett/units/scales/clinical_data.html]. Accessed on: 8 November 2010.
*Conversion: 1.0 mg/dL = 88.4 umol/L.<ref>URL: [http://www.sydpath.stvincents.com.au/other/Conversions/ConversionMasterF3.htm http://www.sydpath.stvincents.com.au/other/Conversions/ConversionMasterF3.htm]. Accessed on: 8 November 2010.</ref><ref>URL: [http://www.unc.edu/~rowlett/units/scales/clinical_data.html http://www.unc.edu/~rowlett/units/scales/clinical_data.html]. Accessed on: 8 November 2010.
</ref>
</ref>
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Cut points:<ref>URL: [http://www.fpnotebook.com/urology/lab/urnprtntcrtnrt.htm http://www.fpnotebook.com/urology/lab/urnprtntcrtnrt.htm]. Accessed on: 8 November 2010.</ref>
Cut points:<ref>URL: [http://www.fpnotebook.com/urology/lab/urnprtntcrtnrt.htm http://www.fpnotebook.com/urology/lab/urnprtntcrtnrt.htm]. Accessed on: 8 November 2010.</ref>
*Normal (2 years and older): <0.2 g protein / g Creatinine
*Normal (2 years and older): <0.2 g protein / g Creatinine
*Nephrotic range: >3.5 g protein / g Creatinine.  
*[[Nephrotic syndrome|Nephrotic]] range: >3.5 g protein / g Creatinine.  


===Complement===
===Complement===
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*Changed:
*Changed:
**[[Systemic lupus erythematosus]] (SLE); levels decreased = increased disease activity.  
**[[Systemic lupus erythematosus]] (SLE); levels decreased = increased disease activity.  
**Post-infectious GN - decreased.
**[[Post-infectious GN]] - decreased.
**MPGN - decreased. (???)
**[[MPGN]] - decreased. (???)
**Infection.<ref name=pmid3892188>{{cite journal |author=Nusinow SR, Zuraw BL, Curd JG |title=The hereditary and acquired deficiencies of complement |journal=Med. Clin. North Am. |volume=69 |issue=3 |pages=487–504 |year=1985 |month=May |pmid=3892188 |doi= |url=}}</ref><ref>URL: [http://www.google.com/url?sa=t&source=web&cd=3&sqi=2&ved=0CB0QFjAC&url=http%3A%2F%2Fwww.beckmancoulter.com%2Fliterature%2FClinDiag%2FBulletin%25209282C3_C4.pdf&rct=j&q=C3%2C%20C4%20decreased%2C%20l&ei=0evYTPLHHcX9ngfetNDzCQ&usg=AFQjCNFR26FHyAmy4lZ3tm-tLgW-oD73Zg&cad=rja beckmancoulter.com]. Accessed on: 9 November 2010.</ref>
**Infection.<ref name=pmid3892188>{{cite journal |author=Nusinow SR, Zuraw BL, Curd JG |title=The hereditary and acquired deficiencies of complement |journal=Med. Clin. North Am. |volume=69 |issue=3 |pages=487–504 |year=1985 |month=May |pmid=3892188 |doi= |url=}}</ref><ref>URL: [http://www.google.com/url?sa=t&source=web&cd=3&sqi=2&ved=0CB0QFjAC&url=http%3A%2F%2Fwww.beckmancoulter.com%2Fliterature%2FClinDiag%2FBulletin%25209282C3_C4.pdf&rct=j&q=C3%2C%20C4%20decreased%2C%20l&ei=0evYTPLHHcX9ngfetNDzCQ&usg=AFQjCNFR26FHyAmy4lZ3tm-tLgW-oD73Zg&cad=rja beckmancoulter.com]. Accessed on: 9 November 2010.</ref>
**Hemolytic [[anemia]].<ref name=pmid3892188>{{cite journal |author=Nusinow SR, Zuraw BL, Curd JG |title=The hereditary and acquired deficiencies of complement |journal=Med. Clin. North Am. |volume=69 |issue=3 |pages=487–504 |year=1985 |month=May |pmid=3892188 |doi= |url=}}</ref><ref>URL: [http://www.google.com/url?sa=t&source=web&cd=3&sqi=2&ved=0CB0QFjAC&url=http%3A%2F%2Fwww.beckmancoulter.com%2Fliterature%2FClinDiag%2FBulletin%25209282C3_C4.pdf&rct=j&q=C3%2C%20C4%20decreased%2C%20l&ei=0evYTPLHHcX9ngfetNDzCQ&usg=AFQjCNFR26FHyAmy4lZ3tm-tLgW-oD73Zg&cad=rja beckmancoulter.com]. Accessed on: 9 November 2010.</ref>
**Hemolytic [[anemia]].<ref name=pmid3892188>{{cite journal |author=Nusinow SR, Zuraw BL, Curd JG |title=The hereditary and acquired deficiencies of complement |journal=Med. Clin. North Am. |volume=69 |issue=3 |pages=487–504 |year=1985 |month=May |pmid=3892188 |doi= |url=}}</ref><ref>URL: [http://www.google.com/url?sa=t&source=web&cd=3&sqi=2&ved=0CB0QFjAC&url=http%3A%2F%2Fwww.beckmancoulter.com%2Fliterature%2FClinDiag%2FBulletin%25209282C3_C4.pdf&rct=j&q=C3%2C%20C4%20decreased%2C%20l&ei=0evYTPLHHcX9ngfetNDzCQ&usg=AFQjCNFR26FHyAmy4lZ3tm-tLgW-oD73Zg&cad=rja beckmancoulter.com]. Accessed on: 9 November 2010.</ref>
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**Previously p-ANCA.
**Previously p-ANCA.
**Seen in ANCA-[[vasculitides]], esp. [[microscopic polyangiitis]].  
**Seen in ANCA-[[vasculitides]], esp. [[microscopic polyangiitis]].  
*PR3-ANPA (proteinase 3 antineutrophil cytoplasmic autoantibody).  
*PR3-ANCA (proteinase 3 antineutrophil cytoplasmic autoantibody).  
**Previously c-ANCA.
**Previously c-ANCA.
**Seen in ANCA-[[vasculitides]], esp. [[Wegener granulomatosis]].
**Seen in ANCA-[[vasculitides]], esp. [[granulomatosis with polyangiitis]] (Wegener granulomatosis).


===C4d===
===C4d===
*Suggests humoral immunity (antibody-mediated immunity) at play.
*Suggests humoral immunity (antibody-mediated immunity) at play.
*Important in monitoring of renal transplant recipients.
*Important in monitoring of renal transplant recipients.
*Immunostain also available - see below.
*[[Immunostain]] also available - see below.


===Other blood work===
===Other blood work===
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====Mixed====
====Mixed====
*Features of nephritic syndrome and nephrotic syndrome.
*Features of nephritic syndrome and [[nephrotic syndrome]].


=Clinical DDx=
=Clinical DDx=
==Renal failure 101==
*Prerenal.
**Shock - cardiogenic, hypovolemic, neurogenic, septic, anaphylatic.
*Renal.
**What this article deals with.
*Postrenal.
**Outflow obstruction, e.g. [[prostatic nodular hyperplasia]] ([[AKA]] BPH).
==Renal causes==
*Isolated hematuria.
*Isolated proteinuria.
*Nephrotic syndrome.
*Nephritis syndrome.
*Rapidly progressive glomerulonephritis.
*Chronic renal failure.
*A combination of any of the above.
The clinical presentations suggest a pathologic DDx.<ref>URL: [http://www.emedicine.com/med/topic886.htm http://www.emedicine.com/med/topic886.htm] and [http://www.emedicine.com/ped/topic1564.htm http://www.emedicine.com/ped/topic1564.htm]. Accessed on: 8 November 2010.</ref>
The clinical presentations suggest a pathologic DDx.<ref>URL: [http://www.emedicine.com/med/topic886.htm http://www.emedicine.com/med/topic886.htm] and [http://www.emedicine.com/ped/topic1564.htm http://www.emedicine.com/ped/topic1564.htm]. Accessed on: 8 November 2010.</ref>


===Nephritic===
===Isolated hematuria===
*Post-infectious glomerulonephritis.
*[[IgA nephropathy]].<ref name=pmid19949735>{{Cite journal  | last1 = Kim | first1 = BS. | last2 = Kim | first2 = YK. | last3 = Shin | first3 = YS. | last4 = Kim | first4 = YO. | last5 = Song | first5 = HC. | last6 = Kim | first6 = YS. | last7 = Choi | first7 = EJ. | title = Natural history and renal pathology in patients with isolated microscopic hematuria. | journal = Korean J Intern Med | volume = 24 | issue = 4 | pages = 356-61 | month = Dec | year = 2009 | doi = 10.3904/kjim.2009.24.4.356 | PMID = 19949735 }}</ref>
*[[Alport syndrome]].
*[[Thin glomerular basement membrane disease]].
 
===Proteinuria===
*[[Diabetic nephropathy]].
*Classic causes of nephrotic syndrome:
**[[FSGS]].
**[[MCD]].
**[[MN]].
*Paraprotein-related:
**[[Renal amyloidosis]].
**Monoclonal immunoglobulin deposition disease (MIDD).
*Weird stuff:
**[[Fibrillary glomerulopathy]].
**[[Immunotactoid glomerulopathy]].
 
===Nephritic syndrome===
Classic:
*[[Post-infectious glomerulonephritis]].
**Classically streptococcal.
**Classically streptococcal.
*Crescentic glomerulonephritis ([[AKA]] rapidly progressive glomerulonephritis (RPGN)).
*Crescentic glomerulonephritis ([[AKA]] rapidly progressive glomerulonephritis (RPGN)).


====RPGN====  
Others:
*[[IgA nephropathy]].
*[[Lupus nephritis]].
 
====Rapidly progressive glomerulonephritis====  
Classification:<ref>URL: [http://bestpractice.bmj.com/best-practice/monograph/207/basics/classification.html http://bestpractice.bmj.com/best-practice/monograph/207/basics/classification.html]. Accessed on: 17 November 2011.</ref>
Classification:<ref>URL: [http://bestpractice.bmj.com/best-practice/monograph/207/basics/classification.html http://bestpractice.bmj.com/best-practice/monograph/207/basics/classification.html]. Accessed on: 17 November 2011.</ref>
#Linear immune deposits.
#Linear immune deposits.
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#**[[Microscopic polyangiitis]].
#**[[Microscopic polyangiitis]].


===Nephrotic===
===Nephrotic syndrome===
*Minimal segmental disease (MSD) - [[AKA]] ''minimal change disease'' (MCD).  
*Minimal segmental disease (MSD) - [[AKA]] ''minimal change disease'' (MCD).  
*[[Focal segmental glomerulosclerosis]] (FSGS).  
*[[Focal segmental glomerulosclerosis]] (FSGS).  
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*[[Membranoproliferative glomerulonephritis]] (MPGN).
*[[Membranoproliferative glomerulonephritis]] (MPGN).


=Normal=
=Normal kidney=
===Cells of the glomerulus===
===Cells of the glomerulus===
*Podocytes.
*Podocytes.
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**Another autopsy series suggests the mean diameter is: 225 micrometers.<ref>{{Cite journal  | last1 = Nyengaard | first1 = JR. | last2 = Bendtsen | first2 = TF. | title = Glomerular number and size in relation to age, kidney weight, and body surface in normal man. | journal = Anat Rec | volume = 232 | issue = 2 | pages = 194-201 | month = Feb | year = 1992 | doi = 10.1002/ar.1092320205 | PMID = 1546799 }}</ref>
**Another autopsy series suggests the mean diameter is: 225 micrometers.<ref>{{Cite journal  | last1 = Nyengaard | first1 = JR. | last2 = Bendtsen | first2 = TF. | title = Glomerular number and size in relation to age, kidney weight, and body surface in normal man. | journal = Anat Rec | volume = 232 | issue = 2 | pages = 194-201 | month = Feb | year = 1992 | doi = 10.1002/ar.1092320205 | PMID = 1546799 }}</ref>


Note:
*If the glomerulus fills half the field at 40x on a microscope with 22 mm diameter eye pieces it is ~ 0.275 mm or 275 micrometers.
====Glomerular basement membrane====
====Glomerular basement membrane====
The glomerular basement membrane (GBM) should be thinner than the tubular basement membrane.
The glomerular basement membrane (GBM) should be thinner than the tubular basement membrane.
===Images===
<gallery>
Image: Benign kidney -- very low mag.jpg | Benign kidney - very low mag.
Image: Benign kidney -- low mag.jpg | Benign kidney - low mag.
Image: Benign kidney - alt -- low mag.jpg | Benign kidney - low mag.
Image: Benign kidney -- intermed mag.jpg | Benign kidney - intermed. mag.
</gallery>


=Basic approach to renal biopsy=
=Basic approach to renal biopsy=
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*Interstitium.
*Interstitium.
*Vessels.
*Vessels.
Memory device ''GI TV'': glomeruli, interstitium, tubules, vessels.


===Glomeruli===
===Glomeruli===
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*Count the number of glomeruli.
*Count the number of glomeruli.
*Count number of the obsolete glomeruli.
*Count number of the obsolete glomeruli.
Notes:
*Endocapillary hypercellularity has a DDx - it includes: [[IgA nephropathy]],<ref name=pmid21258273>{{Cite journal  | last1 = Grcevska | first1 = L. | last2 = Ristovska | first2 = V. | last3 = Nikolov | first3 = V. | last4 = Petrusevska | first4 = G. | last5 = Milovanceva-Popovska | first5 = M. | last6 = Polenakovic | first6 = M. | title = The Oxford classification of IgA nephropathy: single centre experience. | journal = Prilozi | volume = 31 | issue = 2 | pages = 7-16 | month = Dec | year = 2010 | doi =  | PMID = 21258273 }}</ref> and [[lupus nephritis]].<ref name=pmid6975091>{{Cite journal  | last1 = Jothy | first1 = S. | last2 = Sawka | first2 = RJ. | title = Presence of monocytes in systemic lupus erythematosus-associated glomerulonephritis: marker study and significance. | journal = Arch Pathol Lab Med | volume = 105 | issue = 11 | pages = 590-3 | month = Nov | year = 1981 | doi =  | PMID = 6975091 }}</ref>


====Components of the glomeruli (anatomical)====
====Components of the glomeruli (anatomical)====
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Consider:
Consider:
*Vasculitis? - inflammatory cells in vessel wall.
*[[Vasculitis]]? - inflammatory cells in vessel wall.
*Amyloid? - pink.
*[[Amyloid]]? - pink.
*Rejection? - PMNs.
*Rejection? - [[PMN]]s.
*Cholesterol emboli?
*[[Cholesterol emboli]]?


====Arteriolar hyalinosis====
====Arteriolar hyalinosis====
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*'''A'''ging, '''D'''iabetes, '''H'''ypertension, '''D'''rugs.
*'''A'''ging, '''D'''iabetes, '''H'''ypertension, '''D'''rugs.


Image:
=====Image=====
*[http://commons.wikimedia.org/wiki/File:Renal_arterial_hyalinosis_-_he_-_very_high_mag.jpg Arterial hyaline - HE - very high mag. (WC)].
<gallery>
*[http://commons.wikimedia.org/wiki/File:Renal_arterial_hyalinosis_-_pas_-_very_high_mag.jpg Arterial hyaline - PAS - very high mag. (WC)].
Image:Renal_arterial_hyalinosis_-_he_-_very_high_mag.jpg | Arterial hyaline - HE - very high mag. (WC/Nephron)
*[http://commons.wikimedia.org/wiki/File:Renal_arterial_hyalinosis_-_hps_-_very_high_mag.jpg Arterial hyaline - HPS - very high mag. (WC)].
Image:Renal_arterial_hyalinosis_-_pas_-_very_high_mag.jpg | Arterial hyaline - PAS - very high mag. (WC/Nephron)
Image:Renal_arterial_hyalinosis_-_hps_-_very_high_mag.jpg | Arterial hyaline - HPS - very high mag. (WC/Nephron)
</gallery>


====Atherosclerosis====
====Atherosclerosis====
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Can be:
Can be:
*Subepithelial - distal to basement membrane (BM), closer to the urinary space.
*Subepithelial - distal to basement membrane (BM), closer to the urinary space.
**[[Membranous nephropathy]], [[Post-infectious glomerulonephritis]] (hump-like), [[MPGN]].
*Subendothelial - proximal to BM, closer to the glomerular capillary.
*Subendothelial - proximal to BM, closer to the glomerular capillary.
 
**[[MPGN]] - classic location.


==Pathologic differential diagnosis==
==Pathologic differential diagnosis==
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===Mesangial expansion===
===Mesangial expansion===
*A patch of matrix can contain more than three mesangial cell nuclei.
**Highly subjective.
DDx:
DDx:
*Diabetes mellitus.<ref name=pmid17418688>{{cite journal |author=Fioretto P, Mauer M |title=Histopathology of diabetic nephropathy |journal=Semin. Nephrol. |volume=27 |issue=2 |pages=195-207 |year=2007 |month=March |pmid=17418688 |doi=10.1016/j.semnephrol.2007.01.012 |url=}}</ref>
*[[Diabetes mellitus]].<ref name=pmid17418688>{{cite journal |author=Fioretto P, Mauer M |title=Histopathology of diabetic nephropathy |journal=Semin. Nephrol. |volume=27 |issue=2 |pages=195-207 |year=2007 |month=March |pmid=17418688 |doi=10.1016/j.semnephrol.2007.01.012 |url=}}</ref>
*Immune complex mediated disease
*MPGN:
**[[IgA nephropathy]].
**[[IgA nephropathy]].
**[[Henoch-Schoenlein purpura]].
**[[Henoch-Schoenlein purpura]].
**[[Lupus nephritis]].
**[[Lupus nephritis]].
*[[Fibrillary glomerulopathy]].
*[[Immunotactoid glomerulopathy]].
*[[Renal amyloidosis]].
*Monoclonal immunoglobulin deposition disease.


===Glomerular crescents===
===Glomerular crescents===
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===Primary light microscopy patterns===
===Primary light microscopy patterns===
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
| '''Pattern'''
! Pattern
| '''Key feature'''
! Key feature
| '''Other findings'''
! Other findings
| '''DDx of the pattern'''
! DDx of the pattern
| '''DDx'''
! DDx
| '''Image'''
! Image
|-
|-
|Normal light microscopy
|Normal light microscopy
| Normal histology
| Normal histology
| none
| none ''or'' few glomeruli in a biopsy of an under sampled FSGS
| [[membranous nephropathy]], FSGS
| [[membranous nephropathy]], [[focal segmental glomerulosclerosis]] (FSGS)
| normal kidney, [[minimal change disease]], [[thin glomerular basement membrane disease]], [[Alport syndrome]], early [[membranous nephropathy]]
| normal kidney, [[minimal change disease]], [[thin glomerular basement membrane disease]], [[Alport syndrome]], early [[membranous nephropathy]]
| Image?
| [[Image:Benign kidney -- intermed mag.jpg|thumb|center|150px|Benign kidney (WC)]]
|-
|-
|Nodular mesangial expansion
|Nodular mesangial expansion
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| GBM thickening, both afferent and efferent arteriole hyalinized
| GBM thickening, both afferent and efferent arteriole hyalinized
| diffuse proliferative GN, membranous nephropathy
| diffuse proliferative GN, membranous nephropathy
| [[diabetic nephropathy]], [[idiopathic nodular glomerulosclerosis]], [[amyloidosis]], light chain deposition disease
| [[diabetic nephropathy]], [[idiopathic nodular glomerulosclerosis]], [[renal amyloidosis]], light chain deposition disease, [[fibrillary glomerulopathy]], [[immunotactoid glomerulopathy]]
| [http://commons.wikimedia.org/wiki/File:Nodular_glomerulosclerosis.jpeg (WC)]
| [[Image:Nodular_glomerulosclerosis.jpeg|thumb|center|150px|Nodular GS (WC)]]
|-
|-
|[[Membranous nephropathy]] (MN)
|[[Membranous nephropathy]] (MN)
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| normal light microscopy, light chain deposition disease
| normal light microscopy, light chain deposition disease
| [[hepatitis B]], [[hepatitis C]], carcinoma, NSAID toxicity, SLE, idiopathic (MN); light chain deposition disease ([[plasma cell neoplasm]])
| [[hepatitis B]], [[hepatitis C]], carcinoma, NSAID toxicity, SLE, idiopathic (MN); light chain deposition disease ([[plasma cell neoplasm]])
| [http://commons.wikimedia.org/wiki/File:Membranous_nephropathy_-_mpas_-_very_high_mag.jpg (WC)]  
| [[Image:Membranous_nephropathy_-_mpas_-_very_high_mag.jpg|thumb|center|150px|MN (WC)]]  
|-
|-
|[[Focal segmental glomerulosclerosis]]
|[[Focal segmental glomerulosclerosis]]
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| +/-glomerular enlargement, +/- tuft-capsule adhesions
| +/-glomerular enlargement, +/- tuft-capsule adhesions
| [[rapidly progressive glomerulonephritis]], nodular mesangial expansion
| [[rapidly progressive glomerulonephritis]], nodular mesangial expansion
| primary FSGS, secondary FSGS ([[HIV]], [[IVDU]], obesity, [[parvovirus B19]], [[Alport syndrome]]
| primary FSGS, secondary FSGS ([[HIV]], [[IVDU]], [[obesity]], [[parvovirus B19]], [[Alport syndrome]]
| [http://commons.wikimedia.org/wiki/File:Focal_segmental_glomerulosclerosis_-_high_mag.jpg (WC)]
| [[Image:Focal_segmental_glomerulosclerosis_-_high_mag.jpg|thumb|center|150px|FSGS (WC)]]
|-
|-
|Diffuse proliferative glomerulonephritis
|Diffuse proliferative glomerulonephritis
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| nodular glomerulosclerosis
| nodular glomerulosclerosis
| [[post-infectious glomerulonephritis]], [[MPGN]], [[dense deposit disease]], diffuse proliferative [[lupus nephritis]] (class IV), cryoglobulinemic GN
| [[post-infectious glomerulonephritis]], [[MPGN]], [[dense deposit disease]], diffuse proliferative [[lupus nephritis]] (class IV), cryoglobulinemic GN
| [http://commons.wikimedia.org/wiki/File:Membranoproliferative_glomerulonephritis_-_very_high_mag.jpg (WC)]
| [[Image:Membranoproliferative_glomerulonephritis_-_very_high_mag.jpg|thumb|center|150px|MPGN (WC)]]
|-  
|-  
|[[Rapidly progressive glomerulonephritis]]
|[[Rapidly progressive glomerulonephritis]]
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| [[FSGS]], diffuse proliferative glomerulonephritis
| [[FSGS]], diffuse proliferative glomerulonephritis
| [[AGBM]], [[post-infectious glomerulonephritis]], pauci-immune GN, immune complex diseases ([[lupus nephritis]], [[IgA nephropathy]], others)
| [[AGBM]], [[post-infectious glomerulonephritis]], pauci-immune GN, immune complex diseases ([[lupus nephritis]], [[IgA nephropathy]], others)
| [http://en.wikipedia.org/wiki/File:Crescentic_glomerulonephritis_%282%29.jpg (WC)]
| [[Image:Crescentic_glomerulonephritis_%282%29.jpg|thumb|center|150px|Crescentic GN (WC)]]
|-
|-
|Tubular injury
|Tubular injury
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| normal light microscopy
| normal light microscopy
| [[acute tubular necrosis]], [[chronic allograft nephropathy]]
| [[acute tubular necrosis]], [[chronic allograft nephropathy]]
| [http://commons.wikimedia.org/wiki/File:Chronic_allograft_nephropathy_-_intermed_mag.jpg (WC)]
| [[Image:Chronic_allograft_nephropathy_-_intermed_mag.jpg|thumb|center|150px|CAN (WC)]]
|-
|-
|Small vessel pathology
|Small vessel pathology
Line 444: Line 513:
| none
| none
| [[thrombotic microangiopathy]] (malignant hypertension, [[scleroderma]] renal crisis, DIC, HUS, TTP,
| [[thrombotic microangiopathy]] (malignant hypertension, [[scleroderma]] renal crisis, DIC, HUS, TTP,
| [http://commons.wikimedia.org/wiki/File:Renal_arterial_hyalinosis_-_pas_-_very_high_mag.jpg hyalinosis (WC)], [http://commons.wikimedia.org/wiki/File:Thrombotic_microangiopathy_-_very_high_mag.jpg TMA (WC)]
| [[Image:Renal_arterial_hyalinosis_-_pas_-_very_high_mag.jpg |thumb|center|150px| Hyalinosis (WC)]] [[Image:Thrombotic_microangiopathy_-_very_high_mag.jpg|thumb|center|150px| TMA (WC)]]
|-
|-
|Large vessel pathology
|Large vessel pathology
Line 451: Line 520:
| none
| none
| [[atherosclerosis]], [[cholesterol embolism]], other types of embolism, [[thrombosis]]
| [[atherosclerosis]], [[cholesterol embolism]], other types of embolism, [[thrombosis]]
| [http://commons.wikimedia.org/wiki/File:Cholesterol_embolus_-_intermed_mag.jpg (WC)]
| [[Image:Cholesterol_embolus_-_intermed_mag.jpg |thumb|center|150px| Cholesterol embolus (WC)]]
|- <!--
|- <!--
| Pattern
| Pattern
Line 463: Line 532:
===Diagnoses - Table===
===Diagnoses - Table===
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
| '''Pattern'''
! Pattern
| '''Key feature'''
! Key feature
| '''Other findings'''
! Other findings
| '''IF & EM'''
! IF & EM
| '''Presentation'''
! Presentation
| '''Clinical'''
! Clinical
| '''Pathol. DDx'''
! Pathol. DDx
| '''Image'''
! Image
|-
|-
|[[Nodular glomerulosclerosis]]
|[[Nodular glomerulosclerosis]]
Line 476: Line 545:
| GBM thickening, both afferent and efferent arteriole hyalinized
| GBM thickening, both afferent and efferent arteriole hyalinized
| EM?
| EM?
| nephrotic (???)
| proteinuria - typical presentation, nephrotic syndrome{{fact}}
| [[diabetes mellitus]] (DM)
| [[diabetes mellitus]] (DM)
| [[amyloidosis]], [[idiopathic nodular glomerulosclerosis]] (nodular GS without DM)
| [[amyloidosis]], [[idiopathic nodular glomerulosclerosis]] (nodular GS without DM)
| [http://commons.wikimedia.org/wiki/File:Nodular_glomerulosclerosis.jpeg (WC)]
| [[Image:Nodular_glomerulosclerosis.jpeg|thumb|center|150px|Nodular GS (WC)]]
|-
|-
|[[Focal segmental glomerulosclerosis]] (FSGS)
|[[Focal segmental glomerulosclerosis]] (FSGS)
Line 488: Line 557:
| primary FSGS, secondary FSGS ([[HIV]], [[IVDU]], obesity, [[parvovirus B19]], [[Alport syndrome]]); unresponsive to steroids, worse prognosis than MCD
| primary FSGS, secondary FSGS ([[HIV]], [[IVDU]], obesity, [[parvovirus B19]], [[Alport syndrome]]); unresponsive to steroids, worse prognosis than MCD
| [[minimal change disease]]
| [[minimal change disease]]
| [http://commons.wikimedia.org/wiki/File:Focal_segmental_glomerulosclerosis_-_high_mag.jpg (WC)]
| [[Image:Focal_segmental_glomerulosclerosis_-_high_mag.jpg|thumb|center|150px|FSGS (WC)]]
|-
|-
|[[Membranous nephropathy]]<br>(AKA membranous GN)
|[[Membranous nephropathy]]<br>(AKA membranous GN)
Line 497: Line 566:
| [[hepatitis B]], [[hepatitis C]], carcinoma, NSAID toxicity, SLE, idiopathic
| [[hepatitis B]], [[hepatitis C]], carcinoma, NSAID toxicity, SLE, idiopathic
| Nodular glomerulosclerosis (?)
| Nodular glomerulosclerosis (?)
| [http://www.flickr.com/photos/jian-hua_qiao_md/3989875091/in/set-72157622411941607 silver stain (flickr.com)]
| [[Image:Membranous_nephropathy_-_mpas_-_very_high_mag.jpg|thumb|center|150px|MN (WC)]]  
|-
|-
|[[Minimal change disease]] (MCD)
|[[Minimal change disease]] (MCD)
Line 515: Line 584:
| primary vs. secondary (Henoch-Schoenlein purpura)
| primary vs. secondary (Henoch-Schoenlein purpura)
| [[RPGN]]
| [[RPGN]]
| [http://commons.wikimedia.org/wiki/File:Henoch-Sch%C3%B6nlein_nephritis_IgA_immunostaining.jpg (WC)]
| [[Image:Henoch-Sch%C3%B6nlein_nephritis_IgA_immunostaining.jpg|thumb|center|150px|HSN - IgA IHC (WC)]]
|-
|-
|[[Membranoproliferative glomerulonephritis]] (MPGN)
|[[Membranoproliferative glomerulonephritis]] (MPGN)
Line 522: Line 591:
| subepithelial deposits
| subepithelial deposits
| mixed nephrotic/nephritic
| mixed nephrotic/nephritic
| [[SLE]], cryoglobulinemia, [[hepatitis B]], [[hepatitis C]]
| [[SLE]], [[cryoglobulinemia]], [[hepatitis B]], [[hepatitis C]]
| Pathol. DDx?
| Pathol. DDx?
| [http://commons.wikimedia.org/wiki/File:Membranoproliferative_glomerulonephritis_-_very_high_mag.jpg (WC)]
| [[Image:Membranoproliferative_glomerulonephritis_-_very_high_mag.jpg|thumb|center|MPGN (WC)]]
|-
|-
|[[Focal proliferative glomerosclerosis|Focal proliferative<br> glomerosclerosis]] (FPGS)
|[[Focal proliferative glomerosclerosis|Focal proliferative<br> glomerosclerosis]] (FPGS)
Line 545: Line 614:
|-  
|-  
| [[Dense deposit disease]]
| [[Dense deposit disease]]
| linear C3 with rings
| IF: linear C3 with rings
| +/-thick GBM
| +/-thick GBM
| EM: GBM lamina densa thickening
| EM: GBM lamina densa thickening
Line 565: Line 634:
===Diffuse proliferative glomerulonephritis===
===Diffuse proliferative glomerulonephritis===
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
| '''Pattern'''
! Pattern
| '''Key feature'''
! Key feature
| '''Clinical'''
! Clinical
|-
|-
| [[Post-infectious glomerulonephritis]]
| [[Post-infectious glomerulonephritis]]
Line 574: Line 643:
|-
|-
| [[Membranoproliferative glomerulonephritis]] (MPGN)
| [[Membranoproliferative glomerulonephritis]] (MPGN)
|
| mesangial proliferation with thickening of the glomerular capillary loops<ref name=pmid11682680>{{Cite journal  | last1 = Smet | first1 = AD. | last2 = Kuypers | first2 = D. | last3 = Evenepoel | first3 = P. | last4 = Maes | first4 = B. | last5 = Messiaen | first5 = T. | last6 = Van Damme | first6 = B. | last7 = Vanrenterghem | first7 = Y. | title = 'Full house' positive immunohistochemical membranoproliferative glomerulonephritis in a patient with portosystemic shunt. | journal = Nephrol Dial Transplant | volume = 16 | issue = 11 | pages = 2258-62 | month = Nov | year = 2001 | doi =  | PMID = 11682680 | URL = http://ndt.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=11682680 }}</ref>
 
| low C3, normal C4; primary vs. secondary (often hepatitis C)
| low C3, normal C4; primary vs. secondary (often hepatitis C)
|-
|-
| [[Dense deposit disease]]
| [[Dense deposit disease]]
|
| IF: linear C3 with rings
|
|  
|-
|-
| [[Cryoglobulinemic glomerulonephritis]]
| [[Cryoglobulinemic glomerulonephritis]]
Line 600: Line 670:
*Due to [[diabetes mellitus]] - '''key feature'''.
*Due to [[diabetes mellitus]] - '''key feature'''.
**If there is no history of diabetes... it is ''[[idiopathic nodular glomerulosclerosis]]''.
**If there is no history of diabetes... it is ''[[idiopathic nodular glomerulosclerosis]]''.
*Most common cause of end stage renal disease (ESRD).
*Most common cause of [[end-stage renal disease]] (ESRD).
*Biopsied only if the (clinical) features are atypical.
*Biopsied only if the (clinical) features are atypical.


Line 609: Line 679:
*Mesangial matrix expansion - leads to nodule formation ''Kimmelstiel-Wilson nodules'' (''nodular glomerulosclerosis'').
*Mesangial matrix expansion - leads to nodule formation ''Kimmelstiel-Wilson nodules'' (''nodular glomerulosclerosis'').


Other:
Others:
*Armanni-Ebstein change - cytoplasmic vacuolization of tubular cells (usu. loop of Henle) -- innermost cortex, outer medulla;<ref name=pmid13478656>{{cite journal |author=RITCHIE S, WAUGH D |title=The pathology of Armanni-Ebstein diabetic nephropathy |journal=Am. J. Pathol. |volume=33 |issue=6 |pages=1035–57 |year=1957 |pmid=13478656 |pmc=1934668 |doi= |url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1934668/?page=1}}</ref> not specific to diabetes mellitus.<ref name=pmid20875709>{{cite journal |author=Zhou C, Byard RW |title=Armanni-Ebstein phenomenon and hypothermia |journal=Forensic Sci Int |volume= |issue= |pages= |year=2010 |month=September |pmid=20875709 |doi=10.1016/j.forsciint.2010.08.018 |url=}}</ref>
*Armanni-Ebstein change = cytoplasmic vacuolization of tubular cells (usu. loop of Henle) -- innermost cortex, outer medulla;<ref name=pmid13478656>{{cite journal |author=Ritchie S, Waugh D |title=The pathology of Armanni-Ebstein diabetic nephropathy |journal=Am. J. Pathol. |volume=33 |issue=6 |pages=1035–57 |year=1957 |pmid=13478656 |pmc=1934668 |doi= |url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1934668/?page=1}}</ref> not specific to diabetes mellitus.<ref name=pmid20875709>{{cite journal |author=Zhou C, Byard RW |title=Armanni-Ebstein phenomenon and hypothermia |journal=Forensic Sci Int |volume= |issue= |pages= |year=2010 |month=September |pmid=20875709 |doi=10.1016/j.forsciint.2010.08.018 |url=}}</ref>
*Capsular drop = hyaline material deposited in the Bowman's capsule.<ref name=ndt-edu>URL: [http://www.ndt-educational.org/ferrariodiabete.asp http://www.ndt-educational.org/ferrariodiabete.asp]. Accessed on: 29 April 2012.</ref>
*Fibrin cap = subendothelial deposition of hyaline material.<ref name=ndt-edu/>


Other - with weak evidence:
Other - with weak evidence:
Line 616: Line 688:


Memory device:
Memory device:
*GBM = ''thick '''G'''BM'', '''''b'''oth afferent & efferent artiole thickened'', '''''m'''esangial matrix expansion''.
*GBM = ''thick '''G'''BM'', '''''b'''oth afferent & efferent arterioles thickened'', '''''m'''esangial matrix expansion''.


Images:  
====Images====
*[http://commons.wikimedia.org/wiki/File:Nodular_glomerulosclerosis.jpeg Nodular glomerulosclerosis (WC)].
<gallery>
Image:Diabetic_glomerulosclerosis_%281%29_HE.jpg | Diabetic glomerulosclerosis. (WC/KGH)
Image:Diabetic_glomerulosclerosis_(2)_HE.jpg | Diabetic glomerulosclerosis. (WC/KGH)
Image:Diabetic_glomerulosclerosis_%283%29_HE.jpg | Diabetic glomerulosclerosis. (WC/KGH)
Image:Nodular_glomerulosclerosis.jpeg | Nodular glomerulosclerosis. (WC)
</gallery>
www:
*[http://library.med.utah.edu/WebPath/jpeg1/RENAL027.jpg Nodular GS (med.utah.edu)].
*[http://library.med.utah.edu/WebPath/jpeg1/RENAL027.jpg Nodular GS (med.utah.edu)].
*[http://www.markwickmd.com/images/pages/armani_ebstein_lesion.jpg Armanni-Ebstein lesion (markwickmd.com)].
*[http://www.markwickmd.com/images/pages/armani_ebstein_lesion.jpg Armanni-Ebstein lesion (markwickmd.com)].
Line 682: Line 760:
**Class IV is essentially a dead kidney - doesn't get biopsied.
**Class IV is essentially a dead kidney - doesn't get biopsied.


Images:
====Images====
*[http://commons.wikimedia.org/wiki/File:SLE_Nephritis_Pathology_Diagram.svg SLE nephritis - schematic (WC)].
<gallery>
Image:SLE_Nephritis_Pathology_Diagram.svg| SLE nephritis - schematic. (WC)
</gallery>
*Membranous lupus:
*Membranous lupus:
**[http://www.flickr.com/photos/jian-hua_qiao_md/3989875425/in/set-72157622411941607 H&E (flickr.com)].
**[http://www.flickr.com/photos/jian-hua_qiao_md/3989875425/in/set-72157622411941607 H&E (flickr.com)].
Line 691: Line 771:
=Nephrotic syndrome=
=Nephrotic syndrome=
{{Main|Nephrotic syndrome}}
{{Main|Nephrotic syndrome}}
This includes the following:
This classically includes the following:
*[[Minimal change disease]].
*[[Minimal change disease]].
*[[Focal segmental glomerulosclerosis]].
*[[Focal segmental glomerulosclerosis]].
*[[Membranous nephropathy]].
*[[Membranous nephropathy]].
It also includes:
*[[IgA nephropathy]].
*Pre-eclampsia - uncommon.<ref name=pmid20033418>{{Cite journal  | last1 = Wei | first1 = Q. | last2 = Zhang | first2 = L. | last3 = Liu | first3 = X. | title = Outcome of severe preeclampsia manifested as nephrotic syndrome. | journal = Arch Gynecol Obstet | volume = 283 | issue = 2 | pages = 201-4 | month = Feb | year = 2011 | doi = 10.1007/s00404-009-1338-z | PMID = 20033418 }}</ref>


=Mixed nephrotic and nephritic=
=Mixed nephrotic and nephritic=
Line 704: Line 788:
*Associated with an increased incidence of [[Celiac disease]].<ref name=pmid19332868>{{Cite journal  | last1 = Smerud | first1 = HK. | last2 = Fellström | first2 = B. | last3 = Hällgren | first3 = R. | last4 = Osagie | first4 = S. | last5 = Venge | first5 = P. | last6 = Kristjánsson | first6 = G. | title = Gluten sensitivity in patients with IgA nephropathy. | journal = Nephrol Dial Transplant | volume = 24 | issue = 8 | pages = 2476-81 | month = Aug | year = 2009 | doi = 10.1093/ndt/gfp133 | PMID = 19332868 }}</ref>  
*Associated with an increased incidence of [[Celiac disease]].<ref name=pmid19332868>{{Cite journal  | last1 = Smerud | first1 = HK. | last2 = Fellström | first2 = B. | last3 = Hällgren | first3 = R. | last4 = Osagie | first4 = S. | last5 = Venge | first5 = P. | last6 = Kristjánsson | first6 = G. | title = Gluten sensitivity in patients with IgA nephropathy. | journal = Nephrol Dial Transplant | volume = 24 | issue = 8 | pages = 2476-81 | month = Aug | year = 2009 | doi = 10.1093/ndt/gfp133 | PMID = 19332868 }}</ref>  
*''IgA nephropathy'', in children, with a skin rash ([[leukocytoclastic vasculitis]]) = [[Henoch–Schönlein purpura]].
*''IgA nephropathy'', in children, with a skin rash ([[leukocytoclastic vasculitis]]) = [[Henoch–Schönlein purpura]].
*May present as [[nephrotic syndrome]].<ref name=pmid7849391>{{Cite journal  | last1 = Komatsuda | first1 = A. | last2 = Wakui | first2 = H. | last3 = Yasuda | first3 = T. | last4 = Imai | first4 = H. | last5 = Miura | first5 = AB. | last6 = Tsuda | first6 = A. | last7 = Nakamoto | first7 = Y. | title = Successful delivery in a pregnant women with crescentic IgA nephropathy. | journal = Intern Med | volume = 33 | issue = 11 | pages = 723-6 | month = Nov | year = 1994 | doi =  | PMID = 7849391 }}</ref><ref name=pmid22322610>{{Cite journal  | last1 = Kim | first1 = JK. | last2 = Kim | first2 = JH. | last3 = Lee | first3 = SC. | last4 = Kang | first4 = EW. | last5 = Chang | first5 = TI. | last6 = Moon | first6 = SJ. | last7 = Yoon | first7 = SY. | last8 = Yoo | first8 = TH. | last9 = Kang | first9 = SW. | title = Clinical features and outcomes of IgA nephropathy with nephrotic syndrome. | journal = Clin J Am Soc Nephrol | volume = 7 | issue = 3 | pages = 427-36 | month = Mar | year = 2012 | doi = 10.2215/CJN.04820511 | PMID = 22322610 }}</ref>
*May present as isolated hematuria.<ref name=pmid19949735>{{Cite journal  | last1 = Kim | first1 = BS. | last2 = Kim | first2 = YK. | last3 = Shin | first3 = YS. | last4 = Kim | first4 = YO. | last5 = Song | first5 = HC. | last6 = Kim | first6 = YS. | last7 = Choi | first7 = EJ. | title = Natural history and renal pathology in patients with isolated microscopic hematuria. | journal = Korean J Intern Med | volume = 24 | issue = 4 | pages = 356-61 | month = Dec | year = 2009 | doi = 10.3904/kjim.2009.24.4.356 | PMID = 19949735 }}</ref>


===Microscopic===
===Microscopic===
Line 713: Line 799:
*Diagnosis based on immunofluorescence (IgA+).
*Diagnosis based on immunofluorescence (IgA+).


Images:
====Images====
*[http://library.med.utah.edu/WebPath/jpeg2/RENAL096.jpg IgA nephropathy (med.utah.edu)].
*[http://library.med.utah.edu/WebPath/jpeg2/RENAL096.jpg IgA nephropathy (med.utah.edu)].
*[http://path.upmc.edu/cases/case96.html HSP (upmc.edu)].
*[http://path.upmc.edu/cases/case96.html HSP (upmc.edu)].
 
<gallery>
Image:Henoch-Sch%C3%B6nlein_nephritis_IgA_immunostaining.jpg | HSP - IgA IHC (WC)
</gallery>
====Scoring====
====Scoring====
IgA nephropathy can be scored using an assessment of '''m'''esangial proliferation, '''e'''ndocapillary proliferation, glomerulo'''s'''clerosis and '''t'''ubular atrophy and interstitial fibrosis (abbreviated ''MEST'').<ref>{{Cite journal  | last1 = Coppo | first1 = R. | last2 = Cattran | first2 = D. | last3 = Roberts Ian | first3 = SD. | last4 = Troyanov | first4 = S. | last5 = Camilla | first5 = R. | last6 = Cook | first6 = T. | last7 = Feehally | first7 = J. | title = The new Oxford Clinico-Pathological Classification of IgA nephropathy. | journal = Prilozi | volume = 31 | issue = 1 | pages = 241-8 | month = Jul | year = 2010 | doi =  | PMID = 20693944 }}</ref>
IgA nephropathy can be scored using an assessment of '''m'''esangial proliferation, '''e'''ndocapillary proliferation, glomerulo'''s'''clerosis and '''t'''ubular atrophy and interstitial fibrosis (abbreviated ''MEST'').<ref>{{Cite journal  | last1 = Coppo | first1 = R. | last2 = Cattran | first2 = D. | last3 = Roberts Ian | first3 = SD. | last4 = Troyanov | first4 = S. | last5 = Camilla | first5 = R. | last6 = Cook | first6 = T. | last7 = Feehally | first7 = J. | title = The new Oxford Clinico-Pathological Classification of IgA nephropathy. | journal = Prilozi | volume = 31 | issue = 1 | pages = 241-8 | month = Jul | year = 2010 | doi =  | PMID = 20693944 }}</ref>


===IF===
===IF===
*IgA +ve -- branching pattern.
*IgA +ve in a branching pattern - '''diagnostic'''.


===EM===
===EM===
Line 731: Line 819:
*Old name ''MPGN type 1''.
*Old name ''MPGN type 1''.
===General===
===General===
Clinical:
*Nephrotic syndrome or nephrotic/nephritic syndrome.
Pathology:
*May be primary, i.e. idiopathic, or secondary, i.e. a consequence of another pathologic process.<ref name=pmid11682680>{{Cite journal  | last1 = Smet | first1 = AD. | last2 = Kuypers | first2 = D. | last3 = Evenepoel | first3 = P. | last4 = Maes | first4 = B. | last5 = Messiaen | first5 = T. | last6 = Van Damme | first6 = B. | last7 = Vanrenterghem | first7 = Y. | title = 'Full house' positive immunohistochemical membranoproliferative glomerulonephritis in a patient with portosystemic shunt. | journal = Nephrol Dial Transplant | volume = 16 | issue = 11 | pages = 2258-62 | month = Nov | year = 2001 | doi =  | PMID = 11682680 | URL = http://ndt.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=11682680 }}</ref>
====Secondary causes====
*In adults most common cause: ''[[hepatitis C]]''.
*In adults most common cause: ''[[hepatitis C]]''.


Other causes:
Other causes:
*[[HIV]],<ref name=pmid21743226>{{Cite journal  | last1 = Pakasa | first1 = NM. | last2 = Binda | first2 = PM. | title = HIV-associated immune complex glomerulonephritis with lupus-like features. | journal = Saudi J Kidney Dis Transpl | volume = 22 | issue = 4 | pages = 769-73 | month = Jul | year = 2011 | doi =  | PMID = 21743226 }}</ref> [[SLE]], cryoglobulinemia, [[hepatitis B]].
*[[HIV]].<ref name=pmid21743226>{{Cite journal  | last1 = Pakasa | first1 = NM. | last2 = Binda | first2 = PM. | title = HIV-associated immune complex glomerulonephritis with lupus-like features. | journal = Saudi J Kidney Dis Transpl | volume = 22 | issue = 4 | pages = 769-73 | month = Jul | year = 2011 | doi =  | PMID = 21743226 }}</ref>  
*[[SLE]] - usu. has "full house" on IF.<ref name=pmid11682680/>
*Cryoglobulinemia.
*[[Hepatitis B]].
*Portocaval shunt.<ref name=pmid21743226/>


Clinical:
*Nephrotic syndrome or nephrotic/nephritic syndrome.
===Microscopic===
===Microscopic===
Features:
Features:
Line 747: Line 844:
*[[Nodular glomerulosclerosis]].
*[[Nodular glomerulosclerosis]].


Images:
====Images====
*[http://commons.wikimedia.org/wiki/File:Membranoproliferative_glomerulonephritis_-_intermed_mag.jpg MPGN - intermed. mag. (WC)].
<gallery>
*[http://commons.wikimedia.org/wiki/File:Membranoproliferative_glomerulonephritis_-_very_high_mag.jpg MPGN - very high mag. (WC)].
Image:Membranoproliferative_glomerulonephritis_-_intermed_mag.jpg | MPGN - intermed. mag. (WC/Nephron)
Image:Membranoproliferative_glomerulonephritis_-_very_high_mag.jpg | MPGN - very high mag. (WC/Nephron)
</gallery>
*[http://path.upmc.edu/cases/case593.html MPGN - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case593.html MPGN - several images (upmc.edu)].
===EM===
Features:
*Subendothelial immune deposits - classic finding.<ref name=pmid21839367>{{Cite journal  | last1 = Sethi | first1 = S. | last2 = Fervenza | first2 = FC. | title = Membranoproliferative glomerulonephritis: pathogenetic heterogeneity and proposal for a new classification. | journal = Semin Nephrol | volume = 31 | issue = 4 | pages = 341-8 | month = Jul | year = 2011 | doi = 10.1016/j.semnephrol.2011.06.005 | PMID = 21839367 }}</ref>
*Subepithelial immune deposits - historically considered uncommon.<ref name=pmid2263028>{{Cite journal  | last1 = Sato | first1 = H. | title = [Ultrastructural study on membranoproliferative glomerulonephritis with special reference to subepithelial deposits]. | journal = Nihon Jinzo Gakkai Shi | volume = 32 | issue = 9 | pages = 973-83 | month = Sep | year = 1990 | doi =  | PMID = 2263028 }}</ref>


==Dense deposit disease==
==Dense deposit disease==
Line 812: Line 916:
*+/-[[Vasculitis]].
*+/-[[Vasculitis]].


Images:
====Images====
*WC - KGH:
<gallery>
**[http://commons.wikimedia.org/wiki/File:Crescentic_glomerulonephritis_%282%29.jpg RPGN (WC)].
Image:Crescentic_glomerulonephritis_%282%29.jpg | RPGN (WC/KGH)  
**[http://commons.wikimedia.org/wiki/File:Crescentic_glomerulonephritis_%281%29.jpg RPGN (WC)].
Image:Crescentic_glomerulonephritis_%281%29.jpg | RPGN (WC/KGH)
*WC:
Image:Crescentic_glomerulonephritis_-_intermed_mag.jpg | RPGN - intermed. mag. (WC/Nephron)
**[http://commons.wikimedia.org/wiki/File:Crescentic_glomerulonephritis_-_intermed_mag.jpg RPGN - intermed. mag. (WC)].
Image:Crescentic_glomerulonephritis_-_high_mag.jpg | RPGN - high mag. (WC/Nephron)
**[http://commons.wikimedia.org/wiki/File:Crescentic_glomerulonephritis_-_high_mag.jpg RPGN - high mag. (WC)].
</gallery>
 
==Post-infectious glomerulonephritis==
==Post-infectious glomerulonephritis==
===General===
*Abbreviated ''PIGN''.
*Classically post-streptococcal infection.
{{Main|Post-infectious glomerulonephritis}}
**Lab test: Antistreptolysin O titer (ASOT) +ve.
 
===Microscopic===
Features:
*+/-Neutrophils - in glomerulus.
*[[Glomerular crescents]].
 
Images:
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Post-infectious_glomerulonephritis_-_high_mag.jpg Post-infectious GN - high mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Post-infectious_glomerulonephritis_-_very_high_mag.jpg Post-infectious GN - very high mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Acute_Glomerulonephritis_Pathology_Diagram.svg Post-infectious GN - schematic (WC)].
*www:
**[http://path.upmc.edu/cases/case671.html Post-infectious glomerulonephritis - several images (upmc.edu)].
 
===IF===
*Granular immune deposits.
 
===EM===
*Subepithelial deposits.


=Rare diseases=
=Rare diseases=
Line 854: Line 937:
**Associated with a specific HLA type (HLA-DRB1*1501) and two gene families.
**Associated with a specific HLA type (HLA-DRB1*1501) and two gene families.
**Antibodies directed against COL4A3,<ref>{{OMIM|120070}}</ref> which is ''not'' mutated.
**Antibodies directed against COL4A3,<ref>{{OMIM|120070}}</ref> which is ''not'' mutated.
*Oligouria - poor prognosticator.{{fact}}


Tx:
Tx:
Line 867: Line 951:
**Crescentic glomerulonephritis.
**Crescentic glomerulonephritis.


Images:
====Images====
*[[WC]]:
<gallery>
**[http://commons.wikimedia.org/wiki/File:Crescentic_glomerulonephritis_-_intermed_mag.jpg RPGN - intermed. mag. (WC)].
Image:Crescentic_glomerulonephritis_-_intermed_mag.jpg | RPGN - intermed. mag. (WC/Nephron)
**[http://commons.wikimedia.org/wiki/File:Crescentic_glomerulonephritis_-_high_mag.jpg RPGN - high mag. (WC)].
Image:Crescentic_glomerulonephritis_-_high_mag.jpg | RPGN - high mag. (WC/Nephron)
*www:
</gallery>
**[http://path.upmc.edu/cases/case541.html AGBM disease - several images (upmc.edu)].
www:
*[http://path.upmc.edu/cases/case541.html AGBM disease - several images (upmc.edu)].


===IF===
===IF===
*Linear IgG deposits - '''diagnostic'''.
*Linear IgG deposits - '''diagnostic'''.
*Fibrinogen in crescents.


DDx:
DDx:
Line 923: Line 1,009:
===Microscopic===
===Microscopic===
Features:<ref name=pmid9727383/>
Features:<ref name=pmid9727383/>
*Normal.
*Usu. normal.
*+/-Foamy appearing renal tubular cells.<ref name=Ref_Klatt246>{{Ref Klatt|246}}</ref>


===IF===
===IF===
Line 952: Line 1,039:


==Fabry disease==
==Fabry disease==
===General===
{{Main|Fabry disease}}
*Rare X-linked genetic disease.
**Caused by defect in ''alpha-galactosidase A gene'' (''GLA gene'').<ref name=omim301500>{{OMIM|301500}}</ref>
**Women partially affected
*[[Lysosomal storage disorder]] - 2nd in prevalence only to [[Gaucher disease]].
*Multisystem disease affecting small vessels and [[kidney]].
 
====Presentation====
*Women: usually proteinuria.
*Men: [[angiokeratoma]]s, proteinuria.
 
====Tx====
*Symptomatic treatment.
*Enzyme replacement - ''agalsidase alpha'' (Replagal) or ''agalsidase beta'' (Fabrazyme).
 
===Microscopic===
Features:<ref name=pmid16799480>{{cite journal |author=Fischer EG, Moore MJ, Lager DJ |title=Fabry disease: a morphologic study of 11 cases |journal=Mod. Pathol. |volume=19 |issue=10 |pages=1295-301 |year=2006 |month=October |pmid=16799480 |doi=10.1038/modpathol.3800634 |url=http://www.nature.com/modpathol/journal/v19/n10/abs/3800634a.html}}</ref>
*Foamy podocyte inclusions, best visualized with ''[[toluidine blue]]''.
*Mild mesangial hypercellularity.
 
Images:
*[http://path.upmc.edu/cases/case137/micro.html Fabry disease (upmc.edu)].
*[http://path.upmc.edu/cases/case610.html Fabry disease - several images (upmc.edu)].
 
===EM===
Features:<ref name=pmid16799480/>
*Myelin-like inclusions.
**Concentric bodies with an onion-skin-like appearance.
*Zebra bodies.
**Ovoid inclusions with striped pattern.
 
Note:
*Myelin-like inclusion are not [http://en.wikipedia.org/wiki/Pathognomonic pathognomonic] for Fabry disease; they may result from drug use:<ref name=pmid16799480/>
**Amiodarone.
**Aminoglycosides.
**Chloroquine.


==Myeloma==
==Myeloma==
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====Microscopic====
====Microscopic====
Features:<ref>URL: [http://www.kidneypathology.com/English_version/Amyloidosis_and_others.html http://www.kidneypathology.com/English_version/Amyloidosis_and_others.html]. Accessed on: 9 November 2010.</ref>
Features:<ref>URL: [http://www.kidneypathology.com/English_version/Amyloidosis_and_others.html http://www.kidneypathology.com/English_version/Amyloidosis_and_others.html]. Accessed on: 9 November 2010.</ref>
*Crap in tubules.
*Crap in tubules, eosinophilic.
**Classically angulated.
**Refractile.
**Refractile.
*Cast with cellular reaction.
*Cast with cellular reaction - '''virtually diagnostic'''.
**Macrophages (CD68 +ve).
**Macrophages (CD68 +ve).


Images:
=====Images=====
*www:  
www:  
**[http://www.kidneypathology.com/Imagenes/Amiloidosis%20y/MM.3.w.jpg Cast nephropathy in myeloma (kidneypathology.com)].
*[http://www.kidneypathology.com/Imagenes/Amiloidosis%20y/MM.3.w.jpg Cast nephropathy in myeloma (kidneypathology.com)].
**[http://www.kidneypathology.com/Imagenes/Amiloidosis%20y/MM.5.w.jpg Cast nephropathy in myeloma - refractile crap (kidneypathology.com)].
*[http://www.kidneypathology.com/Imagenes/Amiloidosis%20y/MM.5.w.jpg Cast nephropathy in myeloma - refractile crap (kidneypathology.com)].
*[[WC]]:
<gallery>
**[http://commons.wikimedia.org/wiki/File:Cast_nephropathy_-_high_mag.jpg Myeloma cast nephropathy - high mag. (WC)].
Image:Cast_nephropathy_-_high_mag.jpg | Myeloma cast nephropathy - high mag. (WC/Nephron)
**[http://commons.wikimedia.org/wiki/File:Cast_nephropathy_-_2_cropped_-_very_high_mag.jpg Myeloma cast nephropathy - cropped - very high mag. (WC)].
Image:Cast_nephropathy_-_2_cropped_-_very_high_mag.jpg | Myeloma cast nephropathy - cropped - very high mag. (WC/Nephron)
 
</gallery>
====Stains====
====Stains====
*Myeloma casts = PAS -ve.
*Myeloma casts = PAS -ve.
Line 1,022: Line 1,075:
===Light chain deposition===
===Light chain deposition===
*Usually associated with kappa clone.
*Usually associated with kappa clone.
==Immunotactoid glomerulopathy==
===General===
*Uncommon.
*Thought to be a primary glomerulopathy.
**Must exclude [[lupus nephritis]], paraproteinemias, [[fibrillary glomerulopathy]], cryoglobulinemia, [[renal amyloidosis]].
*"Not universally" considered distinct from [[fibrillary glomerulopathy]].<ref name=pmid18045849>{{Cite journal  | last1 = Alpers | first1 = CE. | last2 = Kowalewska | first2 = J. | title = Fibrillary glomerulonephritis and immunotactoid glomerulopathy. | journal = J Am Soc Nephrol | volume = 19 | issue = 1 | pages = 34-7 | month = Jan | year = 2008 | doi = 10.1681/ASN.2007070757 | PMID = 18045849 | URL = http://jasn.asnjournals.org/content/19/1/34.full }}</ref>
Presentation:
*Nephrotic range proteinuria ~ 60% of cases.<ref name=pmid1996564>{{Cite journal  | last1 = Korbet | first1 = SM. | last2 = Schwartz | first2 = MM. | last3 = Lewis | first3 = EJ. | title = Immunotactoid glomerulopathy. | journal = Am J Kidney Dis | volume = 17 | issue = 3 | pages = 247-57 | month = Mar | year = 1991 | doi =  | PMID = 1996564 }}</ref>
*[[Hypertension]].
===Microscopic===
Features:
*Mesangial expansion.
DDx:
*Other causes of mesangial expansion.
===IHC===
*[[Congo red stain]] -ve.


==Fibrillary glomerulonephritis==
==Fibrillary glomerulonephritis==
*[[AKA]] ''fibrillary glomerulopathy''.
*[[AKA]] ''fibrillary glomerulopathy''.
===General===
===General===
*Rare ~ 1% native kidney biopsies.<ref name=pmid18045849>{{Cite journal  | last1 = Alpers | first1 = CE. | last2 = Kowalewska | first2 = J. | title = Fibrillary glomerulonephritis and immunotactoid glomerulopathy. | journal = J Am Soc Nephrol | volume = 19 | issue = 1 | pages = 34-7 | month = Jan | year = 2008 | doi = 10.1681/ASN.2007070757 | PMID = 18045849 | URL = http://jasn.asnjournals.org/content/19/1/34.long }}</ref>
*Rare ~ 1% native kidney biopsies.<ref name=pmid18045849>{{Cite journal  | last1 = Alpers | first1 = CE. | last2 = Kowalewska | first2 = J. | title = Fibrillary glomerulonephritis and immunotactoid glomerulopathy. | journal = J Am Soc Nephrol | volume = 19 | issue = 1 | pages = 34-7 | month = Jan | year = 2008 | doi = 10.1681/ASN.2007070757 | PMID = 18045849 | URL = http://jasn.asnjournals.org/content/19/1/34.full }}</ref>
*Presents as [[nephrotic syndrome]].
*Presents as [[nephrotic syndrome]].
*Prognosis poor; large number progress to ESRD.
*Prognosis poor; large number progress to ESRD.
Line 1,054: Line 1,128:


Note:
Note:
*[[Amyloid]] between 8 and 15 nm (diameter), most often 8-12 nm.<ref name=pmid18045849>{{Cite journal  | last1 = Alpers | first1 = CE. | last2 = Kowalewska | first2 = J. | title = Fibrillary glomerulonephritis and immunotactoid glomerulopathy. | journal = J Am Soc Nephrol | volume = 19 | issue = 1 | pages = 34-7 | month = Jan | year = 2008 | doi = 10.1681/ASN.2007070757 | PMID = 18045849 | URL = http://jasn.asnjournals.org/content/19/1/34.long }}</ref>
*[[Amyloid]] between 8 and 15 nm (diameter), most often 8-12 nm.<ref name=pmid18045849>{{Cite journal  | last1 = Alpers | first1 = CE. | last2 = Kowalewska | first2 = J. | title = Fibrillary glomerulonephritis and immunotactoid glomerulopathy. | journal = J Am Soc Nephrol | volume = 19 | issue = 1 | pages = 34-7 | month = Jan | year = 2008 | doi = 10.1681/ASN.2007070757 | PMID = 18045849 | URL = http://jasn.asnjournals.org/content/19/1/34.full }}</ref>


==Aristolochic acid nephropathy==
==Aristolochic acid nephropathy==
Line 1,084: Line 1,158:
{{Main|Cystic kidney diseases}}
{{Main|Cystic kidney diseases}}
These are discussed in a separate article and include:
These are discussed in a separate article and include:
*Autosomal dominant polycystic kidney disease (ADPKD).
*[[Autosomal dominant polycystic kidney disease]] (ADPKD).
*Adult-onset medullary cystic disease.
*Adult-onset medullary cystic disease.
*Acquired renal cystic disease.
*[[Acquired renal cystic disease]].
*Autosomal recessive polycystic kidney disease (ARPKD).
*[[Autosomal recessive polycystic kidney disease]] (ARPKD).
*Medullary sponge kidney.
*Medullary sponge kidney.
*Nephronophthisis.
*Nephronophthisis.
Line 1,104: Line 1,178:
*[[AKA]] ''diffuse suppurative nephritis''.
*[[AKA]] ''diffuse suppurative nephritis''.
===General===
===General===
*Typically preceeded by a (lower) urinary tract infection (UTI).
*Typically preceeded by a (lower) [[urinary tract infection]] (UTI).
*Usually diagnosed clinically:  
*Usually diagnosed clinically:  
**Urine C&S, urine R&M, +/-CT abdomen.
**Urine C&S, urine R&M, +/-CT abdomen.
Line 1,115: Line 1,189:
===Microscopic===
===Microscopic===
Features:
Features:
*Neutrophils within the renal tubules and interstitum.<ref name=Ref_Sternberg5_1726>{{Ref Sternberg5|1726}}</ref>
*[[Neutrophil]]s within the renal tubules and interstitium.<ref name=Ref_Sternberg5_1726>{{Ref Sternberg5|1726}}</ref>


====Images====
<gallery>
Image:Acute_pyelonephritis_-_intermed_mag.jpg | Acute pyelonephritis - intermed. mag. (WC/Nephron)
Image:Acute_pyelonephritis_-_2_-_high_mag.jpg | Acute pyelonephritis - high mag. (WC/Nephron)
Image:Acute_pyelonephritis_-_2_-_very_high_mag.jpg | Acute pyelonephritis - very high mag. (WC/Nephron)
</gallery>
==Chronic pyelonephritis==
==Chronic pyelonephritis==
*''Reflux nephropathy'' is considered synonym in some sources.<ref name=Ref_Sternberg5_1728>{{Ref Sternberg5|1728}}</ref>
*''Reflux nephropathy'' is considered synonym in some sources.<ref name=Ref_Sternberg5_1728>{{Ref Sternberg5|1728}}</ref>
===General===
===General===
*Inflammation of the kidney (''nephritis'') and renal pelvis (''pyelo-''<ref>URL: [http://medical-dictionary.thefreedictionary.com/pyelo- http://medical-dictionary.thefreedictionary.com/pyelo-]. Accessed on: 5 June 2015.</ref>).
*May be associated with vesicoureteral reflux.
*May be associated with vesicoureteral reflux.
*Chronic pyelonephritis may be a reason for nephrectomy.<ref>URL: [https://secure.health.utas.edu.au/intranet/cds/pathprac/Files/Cases/Renal/Case44/Case44.htm https://secure.health.utas.edu.au/intranet/cds/pathprac/Files/Cases/Renal/Case44/Case44.htm]. Accessed on: 26 July 2011.</ref>
*Chronic [[pyelonephritis]] may be a reason for nephrectomy.<ref>URL: [https://secure.health.utas.edu.au/intranet/cds/pathprac/Files/Cases/Renal/Case44/Case44.htm https://secure.health.utas.edu.au/intranet/cds/pathprac/Files/Cases/Renal/Case44/Case44.htm]. Accessed on: 26 July 2011.</ref>


===Gross===
===Gross===
Line 1,133: Line 1,214:
*Interstitial fibrosis.
*Interstitial fibrosis.
*+/-Renal casts (PAS positive); may result in a thyroid-like apparance.
*+/-Renal casts (PAS positive); may result in a thyroid-like apparance.
DDx:
*[[End-stage kidney]].
*[[Myeloma cast nephropathy]].


===Stains===
===Stains===
Line 1,138: Line 1,223:


=Disease that does not commonly get biopsied=
=Disease that does not commonly get biopsied=
==End stage kidney==
==End-stage kidney==
===General===
{{Main|End-stage kidney}}
*Many end-stage renal disease (ESRD) kidneys have a similar appearance.
**Acception: polycystic kidney diseases have distinctive appearance, e.g. [[ADPKD]].
 
===Gross===
*Small kidneys.
*Thinned renal cortex.
*+/-Dilated renal calyces.
*+/-Cysts.
 
===Microscopic===
Features:
*Global sclerosis of the glomeruli.
*Interstitial fibrosis.
*+/-Thyroidization - colloid-like hyaline cast formation that impart an appearance that mimics the [[thyroid gland]]:<ref name=pmid19594588>{{Cite journal  | last1 = Ito | first1 = S. | last2 = Kobayashi | first2 = A. | last3 = Tsuchiya | first3 = T. | last4 = Moriyama | first4 = Y. | last5 = Kikuchi | first5 = M. | last6 = Deguchi | first6 = T. | last7 = Yamaguchi | first7 = Y. | title = Thyroidization in renal allografts. | journal = Clin Transplant | volume = 23 Suppl 20 | issue =  | pages = 6-9 | month = Aug | year = 2009 | doi = 10.1111/j.1399-0012.2009.01001.x | PMID = 19594588 }}</ref>
**Typical of [[chronic pyelonephritis]] and obstructive nephropathy.
Image:
*[http://library.med.utah.edu/WebPath/RENAHTML/RENAL108.html Thyroidization of the kidney (utah.edu)].


==Malignant hypertension==
==Malignant hypertension==
Line 1,167: Line 1,234:
*Often abbreviated ''ATN''.
*Often abbreviated ''ATN''.
===General===
===General===
*Best diagnosed clinically (using urine R&M) - hemegranular casts are diagnostic.
Diagnosed clinically:
*Using urine R&M - hemegranular casts<ref name=pmid19921458>{{Cite journal  | last1 = Kanbay | first1 = M. | last2 = Kasapoglu | first2 = B. | last3 = Perazella | first3 = MA. | title = Acute tubular necrosis and pre-renal acute kidney injury: utility of urine microscopy in their evaluation- a systematic review. | journal = Int Urol Nephrol | volume = 42 | issue = 2 | pages = 425-33 | month = Jun | year = 2010 | doi = 10.1007/s11255-009-9673-3 | PMID = 19921458 }}</ref> are diagnostic.
*Anuria or low urine output.
 
===Gross===
*Poorly defined corticomedullary junction - soft finding.
*Slightly heavier ~ 180 grams.<ref name=pmid19207286/>


===Microscopic===
===Microscopic===
Features:<ref>PS. April 2009.</ref>
Features:<ref name=pmid19207286>{{Cite journal  | last1 = Kocovski | first1 = L. | last2 = Duflou | first2 = J. | title = Can renal acute tubular necrosis be differentiated from autolysis at autopsy? | journal = J Forensic Sci | volume = 54 | issue = 2 | pages = 439-42 | month = Mar | year = 2009 | doi = 10.1111/j.1556-4029.2008.00956.x | PMID = 19207286 }}</ref>
*Hemegranular casts in the lumen.
*Tubular epithelial whorls - present in approx. one third of cases - '''most important'''.
*Regenerative activity.
**Detached epithelium within the luminal space surrounded by epithelium.
**Mitoses - '''diagnositic''' but uncommon.
***Similar to ''epithelial telescoping'' seen in endometrial biopsies.
*Tubulorrhexis - present in approx. one third of cases.
**Disruption of the tubular basement membrane.
*Interstitial edema - sensitive... but not specific.
 
Notes - not particularily useful findings:
*Mitoses.
*Casts in tubules.
 
===IHC===
*Ki-67 - focal nuclear staining of the tubular epithelium.<ref name=pmid19207286/>


==Hepatorenal syndrome==
==Hepatorenal syndrome==
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*[http://www.fondazionedamico.org/biopsiarenale_atlas/seco/crio/crio.htm Cryoglobulinemic nephritis (fondazionedamico.org)].
*[http://www.fondazionedamico.org/biopsiarenale_atlas/seco/crio/crio.htm Cryoglobulinemic nephritis (fondazionedamico.org)].


[[Category: Genitourinary pathology]]
[[Category: Medical kidney pathology]]
Michael
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