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Difference between revisions of "Nephrotic syndrome"
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'''Nephrotic syndrome''' is a constellation of clinical findings seen in a number of [[medical kidney diseases]]. This article deals with them. | '''Nephrotic syndrome''' is a constellation of clinical findings seen in a number of [[medical kidney diseases]]. This article deals with them. It should '''not''' be confused with ''[[nephritic syndrome]]'' which has an almost identical spelling. | ||
In children nephrotic syndrome is assumed to be ''minimal change disease''. Biopsies are done only there is no response to | An introduction to the medical kidney is in the ''[[medical kidney diseases]]'' article. In children nephrotic syndrome is assumed to be ''minimal change disease''. Biopsies are done only there is no response to steroids. | ||
=Clinical definition= | =Clinical definition= | ||
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Immune complex positive: | Immune complex positive: | ||
#[[MN]]. | #[[MN]]. | ||
#[[IgA nephropathy]]. | #[[IgA nephropathy]].<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> | ||
Other: | |||
*Pre-eclampsia.<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> | |||
=Specific entities= | =Specific entities= | ||
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Features: | Features: | ||
*No changes on light microscopy. | *No changes on light microscopy. | ||
DDx: | |||
*[[Focal segmental glomerulosclerosis]] - that is undersampled. | |||
===EM=== | ===EM=== | ||
| Line 45: | Line 50: | ||
===General=== | ===General=== | ||
*Presents as nephrotic syndrome. | *Presents as nephrotic syndrome. | ||
*Does not respond to steroids (unlike MCD). | *Does not respond to steroids (unlike [[MCD]]). | ||
*Usually less rapid onset than MCD. | |||
**Fibrosis usually takes some time. | |||
====Etiology==== | ====Etiology==== | ||
| Line 51: | Line 58: | ||
**May be familial.<ref name=pmid12778884>{{cite journal |author=Sánchez de la Nieta MD, Arias LF, Alcázar R, ''et al.'' |title=[Familial focal and segmentary hyalinosis] |language=Spanish; Castilian |journal=Nefrologia |volume=23 |issue=2 |pages=172–6 |year=2003 |pmid=12778884 |doi= |url=}}</ref> | **May be familial.<ref name=pmid12778884>{{cite journal |author=Sánchez de la Nieta MD, Arias LF, Alcázar R, ''et al.'' |title=[Familial focal and segmentary hyalinosis] |language=Spanish; Castilian |journal=Nefrologia |volume=23 |issue=2 |pages=172–6 |year=2003 |pmid=12778884 |doi= |url=}}</ref> | ||
*Secondary.<ref>URL: [http://www.kidneypathology.com/English_version/Focal_segmental_GS.html http://www.kidneypathology.com/English_version/Focal_segmental_GS.html]. Accessed on: 11 February 2011.</ref> | *Secondary.<ref>URL: [http://www.kidneypathology.com/English_version/Focal_segmental_GS.html http://www.kidneypathology.com/English_version/Focal_segmental_GS.html]. Accessed on: 11 February 2011.</ref> | ||
**HIV. | **HIV, [[parvovirus B19]].<ref name=pmid12704581/> | ||
**Drug use. | **Drug use. | ||
**Reduced renal mass. | **Reduced renal mass. | ||
| Line 87: | Line 94: | ||
*Partial sclerosis of less than 50% of glomeruli. | *Partial sclerosis of less than 50% of glomeruli. | ||
*+/-Adhesions between the glomerular tuft and Bowman's capsule. | *+/-Adhesions between the glomerular tuft and Bowman's capsule. | ||
*+/-Glomerular enlargement. | |||
DDx: | |||
*[[Minimal change disease]]. | |||
*[[C1q nephropathy]]. | |||
Images: | |||
*[http://library.med.utah.edu/WebPath/jpeg1/RENAL083.jpg FSGS (utah.edu)]. | *www: | ||
**[http://library.med.utah.edu/WebPath/jpeg1/RENAL083.jpg FSGS (utah.edu)]. | |||
*[[WC]]: | |||
**[http://commons.wikimedia.org/wiki/File:Focal_segmental_glomerulosclerosis_-_high_mag.jpg FSGS - high mag. (WC)]. | |||
**[http://commons.wikimedia.org/wiki/File:Collapsing_glomerulopathy_-_high_mag.jpg Collapsing glomerulopathy - high mag. (WC)]. | |||
**[http://commons.wikimedia.org/wiki/File:Collapsing_glomerulopathy_-_very_high_mag.jpg Collapsing glomerulopathy - very high mag. (WC)]. | |||
====Histologic classification==== | ====Histologic classification==== | ||
| Line 100: | Line 117: | ||
|abrupt severe onset | |abrupt severe onset | ||
|- | |- | ||
|Collapsing | |Collapsing ([[AKA]] collapsing glomerulopathy<ref name=pmid12704581>{{Cite journal | last1 = Schwimmer | first1 = JA. | last2 = Markowitz | first2 = GS. | last3 = Valeri | first3 = A. | last4 = Appel | first4 = GB. | title = Collapsing glomerulopathy. | journal = Semin Nephrol | volume = 23 | issue = 2 | pages = 209-18 | month = Mar | year = 2003 | doi = 10.1053/snep.2003.50019 | PMID = 12704581 }}</ref>) | ||
|poor prognosis, viral/toxic etiology | |poor prognosis, viral/toxic etiology | ||
|- | |- | ||
| Line 134: | Line 151: | ||
===General=== | ===General=== | ||
*Presents as nephrotic syndrome. | *Presents as [[nephrotic syndrome]]. | ||
*Variable course. | *Variable course. | ||
*Pathogenesis: autoantibodies directed against ''phospholipase A2 receptor 1'', abbreviated PLA2R.<ref name=pmid22388552>{{Cite journal | last1 = Glassock | first1 = RJ. | title = The pathogenesis of membranous nephropathy: evolution and revolution. | journal = Curr Opin Nephrol Hypertens | volume = | issue = | pages = | month = Mar | year = 2012 | doi = 10.1097/MNH.0b013e3283522ea8 | PMID = 22388552 }}</ref><ref name=omim604939>{{OMIM|604939}}</ref> | |||
Clinical DDx:<ref>{{Ref Klatt|241}}</ref> | Clinical DDx:<ref>{{Ref Klatt|241}}</ref> | ||
*Hepatitis B. | *[[Hepatitis B]]. | ||
*Hepatitis C. | *[[Hepatitis C]]. | ||
*Carcinoma. | *Carcinoma. | ||
*NSAID toxicity. | *[[NSAID]] toxicity. | ||
*SLE. | *[[SLE]]. | ||
*Idiopathic. | *Idiopathic. | ||
===Microscopic=== | ===Microscopic=== | ||
Features: | Features: | ||
* | *Rigid (thickened) loop-like (glomerular) capillaries due to subepithelial immune complex deposition: | ||
**Spikes or pinholes - seen on silver stain. | **Spikes or pinholes - seen on silver stain - '''key light microscopy feature'''. | ||
**+/-Tram-tracking | **+/-Tram-tracking - seen in advanced lesions. | ||
Note: | |||
*www: | *Normal glomerular capillaries have a wavy wall with some undulation. | ||
*Early lesions may have an essential normal appearance on light microscopy. | |||
Lame memory device ''Oh ABCDEs'': | |||
*Nephr'''o'''tic syndrome (caused by) '''a'''utoimmune disorders, hepatitis '''B''', '''c'''ancer, '''d'''rugs (with), sub'''e'''pithelial deposits & '''s'''pikes. | |||
====Images==== | |||
www: | |||
*[http://www.flickr.com/photos/jian-hua_qiao_md/3989875091/in/set-72157622411941607 MN - silver stain (flickr.com)]. | |||
<gallery> | |||
Image:Membranous_nephropathy_-_he_-_very_high_mag.jpg | MN - very high mag. (WC) | |||
Image:Membranous_nephropathy_-_pas_-_very_high_mag.jpg | MN - PAS - very high mag. (WC) | |||
Image:Membranous_nephropathy_-_mpas_-_very_high_mag.jpg | MN - MPAS - very high mag. (WC) | |||
Image:Membranous_nephropathy_-_cropped_-_mpas_-_very_high_mag.jpg | MN - MPAS - very high mag. (WC) | |||
</gallery> | |||
===IF=== | ===IF=== | ||
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===EM=== | ===EM=== | ||
*Diffuse subepithelial deposits - spike forming. | *Diffuse subepithelial deposits - spike forming. | ||
**The size of the deposits measured perpendicular to the basement membrane is smaller than seen in [[post-infectious glomerulonephritis]] (PIGN), and more diffuse. In other words, MN has a thin band-like deposits. PIGN has scattered tombstone-like ones. | |||
Image: | |||
*[http://missinglink.ucsf.edu/lm/IDS_102_cases_glomerular/images/membranous_EM.jpg MN (ucsf.edu)].<ref>URL: [http://missinglink.ucsf.edu/lm/IDS_102_cases_glomerular/Nephrotic_syndrome.htm http://missinglink.ucsf.edu/lm/IDS_102_cases_glomerular/Nephrotic_syndrome.htm]. Accessed on: 2 April 2012.</ref> | |||
==C1q nephropathy== | ==C1q nephropathy== | ||
===General=== | ===General=== | ||
*Distinct entity<ref name=pmid3875286>{{Cite journal | last1 = Jennette | first1 = JC. | last2 = Hipp | first2 = CG. | title = C1q nephropathy: a distinct pathologic entity usually causing nephrotic syndrome. | journal = Am J Kidney Dis | volume = 6 | issue = 2 | pages = 103-10 | month = Aug | year = 1985 | doi = | PMID = 3875286 }}</ref> ''or'' a variant of FSGS | *Distinct entity<ref name=pmid3875286>{{Cite journal | last1 = Jennette | first1 = JC. | last2 = Hipp | first2 = CG. | title = C1q nephropathy: a distinct pathologic entity usually causing nephrotic syndrome. | journal = Am J Kidney Dis | volume = 6 | issue = 2 | pages = 103-10 | month = Aug | year = 1985 | doi = | PMID = 3875286 }}</ref> ''or'' a variant of [[focal segmental glomerulosclerosis]] (FSGS)<ref name=pmid12969141>{{Cite journal | last1 = Markowitz | first1 = GS. | last2 = Schwimmer | first2 = JA. | last3 = Stokes | first3 = MB. | last4 = Nasr | first4 = S. | last5 = Seigle | first5 = RL. | last6 = Valeri | first6 = AM. | last7 = D'Agati | first7 = VD. | title = C1q nephropathy: a variant of focal segmental glomerulosclerosis. | journal = Kidney Int | volume = 64 | issue = 4 | pages = 1232-40 | month = Oct | year = 2003 | doi = 10.1046/j.1523-1755.2003.00218.x | PMID = 12969141 }}</ref> -- entity is controversial.<ref>{{Cite journal | last1 = Reeves-Daniel | first1 = AM. | last2 = Iskandar | first2 = SS. | last3 = Bowden | first3 = DW. | last4 = Bostrom | first4 = MA. | last5 = Hicks | first5 = PJ. | last6 = Comeau | first6 = ME. | last7 = Langefeld | first7 = CD. | last8 = Freedman | first8 = BI. | title = Is collapsing C1q nephropathy another MYH9-associated kidney disease? A case report. | journal = Am J Kidney Dis | volume = 55 | issue = 5 | pages = e21-4 | month = May | year = 2010 | doi = 10.1053/j.ajkd.2009.10.060 | PMID = 20116156 }}</ref> | ||
*Nephrotic syndrome. | *Nephrotic syndrome. | ||
*Steroid resistant - like FSGS.<ref name=pmid3875286>{{Cite journal | last1 = Jennette | first1 = JC. | last2 = Hipp | first2 = CG. | title = C1q nephropathy: a distinct pathologic entity usually causing nephrotic syndrome. | journal = Am J Kidney Dis | volume = 6 | issue = 2 | pages = 103-10 | month = Aug | year = 1985 | doi = | PMID = 3875286 }}</ref> | *Steroid resistant - like FSGS.<ref name=pmid3875286>{{Cite journal | last1 = Jennette | first1 = JC. | last2 = Hipp | first2 = CG. | title = C1q nephropathy: a distinct pathologic entity usually causing nephrotic syndrome. | journal = Am J Kidney Dis | volume = 6 | issue = 2 | pages = 103-10 | month = Aug | year = 1985 | doi = | PMID = 3875286 }}</ref> | ||
*[[ANA]] -ve. | |||
===Microscopic=== | ===Microscopic=== | ||
| Line 180: | Line 209: | ||
===IF=== | ===IF=== | ||
*C1q +ve. | *C1q +ve - '''key feature'''. | ||
=See also= | =See also= | ||