48,790
edits
(→Normal) |
|||
(301 intermediate revisions by the same user not shown) | |||
Line 1: | Line 1: | ||
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. | [[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= | ||
=== | ===Creatinine=== | ||
* | *The standard screening test for renal function. | ||
*The | *300 mmol/L is the general cut-point for referral to a nephrologist.<ref name=pmid>{{cite journal |author=Mendelssohn DC, Barrett BJ, Brownscombe LM, ''et al.'' |title=Elevated levels of serum creatinine: recommendations for management and referral |journal=CMAJ |volume=161 |issue=4 |pages=413–7 |year=1999 |month=August |pmid=10478168 |pmc=1230545 |doi= |url=http://www.cmaj.ca/cgi/content/full/161/4/413}}</ref> | ||
Notes: | |||
*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. | |||
</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=== | |||
*Indicator of proteinuria. | |||
*Predictor of glomerular filtration rate.<ref name=pmid9501711>{{cite journal |author=Ruggenenti P, Gaspari F, Perna A, Remuzzi G |title=Cross sectional longitudinal study of spot morning urine protein:creatinine ratio, 24 hour urine protein excretion rate, glomerular filtration rate, and end stage renal failure in chronic renal disease in patients without diabetes |journal=BMJ |volume=316 |issue=7130 |pages=504–9 |year=1998 |month=February |pmid=9501711 |pmc=2665663 |doi= |url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2665663/pdf/9501711.pdf}}</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 | |||
*[[Nephrotic syndrome|Nephrotic]] range: >3.5 g protein / g Creatinine. | |||
===Complement=== | |||
C3, C4 levels:<ref name=pmid4549215>{{cite journal |author=Levo Y, Pick AI |title=The significance of C3 and C4 complement levels in lupus nephritis |journal=Int Urol Nephrol |volume=6 |issue=3-4 |pages=233–8 |year=1974 |pmid=4549215 |doi= |url=http://www.springerlink.com/content/l1657797661468g1/fulltext.pdf}}</reF> | |||
*Changed: | |||
**[[Systemic lupus erythematosus]] (SLE); levels decreased = increased disease activity. | |||
**[[Post-infectious GN]] - 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> | |||
**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: | |||
**[[Minimal change disease]]. | |||
**Chronic pyelonephritis. | |||
**Renal vein [[thrombosis]]. | |||
**[[Amyloidosis]]. | |||
===ANCA=== | |||
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). | |||
**Previously p-ANCA. | |||
**Seen in ANCA-[[vasculitides]], esp. [[microscopic polyangiitis]]. | |||
*PR3-ANCA (proteinase 3 antineutrophil cytoplasmic autoantibody). | |||
**Previously c-ANCA. | |||
**Seen in ANCA-[[vasculitides]], esp. [[granulomatosis with polyangiitis]] (Wegener granulomatosis). | |||
===C4d=== | |||
*Suggests humoral immunity (antibody-mediated immunity) at play. | |||
*Important in monitoring of renal transplant recipients. | |||
*[[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=== | |||
Findings:<ref>URL: [http://www.nlm.nih.gov/medlineplus/ency/article/003586.htm http://www.nlm.nih.gov/medlineplus/ency/article/003586.htm]. Accessed on: 20 September 2010.</ref> | |||
*RBC casts = acute bleed, e.g. nephritic syndrome. | |||
*WBC casts = interstitial nephritis, e.g. pylonephritis, parenchymal infection. | |||
*Hemegranular casts = acute tubular necrosis, transplant rejection. | |||
Notes: | |||
*"Active sediment" = RBCs, RBC casts;<ref>URL: [http://emedicine.medscape.com/article/238158-overview http://emedicine.medscape.com/article/238158-overview]. Accessed on: 9 November 2010.</ref> implies glomerulonephritis. | |||
**Some include the above (RBCs, RBC casts) + WBCs & protein.<ref>URL: [http://www.nephrologychannel.com/agn/index.shtml http://www.nephrologychannel.com/agn/index.shtml]. Accessed on: 9 November 2010.</ref> | |||
====Urine crystals==== | |||
{{main|Crystals in body fluids}} | |||
==Clinical presentations== | ===Clinical presentations=== | ||
===Nephrotic syndrome=== | ====Nephrotic syndrome==== | ||
Features: | Features: | ||
*Anasarca (whole body - edema). | *Anasarca (whole body - edema). | ||
Line 17: | Line 96: | ||
*Hypoalbuminemia. | *Hypoalbuminemia. | ||
===Nephritic syndrome=== | ====Nephritic syndrome==== | ||
Features - mnemonic ''PHAROH'':<ref>URL: [http://books.google.com/books?id=5bmg8xiLxkMC&pg=PA249&lpg=PA249&dq=Nephritic+syndrome+PHAROH#v=onepage&q=Nephritic%20syndrome%20PHAROH&f=false http://books.google.com/books?id=5bmg8xiLxkMC&pg=PA249&lpg=PA249&dq=Nephritic+syndrome+PHAROH#v=onepage&q=Nephritic%20syndrome%20PHAROH&f=false]. Accessed on: 9 December 2009.</ref> | Features - mnemonic ''PHAROH'':<ref>URL: [http://books.google.com/books?id=5bmg8xiLxkMC&pg=PA249&lpg=PA249&dq=Nephritic+syndrome+PHAROH#v=onepage&q=Nephritic%20syndrome%20PHAROH&f=false http://books.google.com/books?id=5bmg8xiLxkMC&pg=PA249&lpg=PA249&dq=Nephritic+syndrome+PHAROH#v=onepage&q=Nephritic%20syndrome%20PHAROH&f=false]. Accessed on: 9 December 2009.</ref> | ||
*Proteinuria. | *Proteinuria. | ||
Line 26: | Line 105: | ||
*Hematuria. | *Hematuria. | ||
===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 kidney= | |||
===Cells of the glomerulus=== | |||
*Podocytes. | |||
*Mesangial cells. | |||
*Endothelium. | |||
===Epithelium=== | |||
Features:<ref name=Ref_PBoD956>{{Ref PBoD|956}}</ref> | |||
*The glomeruli visceral epithelium is part of the capillary wall (part of the glomerular tuft). | |||
*The parietal epithelium is part of Bowman's capsule. | |||
Remember: '''v'''isceral has '''v'''essels. | |||
===Glomerulus=== | |||
*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==== | |||
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 components=== | |||
*Glomeruli. | *Glomeruli. | ||
*Tubules. | *Tubules. | ||
*Interstitium. | *Interstitium. | ||
*Vessels. | *Vessels. | ||
Memory device ''GI TV'': glomeruli, interstitium, tubules, vessels. | |||
===Glomeruli=== | ===Glomeruli=== | ||
#Mesangium | #Mesangium | ||
#*Matrix should be: " | #*Matrix should be: "one cell thick" (expanded in diabetes mellitus). | ||
#*Cellularity of the mesangium - normal = '''upto 3 cells''' (don't count cell abutting the capillary lumen). | #*Cellularity of the mesangium - normal = '''upto 3 cells''' (don't count cell abutting the capillary lumen, don't count at the hilum). | ||
#Capillary loops "open" | #Capillary loops "open" | ||
#*Lumina patent? If ''not'' patent is it due to ''matrix'' or ''cells'' (endocapillary hypercellularity). | #*Lumina patent? If ''not'' patent is it due to ''matrix'' or ''cells'' (endocapillary hypercellularity). | ||
Line 56: | Line 231: | ||
*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? - | *Rejection? - [[PMN]]s. | ||
*[[Cholesterol emboli]]? | |||
===Tubules | ====Arteriolar hyalinosis==== | ||
Microscopic: | |||
*Small vessels (afferent +/- efferent arteriole) with: | |||
**Glassy eosinophilic material in arteriolar wall. | |||
***[[PAS stain]] +ve. | |||
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== | ||
===Staining=== | ===Staining=== | ||
The standard [[stain]] in kidney pathology is ''PAS''. | The standard [[stain]] in kidney pathology is ''PAS''. | ||
Line 109: | Line 336: | ||
*IgA. | *IgA. | ||
*IgM. | *IgM. | ||
*C1q | |||
*C3. | *C3. | ||
*Fibrinogen. | *Fibrinogen. | ||
*Albumin. | |||
Optional: | Optional: | ||
*Kappa. | *Kappa. | ||
*Lambda. | *Lambda. | ||
*C4d. | |||
**Positive staining = peri-tubular capillaries stain. | |||
====Negative immunofluorescence==== | |||
*Excludes all immune complex associated disease. | |||
Seen in: | |||
* [[Minimal change disease]]. | |||
* [[Focal segmental glomerulosclerosis]]. | |||
====Positive immunofluorescence==== | |||
*Positive immunofluorescence is usually diagnostic. | |||
Basic patterns: | |||
#Linear. | |||
#Granular. | |||
#Ring-like. | |||
Examples: | |||
*Linear IgG: | |||
**[[Antiglomerular basement membrane disease]]. | |||
**[[Goodpasture syndrome]]. | |||
*Granular deposits: | |||
**"Full house" - all present. | |||
***[[Lupus nephritis]]. | |||
**Branching IgA. | |||
***[[IgA nephropathy]]. | |||
**IgG, C3, kappa, lambda. | |||
***[[Membranous glomerulonephritis]]. | |||
*Linear C3 with mesangial rings; IgG -ve, IgA -ve. | |||
**[[Dense deposit disease]] (DDD). | |||
Notes: | |||
*Nonspecific linear IgG staining may be seen in [[diabetic nephropathy]]. | |||
===Immune complex-related disease=== | ===Immune complex-related disease=== | ||
Can be: | Can be: | ||
*Subepithelial - distal to basement membrane (BM). | *Subepithelial - distal to basement membrane (BM), closer to the urinary space. | ||
*Subendothelial - proximal to BM. | **[[Membranous nephropathy]], [[Post-infectious glomerulonephritis]] (hump-like), [[MPGN]]. | ||
*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> | ||
#MPGN. | #[[MPGN]]. | ||
#Thrombotic | #[[Thrombotic microangiopathy]] (TMA). | ||
#Transplant glomerulopathy (TG). | #[[Transplant glomerulopathy]] (TG). | ||
===Mesangial hypercellularity=== | |||
DDx: | |||
#[[Lupus nephritis]]. | |||
#[[IgA nephropathy]]. | |||
===Mesangial expansion=== | |||
*A patch of matrix can contain more than three mesangial cell nuclei. | |||
**Highly subjective. | |||
DDx: | DDx: | ||
*Diabetes mellitus. | *[[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> | ||
* | *MPGN: | ||
* | **[[IgA nephropathy]]. | ||
* | **[[Henoch-Schoenlein purpura]]. | ||
**[[Lupus nephritis]]. | |||
*[[Fibrillary glomerulopathy]]. | |||
*[[Immunotactoid glomerulopathy]]. | |||
*[[Renal amyloidosis]]. | |||
*Monoclonal immunoglobulin deposition disease. | |||
===Glomerular crescents=== | |||
*[[AKA]] crescents. | |||
====General==== | |||
*Indicates a rapidly progressive disease. | |||
*Etiology/definition: break in the glomerular basement membrane (GBM). | |||
* | ====Microscopic==== | ||
Features: | |||
*Crescentic-shaped lesion in the urinary space of a glomerulus. | |||
**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'''. | |||
**Best seen on a silver stain (e.g. [[Jones stain]]). | |||
*Fibrin. | |||
**Orange on [[HPS stain|HPS]]. | |||
**Pink on [[H&E stain|H&E]]. | |||
*Inflammatory cells (lymphocytes, plasma cells, eosinophils, macrophages) - extravascular - '''low power''' feature. | |||
* | |||
DDx: | |||
* | *Glomerular sclerosis: | ||
* | **GBM visible through-out glomerulus. | ||
* | **No fibrin. | ||
* | **Collagen deposition within the glomerular tuft. | ||
***Seen with [[trichrome stain]]. | |||
**+/-Glomerular tuft-capsule adhesions. | |||
**+/-Glomerular enlargement. | |||
**Usu. no significant inflammation. | |||
== | ====Bland necrotic crescents==== | ||
DDx: | |||
*ANCA-related glomerulonephritis. | |||
*Anti-GBM disease. | |||
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> | |||
=== | ===Primary light microscopy patterns=== | ||
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto" | |||
! Pattern | |||
! Key feature | |||
! Other findings | |||
! DDx of the pattern | |||
! DDx | |||
! Image | |||
|- | |||
|Normal light microscopy | |||
| Normal histology | |||
| none ''or'' few glomeruli in a biopsy of an under sampled FSGS | |||
| [[membranous nephropathy]], [[focal segmental glomerulosclerosis]] (FSGS) | |||
| normal kidney, [[minimal change disease]], [[thin glomerular basement membrane disease]], [[Alport syndrome]], early [[membranous nephropathy]] | |||
| [[Image:Benign kidney -- intermed mag.jpg|thumb|center|150px|Benign kidney (WC)]] | |||
|- | |||
|Nodular mesangial expansion | |||
| nodular mesangial matrix expansion, no mesangial hypercellularity | |||
| GBM thickening, both afferent and efferent arteriole hyalinized | |||
| 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=== | ||
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto" | |||
! Pattern | |||
! Key feature | |||
! Other findings | |||
! IF & EM | |||
! Presentation | |||
! Clinical | |||
! Pathol. DDx | |||
! Image | |||
|- | |||
|[[Nodular glomerulosclerosis]] | |||
| nodular mesangial matrix expansion | |||
| GBM thickening, both afferent and efferent arteriole hyalinized | |||
| EM? | |||
| proteinuria - typical presentation, nephrotic syndrome{{fact}} | |||
| [[diabetes mellitus]] (DM) | |||
| [[amyloidosis]], [[idiopathic nodular glomerulosclerosis]] (nodular GS without DM) | |||
| [[Image:Nodular_glomerulosclerosis.jpeg|thumb|center|150px|Nodular GS (WC)]] | |||
|- | |||
|[[Focal segmental glomerulosclerosis]] (FSGS) | |||
| focal sclerosis of gloms | |||
| +/-interstitial fibrosis | |||
| IF: negative; EM: foot process loss | |||
| nephrotic syndrome | |||
| primary FSGS, secondary FSGS ([[HIV]], [[IVDU]], obesity, [[parvovirus B19]], [[Alport syndrome]]); unresponsive to steroids, worse prognosis than MCD | |||
| [[minimal change disease]] | |||
| [[Image:Focal_segmental_glomerulosclerosis_-_high_mag.jpg|thumb|center|150px|FSGS (WC)]] | |||
|- | |||
|[[Membranous nephropathy]]<br>(AKA membranous GN) | |||
| spikes or pinholes with silver stain | |||
| mesangial hypercellularity; +/-tram-tracking/wireloop GBM | |||
| IF: diffuse granular capillary loop IgG, C3, kappa, lambda; EM: diffuse subepithelial deposits - spike forming | |||
| nephrotic syndrome | |||
| [[hepatitis B]], [[hepatitis C]], carcinoma, NSAID toxicity, SLE, idiopathic | |||
| Nodular glomerulosclerosis (?) | |||
| [[Image:Membranous_nephropathy_-_mpas_-_very_high_mag.jpg|thumb|center|150px|MN (WC)]] | |||
|- | |||
|[[Minimal change disease]] (MCD) | |||
| foot process loss on [[EM]] | |||
| usu. none | |||
| EM: foot process loss | |||
| [[nephrotic syndrome]] | |||
| primary vs. secondary ([[lymphoproliferative disorder]], NSAIDs); idiopathic responds to steroids | |||
| [[FSGS]], [[thin glomerular basement membrane disease]] (histologic DDx) | |||
| Image? | |||
|- | |||
|[[IgA nephropathy]] | |||
| IgA branching pattern | |||
| +/-mesangial hypercellularity, +/-crescents | |||
| IF: IgA +ve (branching pattern); EM: dense mesangial deposits | |||
| mixed nephrotic/nephritic | |||
| primary vs. secondary (Henoch-Schoenlein purpura) | |||
| [[RPGN]] | |||
| [[Image:Henoch-Sch%C3%B6nlein_nephritis_IgA_immunostaining.jpg|thumb|center|150px|HSN - IgA IHC (WC)]] | |||
|- | |||
|[[Membranoproliferative glomerulonephritis]] (MPGN) | |||
| thick GBM | |||
| Other findings? | |||
| subepithelial deposits | |||
| mixed nephrotic/nephritic | |||
| [[SLE]], [[cryoglobulinemia]], [[hepatitis B]], [[hepatitis C]] | |||
| Pathol. DDx? | |||
| [[Image:Membranoproliferative_glomerulonephritis_-_very_high_mag.jpg|thumb|center|MPGN (WC)]] | |||
|- | |||
|[[Focal proliferative glomerosclerosis|Focal proliferative<br> glomerosclerosis]] (FPGS) | |||
| <50% of glomeruli partially sclerosis | |||
| Other findings? | |||
| EM? | |||
| mixed nephrotic/nephritic | |||
| Clinical? | |||
| Pathol. DDx? | |||
| Image? | |||
|- | |||
|Rapidly progressive GN (RPGN) | |||
| crescents | |||
| Other findings? | |||
| EM? | |||
| nephritic syndrome | |||
| [[AGBM]], ANCA-vasculitis | |||
| [[IgA nephropathy]] with crescents | |||
| Image? | |||
|- | |||
| [[Dense deposit disease]] | |||
| IF: linear C3 with rings | |||
| +/-thick GBM | |||
| EM: GBM lamina densa thickening | |||
| Presentation? | |||
| mixed nephrotic/nephritic (???) | |||
| [[MPGN]] | |||
| [http://www.nature.com/modpathol/journal/v20/n6/fig_tab/3800773f1.html (nature.com)] | |||
|- <!-- | |||
| Pattern | |||
| Key feature? | |||
| Other findings? | |||
| EM? | |||
| Presentation? | |||
| Clinical? | |||
| Pathol. DDx? | |||
| Image? --> | |||
|} | |||
== | ===Diffuse proliferative glomerulonephritis=== | ||
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto" | |||
! Pattern | |||
! Key feature | |||
{{ | ! Clinical | ||
|- | |||
| [[Post-infectious glomerulonephritis]] | |||
| IF: capillary loop +/- mesangial IgG/C3; EM: large infreq. hump-like subepithelial deposits | |||
</ | | post-infection | ||
|- | |||
| [[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) | |||
|- | |||
| [[Dense deposit disease]] | |||
| IF: linear C3 with rings | |||
| | |||
|- | |||
| [[Cryoglobulinemic glomerulonephritis]] | |||
| | |||
| | |||
|- | |||
| Diffuse proliferative lupus glomerulonephritis | |||
| | |||
| [[systemic lupus erythematosus]]; low C3, low C4 | |||
|- | |||
| Diffuse proliferative [[IgA nephropathy]] | |||
| IF: IgA +ve (branching pattern) | |||
| | |||
|- | |||
|} | |||
== | =Common diseases= | ||
==Diabetic nephropathy== | |||
===General=== | ===General=== | ||
*Most common cause of end stage renal disease (ESRD). | *Due to [[diabetes mellitus]] - '''key feature'''. | ||
* | **If there is no history of diabetes... it is ''[[idiopathic nodular glomerulosclerosis]]''. | ||
*Most common cause of [[end-stage renal disease]] (ESRD). | |||
*Biopsied only if the (clinical) features are atypical. | |||
===Microscopic=== | ===Microscopic=== | ||
Line 193: | 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''). | ||
Others: | |||
*Armanni-Ebstein change | *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 200: | Line 688: | ||
Memory device: | Memory device: | ||
*GBM = ''thick '''G'''BM'', '''''b'''oth afferent & efferent | *GBM = ''thick '''G'''BM'', '''''b'''oth afferent & efferent arterioles thickened'', '''''m'''esangial matrix expansion''. | ||
Images: | ====Images==== | ||
*[http:// | <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://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 209: | Line 704: | ||
*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=== | |||
*Negative. | |||
*+/-Nonspecific linear IgG. | |||
===EM=== | |||
*Severe thickening of GBM. | |||
*Mesangial sclerosis. | |||
==Lupus nephritis== | |||
*Abbreviated ''LN''. | |||
===General=== | |||
{{Main|Systemic lupus erythematosus}} | |||
*Bread & butter of nephropathology. | |||
*The biopsy is done to determine treatment, i.e. how much immunosuppression is needed. | |||
===Immunofluorescence=== | |||
*"Full house" = all of 'em light up. | |||
===Classification=== | |||
International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification:<ref>{{cite journal |author=Weening JJ, D'Agati VD, Schwartz MM, ''et al.'' |title=The classification of glomerulonephritis in systemic lupus erythematosus revisited |journal=J. Am. Soc. Nephrol. |volume=15 |issue=2 |pages=241–50 |year=2004 |month=February |pmid=14747370 |doi= |url=http://www.nature.com/ki/journal/v55/n2/full/4490631a.html}}</ref><ref>URL: [http://www.med.niigata-u.ac.jp/npa/Lectures/Lupus_Nephritis.htm http://www.med.niigata-u.ac.jp/npa/Lectures/Lupus_Nephritis.htm]. Accessed on: 9 November 2010.</ref> | |||
*Class I - minimal mesangial LN. | |||
*Class II - mesangial proliferative LN. | |||
**Mesangial hypercellularity. (???) | |||
*Class III - focal lupus nephritis; <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 V - Membranous lupus nephritis. | |||
**[[Membranous nephropathy]] due to SLE. | |||
*Class IV - Advanced sclerosing LN; essentially end-stage kidney. | |||
Notes: | |||
*Most of the action is in Class III and Class IV. | |||
**Class I is near normal - doesn't get biopsied. | |||
**Class IV is essentially a dead kidney - doesn't get biopsied. | |||
====Images==== | |||
<gallery> | |||
Image:SLE_Nephritis_Pathology_Diagram.svg| SLE nephritis - schematic. (WC) | |||
</gallery> | |||
*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/3989875091/in/set-72157622411941607/ Jones (flickr.com)]. | |||
**[http://www.flickr.com/photos/jian-hua_qiao_md/3990630972/in/set-72157622411941607/ PAS (flickr.com)]. | |||
=Nephrotic syndrome= | |||
{{Main|Nephrotic syndrome}} | |||
This classically includes the following: | |||
*[[Minimal change disease]]. | |||
*[[Focal segmental glomerulosclerosis]]. | |||
*[[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= | |||
==IgA nephropathy== | ==IgA nephropathy== | ||
*[[AKA]] Berger disease. | |||
**Should '''not''' be confused with ''Buerger disease'' ([[thromboangiitis obliterans]]). | |||
===General=== | |||
*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> | |||
*''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=== | |||
Features: | |||
*Variable: | |||
**Mesangial hypercellularity - may be only light microscopy finding. | |||
Note: | |||
*Diagnosis based on immunofluorescence (IgA+). | *Diagnosis based on immunofluorescence (IgA+). | ||
=== | ====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==== | |||
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=== | ||
* | *IgA +ve in a branching pattern - '''diagnostic'''. | ||
=== | ===EM=== | ||
* | *Mesangial deposits. | ||
**These are electron dense, ergo dark on EM images. | |||
==Membranoproliferative glomerulonephritis== | ==Membranoproliferative glomerulonephritis== | ||
* | *Abbreviated ''MPGN''. | ||
*In adults most common cause: ''hepatitis C''. | *Old name ''MPGN type 1''. | ||
===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]]''. | |||
=== | 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=== | |||
Features: | |||
*Endothelial cell proliferation. | *Endothelial cell proliferation. | ||
*Basement membrane double layering (tram-tracking). | *Basement membrane double layering (tram-tracking). | ||
*Mesangial hypercellularity. | *Mesangial hypercellularity. | ||
==Lupus nephritis== | 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== | |||
*Abbreviated ''DDD''. | |||
*[[AKA]] ''MPGN type 2'' (old name). | |||
===General=== | |||
*Usually children and young adults. | |||
*No longer considered a type of MPGN.<ref name=pmid17396142/> | |||
===Microscopic=== | |||
Features: | |||
*Variable - may be like MPGN. | |||
**Four patterns:<ref name=pmid17396142>{{Cite journal | last1 = Walker | first1 = PD. | last2 = Ferrario | first2 = F. | last3 = Joh | first3 = K. | last4 = Bonsib | first4 = SM. | title = Dense deposit disease is not a membranoproliferative glomerulonephritis. | journal = Mod Pathol | volume = 20 | issue = 6 | pages = 605-16 | month = Jun | year = 2007 | doi = 10.1038/modpathol.3800773 | PMID = 17396142 }}</ref> | |||
**#Hypercellularity and lobular (membranoproliferative-like). | |||
**#Mesangial proliferative. | |||
**#Crescentic. | |||
**#Acute proliferative and exudative. | |||
Images: | |||
*[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=== | |||
*Linear C3 with mesangial rings (donut-like). | |||
*IgG negative. | |||
*IgA negative | |||
Images: | |||
*[http://www.nature.com/modpathol/journal/v20/n6/fig_tab/3800773f3.html#figure-title DDD - IF (nature.com)]. | |||
===EM=== | |||
*Electron dense transformation of GBM lamina densa - key feature. | |||
**Dense = darker. | |||
Images: | |||
*[http://www.nature.com/modpathol/journal/v20/n6/fig_tab/3800773f4.html#figure-title DDD (nature.com)]. | |||
=Nephritic syndrome= | |||
==Rapidly progressive glomerulonephritis== | |||
*Abbreviated ''RPGN''. | |||
*[[AKA]] ''crescentic glomerulonephritis''. | |||
===General=== | |||
*Acute renal dysfunction. | |||
*Nephritic syndrome. | |||
DDx: | |||
#Linear immunofluorescence. | |||
#*[[Antiglomerular basement membrane disease]], [[Goodpasture syndrome]]. | |||
#Granular immunofluorescence | |||
#*[[Lupus nephritis]]. | |||
#*[[Post-infectious glomerulonephritis]]. | |||
#*[[IgA nephropathy]]. | |||
#Pauci-immune. | |||
#*[[Wegener granulomatosis]]. | |||
#*[[Microscopic polyangiitis]]. | |||
===Microscopic=== | |||
Features: | |||
*[[Glomerular crescents]]. | |||
*Interstitial inflammation. | |||
*+/-[[Vasculitis]]. | |||
====Images==== | |||
<gallery> | |||
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== | |||
*Abbreviated ''PIGN''. | |||
{{Main|Post-infectious glomerulonephritis}} | |||
== | =Rare diseases= | ||
==Antiglomerular basement membrane disease== | |||
*Abbreviated ''AGBM''. | |||
===General=== | ===General=== | ||
* | *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> | ||
*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: | |||
*[[Wegener granulomatosis]]. | |||
*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> | |||
===Microscopic=== | |||
Features: | |||
*[[RPGN]]. | |||
* | **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=== | ||
* | *Linear IgG deposits - '''diagnostic'''. | ||
*Fibrinogen in crescents. | |||
DDx: | |||
* | *Goodpasture syndrome with 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== | ||
* | ===General=== | ||
*Collagen IV mutation - the same protein is involved in ''[[Alport syndrome]]''. | |||
Clinical: | |||
* | *Hematuria. | ||
*FHx. | |||
*Nonprogressive. | |||
=== | ===Microscopic=== | ||
* | *Normal. | ||
=== | ===IF=== | ||
*Normal. | *Normal. | ||
=== | ===EM=== | ||
*GBM thin <200-250 - '''key feature'''. | |||
Note: | Note: | ||
* | *Normal GBM: 300-350 nm. | ||
==Alport | ==Alport syndrome== | ||
=== | ===General=== | ||
Clinical: | |||
*Hearing loss (sensorineural). | *Hearing loss (sensorineural). | ||
*Hematuria - usually preceeds hearing loss.<ref name=emedicine981126>[http://emedicine.medscape.com/article/981126-overview http://emedicine.medscape.com/article/981126-overview]</ref> | *Hematuria - usually preceeds hearing loss.<ref name=emedicine981126>URL: [http://emedicine.medscape.com/article/981126-overview http://emedicine.medscape.com/article/981126-overview]</ref> | ||
*Can be thought of a pathologic form of ''thin basement membrane disease''.<ref>AM. 13 August 2009.</ref> | *Can be thought of a pathologic form of ''[[thin basement membrane disease]]''.<ref>AM. 13 August 2009.</ref> | ||
Etiology: | |||
*Genetic defect - collagen type IV. | *Genetic defect - collagen type IV. | ||
Line 316: | Line 1,007: | ||
*Autosomal dominant - 5%. | *Autosomal dominant - 5%. | ||
== | ===Microscopic=== | ||
Features:<ref name=pmid9727383/> | |||
* | *Usu. normal. | ||
* | *+/-Foamy appearing renal tubular cells.<ref name=Ref_Klatt246>{{Ref Klatt|246}}</ref> | ||
===IF=== | |||
*Negative. | |||
== | ===EM=== | ||
Features:<ref name=pmid9727383>{{Cite journal | last1 = Kashtan | first1 = CE. | title = Alport syndrome and thin glomerular basement membrane disease. | journal = J Am Soc Nephrol | volume = 9 | issue = 9 | pages = 1736-50 | month = Sep | year = 1998 | doi = | PMID = 9727383 | url=http://jasn.asnjournals.org/content/9/9/1736.long}}</ref> | |||
*Abnormal glomerular basement membrane (GBM); thinning or thickening. | |||
**Classically thinning with thick lamellation (splitting/multi-layering). | |||
==Idiopathic nodular glomerulosclerosis== | |||
===General=== | ===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== | |||
:See: ''[[Haematopathology]]''. | |||
*[[AKA]] ''myeloma kidney''. | |||
===Myeloma cast nephropathy=== | |||
====General==== | |||
*Renal failure. | |||
====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> | |||
*Crap in tubules, eosinophilic. | |||
**Classically angulated. | |||
**Refractile. | |||
*Cast with cellular reaction - '''virtually diagnostic'''. | |||
**Macrophages (CD68 +ve). | |||
=====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.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==== | |||
*Myeloma casts = PAS -ve. | |||
**Hyaline casts = PAS +ve. | |||
===Amyloidosis=== | |||
{{Main|Amyloidosis}} | |||
*Usually associated with lambda clone. | |||
===Light chain deposition=== | |||
*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: | 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: | 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== | |||
{{Main|Cystic kidney diseases}} | |||
These are discussed in a separate article and include: | |||
*[[Autosomal dominant polycystic kidney disease]] (ADPKD). | |||
*Adult-onset medullary cystic disease. | |||
*[[Acquired renal cystic disease]]. | |||
*[[Autosomal recessive polycystic kidney disease]] (ARPKD). | |||
*Medullary sponge kidney. | |||
*Nephronophthisis. | |||
*Cystic [[renal cell carcinoma]]. | |||
=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=== | ===Gross=== | ||
Features: | 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=== | ===Microscopic=== | ||
Features:<ref name= | 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== | |||
:''See: [[hyperplastic arteriolosclerosis]]''. | |||
* | :''See: [[thrombotic microangiopathy]]''. | ||
*May be seen in [[scleroderma]]. | |||
== | ==Acute tubular necrosis== | ||
*Often abbreviated ''ATN''. | |||
===General=== | |||
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=== | |||
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> | |||
*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. | |||
Notes - not particularily useful findings: | |||
*Mitoses. | |||
*Casts in tubules. | |||
===IHC=== | |||
*Ki-67 - focal nuclear staining of the tubular epithelium.<ref name=pmid19207286/> | |||
==Hepatorenal syndrome== | |||
===General=== | |||
*Acute renal failure secondary to liver failure (e.g. fulminant liver failure, [[cirrhosis]] with marginal liver function). | |||
Clinical: | |||
*Urine sodium is low,<ref name=pmid1261103>{{cite journal |author=Epstein M, Oster JR, de Velasco RE |title=Hepatorenal syndrome following hemihepatectomy |journal=Clin. Nephrol. |volume=5 |issue=3 |pages=129-33 |year=1976 |month=March |pmid=1261103 |doi= |url=}}</ref> unlike in ATN (the main DDx). | |||
Pathophysiology: | |||
*Renal vasoconstriction.<ref name=pmid18304678>{{cite journal |author=Angeli P, Merkel C |title=Pathogenesis and management of hepatorenal syndrome in patients with cirrhosis |journal=J. Hepatol. |volume=48 Suppl 1 |issue= |pages=S93-103 |year=2008 |pmid=18304678 |doi=10.1016/j.jhep.2008.01.010 |url=}}</ref> | |||
Treatment: | |||
Medical and surgical:<ref name=pmid18417039>{{cite journal |author=Wong F |title=Hepatorenal syndrome: current management |journal=Curr Gastroenterol Rep |volume=10 |issue=1 |pages=22-9 |year=2008 |month=February |pmid=18417039 |doi= |url=}}</ref> | |||
*Vasoconstrictors (e.g. midodrine, terlipressin (counteracts splanchnic vasodilation), norepinephrine). | |||
*Albumin. | |||
*TIPS (transjugular intrahepatic portosystemic shunt). | |||
*Liver transplantation. | |||
Note: | |||
*I suspect a ''portal vein pump'' would work... it reduces portal pressure and would likely increase hepatic function. | |||
===Microscopic=== | |||
Features (kidney): | |||
*Normal. | |||
==Nephrocalcinosis== | |||
===General=== | ===General=== | ||
* | *[[Hypercalcemia]]. | ||
===Microscopic=== | ===Microscopic=== | ||
Features: | Features: | ||
*Calcification of the renal parenchyma - '''key feature'''. | |||
Images: | |||
*[http://path.upmc.edu/cases/case514.html Nephrocalcinosis (upmc.edu)]. | |||
=Renal transplant pathology= | |||
{{Main|Renal transplant pathology}} | |||
*Acute rejection. | |||
* | *Chronic rejection. | ||
* | *[[Polyomavirus]]. | ||
*[[Transplant glomerulopathy]]. | |||
*Calcineurin-inhibitor toxicity. | |||
=See also= | |||
*[[Kidney tumours]]. | *[[Kidney tumours]]. | ||
*[[Urinary bladder]]. | *[[Urinary bladder]]. | ||
*[[Genitourinary pathology]]. | *[[Genitourinary pathology]]. | ||
*[[Thrombotic microangiopathy]]. | |||
=References= | |||
{{reflist|2}} | {{reflist|2}} | ||
[[Category: | =External links= | ||
*[http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/rnfrm.html Nephropathology quiz (med.utah.edu)]. | |||
*[http://www.fondazionedamico.org/biopsiarenale_atlas/seco/crio/crio.htm Cryoglobulinemic nephritis (fondazionedamico.org)]. | |||
[[Category: Medical kidney pathology]] |
edits