Difference between revisions of "Medical kidney diseases"

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====Mixed====
====Mixed====
*Features of nephritic syndrome and nephrotic syndrome.
*Features of nephritic syndrome and nephrotic syndrome.
=Clinical DDx=
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===
*Post-infectious glomerulonephritis.
**Classically streptococcal.
*Crescentic glomerulonephritis ([[AKA]] rapidly progressive glomerulonephritis (RPGN)).
====RPGN====
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===
*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=
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===Vessels===
===Vessels===
#Arteriolar hyalinosis - too much pink stuff?
#Arteriolar hyalinosis - too much glassy pink stuff?
#Intimal hyperplasia.
#Intimal hyperplasia (atherosclerosis).


Consider:
Consider:
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*Amyloid? - pink.
*Amyloid? - pink.
*Rejection? - PMNs.
*Rejection? - PMNs.
*Cholesterol emboli?


===Tubules & interstitium===
===Tubules & interstitium===
Tubules - proximal portion is the most important.
Tubules - proximal portion is the most important.
*Casts?
*Casts?
*Degeneration, i.e. apoptosis.
*Necrosis?
*Necrosis?
*Regeneration, e.g. mitoses.


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==
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*Severe: media < intima.
*Severe: media < intima.


==Pathologic differential diagnosis==
===Mesangial hypercellularity===
===Mesangial hypercellularity===
DDx:
DDx:
# [[Lupus nephritis]].
#[[Lupus nephritis]].
# [[IgA nephropathy]].
#[[IgA nephropathy]].


===Mesangial expansion===
===Mesangial expansion===
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 (e.g. IgA nephropathy).
*Immune complex mediated disease  
*[[Henoch-Schoenlein purpura]].
**[[IgA nephropathy]].
*Lupus.
**[[Henoch-Schoenlein purpura]].
**[[Lupus nephritis]].


===Glomerular crescents===
===Glomerular crescents===
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====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====
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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=
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===
*Post-infectious glomerulonephritis.
**Classically streptococcal.
*Crescentic glomerulonephritis ([[AKA]] rapidly progressive glomerulonephritis (RPGN)).
====RPGN====
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.
#*Anti-GBM 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===
*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).




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{{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===
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*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.
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*In adults most common cause: ''hepatitis C''.
*In adults most common cause: ''hepatitis C''.


Clinical:
*Nephrotic syndrome or nephrotic/nephritic syndrome.
===Microscopic===
===Microscopic===
Features:
Features:
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DDx:
DDx:
#Linear immunofluorescence.
#Linear immunofluorescence.
#*[[Antiglomerular basement membrane disease]].
#*[[Antiglomerular basement membrane disease]], [[Goodpasture syndrome]].
#*[[Goodpasture syndrome]].
#Granular immunofluorescence  
#Granular immunofluorescence  
#*[[Lupus nephritis]].
#*[[Lupus nephritis]].
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Features:
Features:
*[[Glomerular crescents]].
*[[Glomerular crescents]].
*Interstitial inflammation.
*+/-[[Vasculitis]].


Image:
Image:
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Features:
Features:
*+/-Neutrophils - in glomerulus.
*+/-Neutrophils - in glomerulus.
*[[Glomerular crescents]].


Image:
Image:
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==Idiopathic nodular glomerulosclerosis==
==Idiopathic nodular glomerulosclerosis==
===General===
===General===
*Not [[diabetes mellitus|diabetic]] '''key feature'''.
*Not [[diabetes mellitus|diabetic]] - '''key feature'''.


Associations:<ref name=pmid18701135/>
Associations:<ref name=pmid18701135/>
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