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


=Clinical=
=Clinical=
===Glomerular filtration rate===
*Abbreviated ''GFR''.
*Ultimate measure of renal function.
*Declines with age.
*Normal range (dependent on age): 116-75 mL/min/1.73m<sup>2</sup>.<ref>URL: [http://www.kidney.org/professionals/KLS/gfr.cfm http://www.kidney.org/professionals/KLS/gfr.cfm]. Accessed on: 8 November 2010.</ref>
===Creatinine===
===Creatinine===
*The standard screening test for renal function.
*The standard screening test for renal function.
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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>
====Glomerular filtration rate====
*Abbreviated ''GFR''.
*Ultimate measure of renal function - usually estimated from the serum creatinine using a formula.
*Declines with age.
*Normal range (dependent on age): 116-75 mL/min/1.73m<sup>2</sup>.<ref>URL: [http://www.kidney.org/professionals/KLS/gfr.cfm http://www.kidney.org/professionals/KLS/gfr.cfm]. Accessed on: 8 November 2010.</ref>
===Urine protein to creatinine ratio===
===Urine protein to creatinine ratio===
*Indicator of proteinuria.
*Indicator of proteinuria.
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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>
*Normal:
*Normal:
**Minimal change disease.
**[[Minimal change disease]].
**Chronic pyelonephritis.
**Chronic pyelonephritis.
**Renal vein thrombosis.
**Renal vein [[thrombosis]].
**[[Amyloidosis]].
**[[Amyloidosis]].


===ANCA===
===ANCA===
Types:<ref>{{Cite journal  | last1 = Kallenberg | first1 = CG. | title = Pathogenesis of ANCA-associated vasculitides. | journal = Ann Rheum Dis | volume = 70 Suppl 1 | issue =  | pages = i59-63 | month = Mar | year = 2011 | doi = 10.1136/ard.2010.138024 | PMID = 21339221 }}</ref>
Types:<ref name=pmid21339221>{{Cite journal  | last1 = Kallenberg | first1 = CG. | title = Pathogenesis of ANCA-associated vasculitides. | journal = Ann Rheum Dis | volume = 70 Suppl 1 | issue =  | pages = i59-63 | month = Mar | year = 2011 | doi = 10.1136/ard.2010.138024 | PMID = 21339221 }}</ref>
*MPO-ANCA (myeloperoxidase antineutrophil cytoplasmic autoantibody).  
*MPO-ANCA (myeloperoxidase antineutrophil cytoplasmic autoantibody).  
**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===
*ANA, dsDNA -- to screen for [[systemic lupus erythematosus]].
*[[Hepatitis B]].
*[[Hepatitis C]].
*[[HIV]].
*Serum protein electrophoresis (SPEP) -- to screen for [[plasma cell neoplasm]]s.
**Often done together with ''urine protein electrophoresis'' (UPEP).
 
===Renal ultrasound===
*Normal adult kidney size ~10.8+/-0.8 cm.<ref name=pmid8125561>{{Cite journal  | last1 = Guzman | first1 = RP. | last2 = Zierler | first2 = RE. | last3 = Isaacson | first3 = JA. | last4 = Bergelin | first4 = RO. | last5 = Strandness | first5 = DE. | title = Renal atrophy and arterial stenosis. A prospective study with duplex ultrasound. | journal = Hypertension | volume = 23 | issue = 3 | pages = 346-50 | month = Mar | year = 1994 | doi =  | PMID = 8125561 }}</ref>
*Good for assessing the major vessels, drainage system and parenchymal lesions.
**Renal artery stenosis?
**Hydronephrosis?
**Pelviectasis?
**Renal cyst?
**Renal mass?


===Urine dip===
===Urine dip===
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====Mixed====
====Mixed====
*Features of nephritic syndrome and nephrotic syndrome.
*Features of nephritic syndrome and [[nephrotic syndrome]].
 
=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>
 
===Isolated hematuria===
*[[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.
*Crescentic glomerulonephritis ([[AKA]] rapidly progressive glomerulonephritis (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>
#Linear immune deposits.
#*[[Antiglomerular basement membrane disease]] & Goodpasture's syndrome.
#Granular immune deposits.
#*Immune complex diseases.
#**[[Systemic lupus erythematosus]].
#**[[IgA nephropathy]].
#**Others.
#Pauci-immune.
#*ANCA vasculitides.
#**[[Wegener's granulomatosis]].
#**[[Microscopic polyangiitis]].
 
===Nephrotic syndrome===
*Minimal segmental disease (MSD) - [[AKA]] ''minimal change disease'' (MCD).
*[[Focal segmental glomerulosclerosis]] (FSGS).
*[[Membranous nephropathy]].
 
===Mixed presentation===
*[[IgA nephropathy]].
*[[Focal proliferative glomerulosclerosis]] (FPGS).
*[[Membranoproliferative glomerulonephritis]] (MPGN).


=Normal=
=Normal kidney=
===Cells of the glomerulus===
===Cells of the glomerulus===
*Podocytes.
*Podocytes.
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===Glomerulus===
===Glomerulus===
*Normal diameter ~ 160-180 micrometres.
*A large [[autopsy]] series suggest it is: 223-292 micrometers.<ref name=pmid12864872>{{Cite journal  | last1 = Hoy | first1 = WE. | last2 = Douglas-Denton | first2 = RN. | last3 = Hughson | first3 = MD. | last4 = Cass | first4 = A. | last5 = Johnson | first5 = K. | last6 = Bertram | first6 = JF. | title = A stereological study of glomerular number and volume: preliminary findings in a multiracial study of kidneys at autopsy. | journal = Kidney Int Suppl | volume =  | issue = 83 | pages = S31-7 | month = Feb | year = 2003 | doi =  | PMID = 12864872 | url = http://www.nature.com/ki/journal/v63/n83s/fig_tab/4493733t2.html#figure-title }}</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)====
*Podocyte - rarely affect by disease  
*Podocyte - rarely affect by disease  
**One notable disease is ''collapsing glomerulopathy'' in [[HIV]].<ref>AH. 13 August 2009.</ref>
**One notable disease is ''[[collapsing glomerulopathy]]'' in [[HIV]].<ref>AH. 13 August 2009.</ref>
*Endothelial cell.
*Endothelial cell.
*Mesangial cell.
*Mesangial cell.
====Obsolete glomeruli====
*Completely sclerosed glomeruli are not important - unless present in larger numbers than expected for the age of the patient.
:Percent of sclerosed glomeruli = (age in years)/2 - 10%.<ref name=Ref_DARP16>{{Ref DARP|16}}</ref>
Example:
*It is normal for an 80 year-old to have 30% sclerosed glomeruli.
====Glomerular disease terms====
Number of glomeruli involved:<ref name=DARP7>{{Ref DARP|7}}</ref>
*Focal = some of the glomeruli.
**In practical terms, defined as: <50% of glomeruli.
*Diffuse = most of glomeruli.
How much of the glomerulus is involved:<ref name=DARP7>{{Ref DARP|7}}</ref>
*Global = most of the glomerulus.
**In practical terms, defined as: >80% of glomerulus.<ref>{{Cite journal  | last1 = Berden | first1 = AE. | last2 = Ferrario | first2 = F. | last3 = Hagen | first3 = EC. | last4 = Jayne | first4 = DR. | last5 = Jennette | first5 = JC. | last6 = Joh | first6 = K. | last7 = Neumann | first7 = I. | last8 = Noël | first8 = LH. | last9 = Pusey | first9 = CD. | title = Histopathologic classification of ANCA-associated glomerulonephritis. | journal = J Am Soc Nephrol | volume = 21 | issue = 10 | pages = 1628-36 | month = Oct | year = 2010 | doi = 10.1681/ASN.2010050477 | PMID = 20616173 |url=http://jasn.asnjournals.org/content/21/10/1628/T1.expansion.html}}</ref>
*Segmental = part of the glomerulus.


===Vessels===
===Vessels===
#Arteriolar hyalinosis - too much pink stuff?
#Arteriolar hyalinosis - too much glassy pink stuff?
#Intimal hyperplasia.
#Intimal hyperplasia (atherosclerosis).


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]]?
 
====Arteriolar hyalinosis====
Microscopic:
*Small vessels (afferent +/- efferent arteriole) with:
**Glassy eosinophilic material in arteriolar wall.
***[[PAS stain]] +ve.


===Tubules & interstitium===
DDx:
*Aging.
*[[Diabetes mellitus]].
*[[Hypertension]].
*Drugs - [[calcineurin inhibitor toxicity|calcineurin inhibitors]] (tacrolimus, cyclosporine).
 
Note:
*Arteriolar hyalinosis - involves ''afferent'' and ''efferent'' arterioles in diabetes, in others it is only the afferent.
 
Memory device ''ADHD'':
*'''A'''ging, '''D'''iabetes, '''H'''ypertension, '''D'''rugs.
 
=====Image=====
<gallery>
Image:Renal_arterial_hyalinosis_-_he_-_very_high_mag.jpg | Arterial hyaline - HE - very high mag. (WC/Nephron)
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====
{{Main|Atherosclerosis}}
Microscopic:
*Intimal thickening of medium-sized vessels.
**Where is the intima/media interface?
***Internal elastic lamina - wavy band of eosinophilic material on H&E that is 1-2 micrometres thick.
 
Grading - based on the thickness of the media and intima:
*Mild: (tunica) media > (tunica) intima.
*Moderate: media = intima.
*Severe: media < intima.
 
===Tubules===
Tubules - proximal portion is the most important.
Tubules - proximal portion is the most important.
Consider:
*Casts?
*Casts?
*Degeneration, i.e. apoptosis.
*Necrosis?
*Necrosis?
*Regeneration, e.g. mitoses.


===Interstitium===
Interstitium
Interstitium
*Fibrosis - prognostically important.
*Fibrosis - prognostically important.
**Grading: mild = <25%, moderate 25-50%, severe >50%.
**Grading: mild = <25%, moderate 25-50%, severe >50%.
*Inflammation?


==Important terms/process related==
==Important terms/process related==
===Obsolete glomeruli===
*Completely sclerosed glomeruli are not important - unless present in larger numbers than expected for the age of the patient.
:Percent of sclerosed glomeruli = (age in years)/2 - 10%.<ref name=Ref_DARP16>{{Ref DARP|16}}</ref>
Example:
*It is normal for an 80 year-old to have 30% sclerosed glomeruli.
===Glomerular disease terms===
Number of glomeruli involved:<ref name=DARP8>{{Ref DARP|8}}</ref>
*Focal = <80% of glomeruli.
*Diffuse = >80% of glomeruli.
How much of the glomerulus is involved:<ref name=DARP8>{{Ref DARP|8}}</ref>
*Global = entire of glomeruli.
*Segmental = part of glomerulus.
===Staining===
===Staining===
The standard [[stain]] in kidney pathology is ''PAS''.
The standard [[stain]] in kidney pathology is ''PAS''.
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Basic patterns:
Basic patterns:
*Linear.
#Linear.
*Granular.
#Granular.
*Ring-like.
#Ring-like.


Examples:
Examples:
*[[Lupus]] - granular, "full house".
*Linear IgG:
*[[IgA nephropathy]] - branching IgA +ve.
**[[Antiglomerular basement membrane disease]].
*[[Dense deposit disease]] (DDD) - linear C3 with mesangial rings; IgG -ve, IgA -ve.
**[[Goodpasture syndrome]].
*Immune complex disease (primary and secondary) - thick granular deposits.
*Granular deposits:
*[[Anti-glomerular basement membrane disease]] - linear IgG.
**"Full house" - all present.
*[[Goodpasture syndrome]] - linear IgG.
***[[Lupus nephritis]].
*[[Membranous glomerulonephritis]] - IgG, C3, kappa, lambda.
**Branching IgA.
***[[IgA nephropathy]].
**IgG, C3, kappa, lambda.
***[[Membranous glomerulonephritis]].
*Linear C3 with mesangial rings; IgG -ve, IgA -ve.
**[[Dense deposit disease]] (DDD).


Notes:
Notes:
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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==
===Tram-tracking of BM===
===Tram-tracking of BM===
DDx:<ref>AH. 17 July 2009.</ref>
DDx:<ref>AH. 17 July 2009.</ref>
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#[[Transplant glomerulopathy]] (TG).
#[[Transplant glomerulopathy]] (TG).


===Arteriolar hyalinosis===
===Mesangial hypercellularity===
Microscopic:
*Small vessels (afferent +/- efferent arteriole) with:
**Glassy eosinophilic material in arteriolar wall.
***[[PAS stain]] +ve.
 
DDx:
DDx:
*Aging.
#[[Lupus nephritis]].
*[[Diabetes mellitus]].
#[[IgA nephropathy]].
*[[Hypertension]].
*Drugs - [[calcineurin inhibitor toxicity|calcineurin inhibitors]] (tacrolimus, cyclosporine).


Note:
===Mesangial expansion===
*Arteriolar hyalinosis - involves ''afferent'' and ''efferent'' arterioles in diabetes, in others it is only the afferent.
*A patch of matrix can contain more than three mesangial cell nuclei.
**Highly subjective.


Memory device ''ADHD'':
*'''A'''ging, '''D'''iabetes, '''H'''ypertension, '''D'''rugs.
===Atherosclerosis===
{{Main|Atherosclerosis}}
Microscopic:
*Intimal thickening of medium-sized vessels.
**Where is the intima/media interface?
***Internal elastic lamina - wavy band of eosinophilic material on H&E that is 1-2 micrometres thick.
Grading - based on the thickness of the media and intima:
*Mild: (tunica) media > (tunica) intima.
*Moderate: media = intima.
*Severe: media < intima.
===Mesangial hypercellularity===
DDx:
DDx:
# [[Lupus nephritis]].
*[[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>
# [[IgA nephropathy]].
*MPGN:
 
**[[IgA nephropathy]].
===Mesangial expansion===
**[[Henoch-Schoenlein purpura]].
*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>
**[[Lupus nephritis]].
*Immune complex mediated disease (e.g. IgA nephropathy).
*[[Fibrillary glomerulopathy]].
*Henoch-Schoenlein disease.
*[[Immunotactoid glomerulopathy]].
*Lupus.
*[[Renal amyloidosis]].
*Monoclonal immunoglobulin deposition disease.


===Glomerular crescents===
===Glomerular crescents===
Line 277: Line 420:


====Microscopic====
====Microscopic====
Features:
*Crescentic-shaped lesion in the urinary space of a glomerulus.
*Crescentic-shaped lesion in the urinary space of a glomerulus.
**Crescent = looks like the moon shortly after new moon.
**Crescent = looks like the moon shortly after new moon.
**Cells & cellular debris in the urinary space.
***Crescents lack GBM -- thus PAS -ve and MPAS -ve.
*Break in the glomerular basement membrane - '''key feature'''.
*Break in the glomerular basement membrane - '''key feature'''.
**Best seen on a silver stain (e.g. [[Jones stain]]).
**Best seen on a silver stain (e.g. [[Jones stain]]).
*Fibrin.
*Fibrin.
**Described as orange on [[HPS stain|HPS]].
**Orange on [[HPS stain|HPS]].
*Urinary space cellular debris.
**Pink on [[H&E stain|H&E]].
 
*Inflammatory cells (lymphocytes, plasma cells, eosinophils, macrophages) - extravascular - '''low power''' feature.
*Inflammatory cells (lymphocytes, plasma cells, eosinophils, macrophages) - extravascular - '''low power''' feature.


DDx:
DDx:
*Glomerular sclerosis:
*Glomerular sclerosis:
**Usu. no significant inflammation.
**GBM visible through-out glomerulus.
**No fibrin.
**No fibrin.
**Collagen deposition within the glomerular tuft.
**Collagen deposition within the glomerular tuft.
***Seen with [[trichrome stain]].
***Seen with [[trichrome stain]].
**+/-Glomerular tuft-capsule adhesions.
**+/-Glomerular enlargement.
**Usu. no significant inflammation.


====Bland necrotic crescents====
====Bland necrotic crescents====
Line 300: Line 450:
Diseases with crescents - is a long list.<ref>URL: [http://path.upmc.edu/cases/case51/dx.html http://path.upmc.edu/cases/case51/dx.html]. Accessed on: 9 November 2010.</ref>
Diseases with crescents - is a long list.<ref>URL: [http://path.upmc.edu/cases/case51/dx.html http://path.upmc.edu/cases/case51/dx.html]. Accessed on: 9 November 2010.</ref>


=Pathologic DDx=
===Primary light microscopy patterns===
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>
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
 
! Pattern
===Nephritic===
! Key feature
*Post-infectious glomerulonephritis.
! Other findings
**Classically streptococcal.
! DDx of the pattern
*Crescentic glomerulonephritis (AKA rapidly progressive glomerulonephritis (RPGN)).
! DDx
**Anti-GBM disease.
! Image
**ANCA disease (e.g. [[Wegener's granulomatosis]]).
|-
**Goodpasture's syndrome.
|Normal light microscopy
 
| Normal histology
===Nephrotic===
| none ''or'' few glomeruli in a biopsy of an under sampled FSGS
*Minimal segmental disease (MSD) - AKA ''minimal change disease'' (MCD).
| [[membranous nephropathy]], [[focal segmental glomerulosclerosis]] (FSGS)
*Focal segmental glomerulosclerosis (FSGS).
| normal kidney, [[minimal change disease]], [[thin glomerular basement membrane disease]], [[Alport syndrome]], early [[membranous nephropathy]]
*Membranous nephropathy.
| [[Image:Benign kidney -- intermed mag.jpg|thumb|center|150px|Benign kidney (WC)]]
 
|-
===Mixed presentation===
|Nodular mesangial expansion
*IgA nephropathy,
| nodular mesangial matrix expansion, no mesangial hypercellularity
*Focal proliferative glomerulosclerosis (FPGS).  
| GBM thickening, both afferent and efferent arteriole hyalinized
*Membranoproliferative glomerulonephritis (MPGN).
| diffuse proliferative GN, membranous nephropathy
 
| [[diabetic nephropathy]], [[idiopathic nodular glomerulosclerosis]], [[renal amyloidosis]], light chain deposition disease, [[fibrillary glomerulopathy]], [[immunotactoid glomerulopathy]]
| [[Image:Nodular_glomerulosclerosis.jpeg|thumb|center|150px|Nodular GS (WC)]]
|-
|[[Membranous nephropathy]] (MN)
| GBM spikes or pinholes with silver stain, GBM thickening on PAS
| +/-mesangial hypercellularity +/-tram-tracking/wireloop GBM
| normal light microscopy, light chain deposition disease
| [[hepatitis B]], [[hepatitis C]], carcinoma, NSAID toxicity, SLE, idiopathic (MN); light chain deposition disease ([[plasma cell neoplasm]])
| [[Image:Membranous_nephropathy_-_mpas_-_very_high_mag.jpg|thumb|center|150px|MN (WC)]]
|-
|[[Focal segmental glomerulosclerosis]]
| sclerosed glomeruli = mesangial expansion with collagen
| +/-glomerular enlargement, +/- tuft-capsule adhesions
| [[rapidly progressive glomerulonephritis]], nodular mesangial expansion
| primary FSGS, secondary FSGS ([[HIV]], [[IVDU]], [[obesity]], [[parvovirus B19]], [[Alport syndrome]]
| [[Image:Focal_segmental_glomerulosclerosis_-_high_mag.jpg|thumb|center|150px|FSGS (WC)]]
|-
|Diffuse proliferative glomerulonephritis
| mesangial hypercellularity
| +/-mesangial expansion, +/-interstitial inflammation
| nodular glomerulosclerosis
| [[post-infectious glomerulonephritis]], [[MPGN]], [[dense deposit disease]], diffuse proliferative [[lupus nephritis]] (class IV), cryoglobulinemic GN
| [[Image:Membranoproliferative_glomerulonephritis_-_very_high_mag.jpg|thumb|center|150px|MPGN (WC)]]
|-
|[[Rapidly progressive glomerulonephritis]]
| [[glomerular crescents]]
| +/-inflammation, +/-vasculitis
| [[FSGS]], diffuse proliferative glomerulonephritis
| [[AGBM]], [[post-infectious glomerulonephritis]], pauci-immune GN, immune complex diseases ([[lupus nephritis]], [[IgA nephropathy]], others)
| [[Image:Crescentic_glomerulonephritis_%282%29.jpg|thumb|center|150px|Crescentic GN (WC)]]
|-
|Tubular injury
| tubular degeneration (loss of cilia in proximal tubule, apoptosis, necrosis), regeneration (mitosis, nucleoli)
| +/-interstitial fibrosis
| normal light microscopy
| [[acute tubular necrosis]], [[chronic allograft nephropathy]]
| [[Image:Chronic_allograft_nephropathy_-_intermed_mag.jpg|thumb|center|150px|CAN (WC)]]
|-
|Small vessel pathology
| luminal narrowing; +/-thrombosis, +/-hyalinosis
| +/-onion-skinning
| none
| [[thrombotic microangiopathy]] (malignant hypertension, [[scleroderma]] renal crisis, DIC, HUS, TTP,
| [[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
| luminal narrowing ''or'' obstruction; +/-intimal thickening; +/-embolization
| +/-medial thinning
| none
| [[atherosclerosis]], [[cholesterol embolism]], other types of embolism, [[thrombosis]]
| [[Image:Cholesterol_embolus_-_intermed_mag.jpg |thumb|center|150px| Cholesterol embolus (WC)]]
|- <!--
| Pattern
| Key feature
| Other findings
| DDx of the pattern
| Pathol. DDx
| Image -->
|}


===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 337: 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 349: 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]]
| Image?
| [[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 358: 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 364: Line 572:
| usu. none
| usu. none
| EM: foot process loss
| EM: foot process loss
| nephrotic syndrome
| [[nephrotic syndrome]]
| primary vs. secondary ([[lymphoproliferative disorder]], NSAIDs); idiopathic responds to steroids
| primary vs. secondary ([[lymphoproliferative disorder]], NSAIDs); idiopathic responds to steroids
| [[FSGS]]
| [[FSGS]], [[thin glomerular basement membrane disease]] (histologic DDx)
| Image?
| Image?
|-
|-
Line 376: 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 383: 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?
| Image?
| [[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 406: 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 426: 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 435: 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 448: Line 657:
| Diffuse proliferative lupus glomerulonephritis
| Diffuse proliferative lupus glomerulonephritis
|
|
| systemic lupus erythematosus; low C3, low C4
| [[systemic lupus erythematosus]]; low C3, low C4
|-
|-
| Diffuse proliferative [[IgA nephropathy]]
| Diffuse proliferative [[IgA nephropathy]]
Line 461: 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 470: 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 477: 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 486: Line 703:
Notes:
Notes:
*Hypertensive kidneys have changes only in the afferent arteriole, i.e. the efferent arteriole is spared (see ''[[hypertension]]'').
*Hypertensive kidneys have changes only in the afferent arteriole, i.e. the efferent arteriole is spared (see ''[[hypertension]]'').
====Grading====
The severity of changes can be indicated by the grade:<ref>{{Cite journal  | last1 = Tervaert | first1 = TW. | last2 = Mooyaart | first2 = AL. | last3 = Amann | first3 = K. | last4 = Cohen | first4 = AH. | last5 = Cook | first5 = HT. | last6 = Drachenberg | first6 = CB. | last7 = Ferrario | first7 = F. | last8 = Fogo | first8 = AB. | last9 = Haas | first9 = M. | title = Pathologic classification of diabetic nephropathy. | journal = J Am Soc Nephrol | volume = 21 | issue = 4 | pages = 556-63 | month = Apr | year = 2010 | doi = 10.1681/ASN.2010010010 | PMID = 20167701 | URL = http://jasn.asnjournals.org/content/21/4/556.full }}</ref>
{| class="wikitable sortable"
! Grade
! Criteria
|-
|Grade I
| GBM thickening on EM only
|-
|Grade II
| Mild-to-moderate mesangial expansion only on LM
|-
|Grade III
| Glomerulus with mesangial nodules (Kimmelstiel–Wilson nodules)
|-
|Grade IV
| Stage III + global sclerosis in >50% of glomeruli
|}


===IF===
===IF===
Line 500: Line 736:
{{Main|Systemic lupus erythematosus}}
{{Main|Systemic lupus erythematosus}}
*Bread & butter of nephropathology.
*Bread & butter of nephropathology.
*The biopsy done to determine treatment, i.e. how much immunosuppression is needed.
*The biopsy is done to determine treatment, i.e. how much immunosuppression is needed.


===Immunofluorescence===
===Immunofluorescence===
Line 509: Line 745:
*Class I - minimal mesangial LN.
*Class I - minimal mesangial LN.
*Class II - mesangial proliferative LN.
*Class II - mesangial proliferative LN.
**Mesangial hypercellularity. (???)
*Class III - focal lupus nephritis; <50% of glomeruli.
*Class III - focal lupus nephritis; <50% of glomeruli.
*Class VI-S - diffuse segmental LN;  >50% of glomeruli.  
**Mesangial hypercellularity. (???)
*Class VI-S - diffuse segmental LN;  >50% of glomeruli.
**Mesangial hypercellularity. (???)
*Class VI-G - global LN; >50% of glomeruli.
*Class VI-G - global LN; >50% of glomeruli.
*Class V - Membranous lupus nephritis.
*Class V - Membranous lupus nephritis.
Line 521: 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 530: 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=
==IgA nephropathy==
==IgA nephropathy==
*[[AKA]] Berger disease.
*[[AKA]] Berger disease.
**Should '''not''' be confused with ''Buerger disease'' ([[thromboangiitis obliterans]]).
===General===
===General===
*More common in Asians.
*More common in Asians.
*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]].
*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 550: Line 799:
*Diagnosis based on immunofluorescence (IgA+).
*Diagnosis based on immunofluorescence (IgA+).


Image: [http://library.med.utah.edu/WebPath/jpeg2/RENAL096.jpg IgA nephropathy (med.utah.edu)].
====Images====
 
*[http://library.med.utah.edu/WebPath/jpeg2/RENAL096.jpg IgA nephropathy (med.utah.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 566: Line 819:
*Old name ''MPGN type 1''.
*Old name ''MPGN type 1''.
===General===
===General===
*In adults most common cause: ''hepatitis C''.
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]]''.
 
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]] - usu. has "full house" on IF.<ref name=pmid11682680/>
*Cryoglobulinemia.
*[[Hepatitis B]].
*Portocaval shunt.<ref name=pmid21743226/>


===Microscopic===
===Microscopic===
Line 573: Line 840:
*Basement membrane double layering (tram-tracking).
*Basement membrane double layering (tram-tracking).
*Mesangial hypercellularity.
*Mesangial hypercellularity.
DDx:
*[[Nodular glomerulosclerosis]].
====Images====
<gallery>
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)].
===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 591: Line 873:


Images:
Images:
*[http://www.nature.com/modpathol/journal/v20/n6/fig_tab/3800773f1.html DDD (nature.com)].<ref name=pmid17396142/>
*[http://www.nature.com/modpathol/journal/v20/n6/fig_tab/3800773f1.html DDD - light microscopy (nature.com)].<ref name=pmid17396142/>
*[http://path.upmc.edu/cases/case148.html DDD - several images (upmc.edu)].


===IF===
===IF===
Line 597: Line 880:
*IgG negative.
*IgG negative.
*IgA negative
*IgA negative
Images:
*[http://www.nature.com/modpathol/journal/v20/n6/fig_tab/3800773f3.html#figure-title DDD - IF (nature.com)].


===EM===
===EM===
Line 608: Line 894:
==Rapidly progressive glomerulonephritis==
==Rapidly progressive glomerulonephritis==
*Abbreviated ''RPGN''.
*Abbreviated ''RPGN''.
*[[AKA]] ''crescentic glomerulonephritis''.
===General===
===General===
*Acute renal dysfunction.
*Acute renal dysfunction.
*Nephritic syndrome.


DDx:
DDx:
*Pauci-immune GN.
#Linear immunofluorescence.
#*[[Antiglomerular basement membrane disease]], [[Goodpasture syndrome]].
#Granular immunofluorescence
#*[[Lupus nephritis]].
#*[[Post-infectious glomerulonephritis]].
#*[[IgA nephropathy]].
#Pauci-immune.
#*[[Wegener granulomatosis]].
#*[[Microscopic polyangiitis]].


===Microscopic===
===Microscopic===
Features:
Features:
*[[Glomerular crescents]].
*[[Glomerular crescents]].
*Interstitial inflammation.
*+/-[[Vasculitis]].


Image:
====Images====
*[http://commons.wikimedia.org/wiki/File:Crescentic_glomerulonephritis_%282%29.jpg RPGN (WC)].
<gallery>
*[http://commons.wikimedia.org/wiki/File:Crescentic_glomerulonephritis_%281%29.jpg RPGN (WC)].
Image:Crescentic_glomerulonephritis_%282%29.jpg | RPGN (WC/KGH)
 
Image:Crescentic_glomerulonephritis_%281%29.jpg | RPGN (WC/KGH)
Image:Crescentic_glomerulonephritis_-_intermed_mag.jpg | RPGN - intermed. mag. (WC/Nephron)
Image:Crescentic_glomerulonephritis_-_high_mag.jpg | RPGN - high mag. (WC/Nephron)
</gallery>
==Post-infectious glomerulonephritis==
==Post-infectious glomerulonephritis==
===General===
*Abbreviated ''PIGN''.
*Post-streptococcal infection.
{{Main|Post-infectious glomerulonephritis}}
**Lab test: Antistreptolysin O titer (ASOT) +ve.
 
===Microscopic===
Features:
*+/-Neutrophils - in glomerulus.
 
Image:
*[http://commons.wikimedia.org/wiki/File:Acute_Glomerulonephritis_Pathology_Diagram.svg Post-infectious GN (WC)].


=Rare diseases=
=Rare diseases=
==Goodpasture syndrome==
==Antiglomerular basement membrane disease==
*[[AKA]] Goodpasture disease.
*Abbreviated ''AGBM''.
===General===
===General===
*Very rare - estimated incidence 1/1-2 million.
*Known as '''Goodpasture disease''' ([[AKA]] '''Goodpasture syndrome'''), if renal failure is accompanied by [[pulmonary hemorrhage]].<ref>{{cite journal | author=Goodpasture EW | title=The significance of certain pulmonary lesions in relation to the etiology of influenza | journal=Am J Med Sci | year=1919 | volume=158 | pages=863–870 | doi=10.1097/00000441-191911000-00012 | issue=6}}</ref>
*Renal failure and pulmonary hemorrhage.<ref>{{cite journal | author=Goodpasture EW | title=The significance of certain pulmonary lesions in relation to the etiology of influenza | journal=Am J Med Sci | year=1919 | volume=158 | pages=863–870 | doi=10.1097/00000441-191911000-00012 | issue=6}}</ref>
*Rare - estimated incidence 1/1-2 million.<ref name=pmid20962523/>
*[[Antibody mediated hypersensitivity]].
*Thought to occur in genetically susceptible individuals.<ref name=pmid20962523>{{Cite journal  | last1 = Zhou | first1 = XJ. | last2 = Lv | first2 = JC. | last3 = Zhao | first3 = MH. | last4 = Zhang | first4 = H. | title = Advances in the genetics of anti-glomerular basement membrane disease. | journal = Am J Nephrol | volume = 32 | issue = 5 | pages = 482-90 | month =  | year = 2010 | doi = 10.1159/000321324 | PMID = 20962523 }}</ref>
**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.
*Oligouria - poor prognosticator.{{fact}}
 
Tx:
*Immune suppression & plasma exchange.<ref name=pmid12597309>{{Cite journal  | last1 = Shah | first1 = MK. | last2 = Hugghins | first2 = SY. | title = Characteristics and outcomes of patients with Goodpasture's syndrome. | journal = South Med J | volume = 95 | issue = 12 | pages = 1411-8 | month = Dec | year = 2002 | doi =  | PMID = 12597309 }}</ref>


Clinical DDx:
Clinical DDx:
*[[Wegener granulomatosis]].
*[[Wegener granulomatosis]].
*Azathioprine toxicity.<ref>{{Cite journal  | last1 = Stetter | first1 = M. | last2 = Schmidl | first2 = M. | last3 = Krapf | first3 = R. | title = Azathioprine hypersensitivity mimicking Goodpasture's syndrome. | journal = Am J Kidney Dis | volume = 23 | issue = 6 | pages = 874-7 | month = Jun | year = 1994 | doi =  | PMID = 8203372 }}</ref>
*Azathioprine toxicity.<ref name=pmid8203372>{{Cite journal  | last1 = Stetter | first1 = M. | last2 = Schmidl | first2 = M. | last3 = Krapf | first3 = R. | title = Azathioprine hypersensitivity mimicking Goodpasture's syndrome. | journal = Am J Kidney Dis | volume = 23 | issue = 6 | pages = 874-7 | month = Jun | year = 1994 | doi =  | PMID = 8203372 }}</ref>
 
Tx:
*Immune suppression & plasma exchange.<ref name=pmid12597309>{{Cite journal  | last1 = Shah | first1 = MK. | last2 = Hugghins | first2 = SY. | title = Characteristics and outcomes of patients with Goodpasture's syndrome. | journal = South Med J | volume = 95 | issue = 12 | pages = 1411-8 | month = Dec | year = 2002 | doi =  | PMID = 12597309 }}</ref>


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


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


DDx:
DDx:
*AGBM disease.
*Goodpasture syndrome with the pulmonary hemorrhage.
**Goodpasture syndrome without the pulmonary hemorrhage.
 
Image:
*[http://path.upmc.edu/cases/case541/images/fig08.jpg Linear IgG in AGBM disease (upmc.edu)].


==Thin glomerular basement membrane disease==
==Thin glomerular basement membrane disease==
Line 680: Line 989:
Note:
Note:
*Normal GBM: 300-350 nm.
*Normal GBM: 300-350 nm.
==Idiopathic nodular glomerulosclerosis==
===General===
*Not [[diabetes mellitus|diabetic]] '''key feature'''.
Associations:<ref name=pmid18701135/>
*[[Smoking]] - common; thought to be important in the etiology.<ref name=pmid21904413>{{Cite journal  | last1 = Costa | first1 = AF. | last2 = Gomes dos Santos | first2 = WA. | last3 = Filho | first3 = MA. | last4 = Farias | first4 = FT. | last5 = Modesto dos Santos | first5 = V. | title = Nodular glomerulosclerosis in a non-diabetic hypertensive smoker with dyslipidemia. | journal = An Sist Sanit Navar | volume = 34 | issue = 2 | pages = 301-8 | month =  | year =  | doi =  | PMID = 21904413 }}</ref>
*[[Hypertension]].
===Microscopic===
Features:<ref name=pmid18701135>{{Cite journal  | last1 = Li | first1 = W. | last2 = Verani | first2 = RR. | title = Idiopathic nodular glomerulosclerosis: a clinicopathologic study of 15 cases. | journal = Hum Pathol | volume = 39 | issue = 12 | pages = 1771-6 | month = Dec | year = 2008 | doi = 10.1016/j.humpath.2008.05.004 | PMID = 18701135 }}</ref>
*Looks like diabetic nodular glomerulosclerosis.
===IF===
Nonspecific.
===EM===
Nonspecific.
==Fabry disease==
===General===
*Rare X-linked genetic disease.
**Caused by defect in ''alpha-galactosidase A gene''.
**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===
LM:<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.
EM:<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.


==Alport syndrome==
==Alport syndrome==
Line 751: 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 760: Line 1,019:
*Abnormal glomerular basement membrane (GBM); thinning or thickening.
*Abnormal glomerular basement membrane (GBM); thinning or thickening.
**Classically thinning with thick lamellation (splitting/multi-layering).
**Classically thinning with thick lamellation (splitting/multi-layering).
==Idiopathic nodular glomerulosclerosis==
===General===
*Not [[diabetes mellitus|diabetic]] - '''key feature'''.
Associations:<ref name=pmid18701135/>
*[[Smoking]] - common; thought to be important in the etiology.<ref name=pmid21904413>{{Cite journal  | last1 = Costa | first1 = AF. | last2 = Gomes dos Santos | first2 = WA. | last3 = Filho | first3 = MA. | last4 = Farias | first4 = FT. | last5 = Modesto dos Santos | first5 = V. | title = Nodular glomerulosclerosis in a non-diabetic hypertensive smoker with dyslipidemia. | journal = An Sist Sanit Navar | volume = 34 | issue = 2 | pages = 301-8 | month =  | year =  | doi =  | PMID = 21904413 }}</ref>
*[[Hypertension]].
===Microscopic===
Features:<ref name=pmid18701135>{{Cite journal  | last1 = Li | first1 = W. | last2 = Verani | first2 = RR. | title = Idiopathic nodular glomerulosclerosis: a clinicopathologic study of 15 cases. | journal = Hum Pathol | volume = 39 | issue = 12 | pages = 1771-6 | month = Dec | year = 2008 | doi = 10.1016/j.humpath.2008.05.004 | PMID = 18701135 }}</ref>
*Looks like diabetic nodular glomerulosclerosis.
===IF===
Nonspecific.
===EM===
Nonspecific.
==Fabry disease==
{{Main|Fabry disease}}


==Myeloma==
==Myeloma==
Line 771: Line 1,051:
====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).


Image:  
=====Images=====
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)].
 
<gallery>
Image:Cast_nephropathy_-_high_mag.jpg | Myeloma cast nephropathy - high mag. (WC/Nephron)
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 790: 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==
*[[AKA]] ''fibrillary glomerulopathy''.
===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.full }}</ref>
*Presents as [[nephrotic syndrome]].
*Prognosis poor; large number progress to ESRD.
===Microscopic===
Features:
*Mesangial matrix expansion.
*Thickened capillary walls.
Images:
*www:
**[http://jasn.asnjournals.org/content/19/1/34/F1.expansion.html FG (asnjournals.org)].<ref name=pmid18045849/>
**[http://path.upmc.edu/cases/case78.html Fibrillary glomerulopathy (upmc.edu)].
===IF===
*IgG predominant.<ref name=pmid18045849/>
===Stains===
*[[Congo red stain]] -ve.
===EM===
*Fibrils:<ref name=pmid18045849/>
**Non-branching.
**Randomly arranged.
**Usu. 18-20 nm in diameter -- larger than amyloid (see below).
***Size range 12-24 nm.
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.full }}</ref>
==Aristolochic acid nephropathy==
*Abbreviated ''AAN''.
*[[AKA]] ''chinese herb nephropathy''.<ref>{{Cite journal  | last1 = Yang | first1 = L. | last2 = Su | first2 = T. | last3 = Li | first3 = XM. | last4 = Wang | first4 = X. | last5 = Cai | first5 = SQ. | last6 = Meng | first6 = LQ. | last7 = Zou | first7 = WZ. | last8 = Wang | first8 = HY. | title = Aristolochic acid nephropathy: variation in presentation and prognosis. | journal = Nephrol Dial Transplant | volume =  | issue =  | pages =  | month = Jun | year = 2011 | doi = 10.1093/ndt/gfr291 | PMID = 21719716 }}</ref><ref name=cin2003>URL: [http://www.uninet.edu/cin2003/conf/cosyns/cosyns.html http://www.uninet.edu/cin2003/conf/cosyns/cosyns.html]. Accessed on: 23 November 2011.</ref>
===General===
*Nephropathy due to chinese herbs for slimming.<ref name=pmid9027778>{{Cite journal  | last1 = Reginster | first1 = F. | last2 = Jadoul | first2 = M. | last3 = van Ypersele de Strihou | first3 = C. | title = Chinese herbs nephropathy presentation, natural history and fate after transplantation. | journal = Nephrol Dial Transplant | volume = 12 | issue = 1 | pages = 81-6 | month = Jan | year = 1997 | doi =  | PMID = 9027778 | url = http://ndt.oxfordjournals.org/content/12/1/81 }}</ref>
*Associated with urothelial atypia/urothelial carcinoma.
Clinical:
*Chinese herb use.
*Low-molecular weight proteinuria.
===Microscopic===
Features:<ref name=pmid9027778/>
*Extensive interstitial fibrosis.
*Tubular atrophy - typically greater in outer cortex than inner cortex.
*Glomeruli spared.
*+/-Nuclear atypia of the urothelium.
DDx:
*Balkan endemic nephropathy.
Image:
*[http://www.uninet.edu/cin2003/conf/cosyns/fig1.jpg AAN (uninet.edu)].<ref name=cin2003/>


==Cystic kidney diseases==
==Cystic kidney diseases==
{{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.
*Cystic [[renal cell carcinoma]].
*Cystic [[renal cell carcinoma]].


=Disease that does not get biopsied=
=Pyelonephritis=
''Pyelonephritis'' is a misnomer; it is ''not'' an inflammatory process affecting the renal pelvis, as the name suggests. ''Pyelonephritis'' actually refers to a ''nephritis'' or ''tubulointerstitial nephritis''.<ref name=Ref_Sternberg5_1725>{{Ref Sternberg5|1725}}</ref>
 
It can be subdivided into:
*[[Acute pyelonephritis]].
*[[Chronic pyelonephritis]].
**[[Xanthogranulomatous pyelonephritis]].
 
==Acute pyelonephritis==
*[[AKA]] ''acute infectious tubulointerstitial nephritis''.
*[[AKA]] ''diffuse suppurative nephritis''.
===General===
*Typically preceeded by a (lower) [[urinary tract infection]] (UTI).
*Usually diagnosed clinically:
**Urine C&S, urine R&M, +/-CT abdomen.
**Fever, costovertebral tenderness.
 
===Gross===
Features:
*+/-[[Necrosis]] of renal papillae.<ref name=Ref_Klatt251>{{Ref Klatt|251}}</ref>
 
===Microscopic===
Features:
*[[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==
*''Reflux nephropathy'' is considered synonym in some sources.<ref name=Ref_Sternberg5_1728>{{Ref Sternberg5|1728}}</ref>
===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.
*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===
*Dilated and distorted renal pelvis.<ref name=Ref_Sternberg5_1729>{{Ref Sternberg5|1729}}</ref>
*+/-[[Necrosis]] of renal papillae.<ref name=Ref_Klatt251>{{Ref Klatt|251}}</ref>
 
===Microscopic===
Features:<ref name=Ref_Sternberg5_1729>{{Ref Sternberg5|1729}}</ref>
*Mononuclear tubulointerstitial infiltrate.
**Usu. more dense at the pelvis.
*Interstitial fibrosis.
*+/-Renal casts (PAS positive); may result in a thyroid-like apparance.
 
DDx:
*[[End-stage kidney]].
*[[Myeloma cast nephropathy]].
 
===Stains===
*PAS +ve -- renal tubular casts.
 
=Disease that does not commonly get biopsied=
==End-stage kidney==
{{Main|End-stage kidney}}
 
==Malignant hypertension==
==Malignant hypertension==
:''See: [[hyperplastic arteriolosclerosis]]''.
:''See: [[hyperplastic arteriolosclerosis]]''.
Line 808: Line 1,231:
*May be seen in [[scleroderma]].
*May be seen in [[scleroderma]].


==Pyelonephritis==
==Acute tubular necrosis==
*Often abbreviated ''ATN''.
===General===
===General===
*Usually diagnosed clinically: urine C&S, urine R&M, +/-CT abdomen.
Diagnosed clinically:
*May be associated with vesicoureteral reflux.
*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.
*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>
*Anuria or low urine output.


===Gross===
===Gross===
Features:<ref name=Ref_Klatt251>{{Ref Klatt|251}}</ref>
*Poorly defined corticomedullary junction - soft finding.
*+/-[[Necrosis]] of renal papillae.
*Slightly heavier ~ 180 grams.<ref name=pmid19207286/>


===Microscopic===
===Microscopic===
Features:
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>
*Interstitial nephritis.
*Tubular epithelial whorls - present in approx. one third of cases - '''most important'''.
**Detached epithelium within the luminal space surrounded by epithelium.
***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.


==Acute tubular necrosis==
Notes - not particularily useful findings:
===General===
*Mitoses.
*Best diagnosed clinically (using urine R&M) - hemegranular casts are diagnostic.
*Casts in tubules.
*Often abbreviated ''ATN''.


===Microscopic===
===IHC===
Features:<ref>PS. April 2009.</ref>
*Ki-67 - focal nuclear staining of the tubular epithelium.<ref name=pmid19207286/>
*Hemegranular casts in the lumen.
*Regenerative activity (mitoses).


==Hepatorenal syndrome==
==Hepatorenal syndrome==
Line 855: Line 1,281:
Features (kidney):
Features (kidney):
*Normal.
*Normal.
==Nephrocalcinosis==
===General===
*[[Hypercalcemia]].
===Microscopic===
Features:
*Calcification of the renal parenchyma - '''key feature'''.
Images:
*[http://path.upmc.edu/cases/case514.html Nephrocalcinosis (upmc.edu)].


=Renal transplant pathology=
=Renal transplant pathology=
Line 878: Line 1,315:
*[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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