Nephrotic syndrome differential diagnosis: Difference between revisions

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__NOTOC__
__NOTOC__
{{Nephrotic syndrome}}
[[Image:Home_logo1.png|right|250px|link=https://www.wikidoc.org/index.php/Nephrotic_syndrome]]
{{CMG}}; {{AE}} [[User:YazanDaaboul|Yazan Daaboul]], [[User:Sergekorjian|Serge Korjian]]
 
{{CMG}}; {{AE}}{{MJ}}


==Overview==
==Overview==
The differential diagnosis of nephrotic syndrome includes other diseases with similar findings on physical examination, such as [[lower extremity edema]], or similar clinical findings, such as [[hypoalbuminemia]].  Differential diagnoses include [[congestive heart failure]], [[liver cirrhosis]], [[protein losing enteropathy]], [[malignancy]], [[lymphedema]], [[venous insufficiency]], and [[malnutrition]].
Nephrotic syndrome should be differentiate from other causes of [[glomerular]] disease such as [[Fabry's disease]], [[post-streptococcal glomerulonephritis]], [[lupus nephritis]], [[Goodpasture syndrome|antiglomerular basement membrane disease]] [[Goodpasture syndrome|(goodpasture's syndrome)]], [[Cryoglobulinemia]], [[Henoch-Schönlein purpura]], [[amyloidosis]], pulmonary-renal syndromes ([[vasculitis]]), [[thin basement membrane disease]], [[Alport syndrome|Alport's Syndrome]], [[Goodpasture syndrome|anti-GBM Disease]], [[hypertensive nephrosclerosis]], and [[subacute bacterial endocarditis]]. The various types of glomerular diseases may be differentiated from each other based on associations, presence of [[pitting edema]], [[hematuria]], [[hypertension]], [[hemoptysis]], [[oliguria]], peri-orbital [[edema]], [[hyperlipidemia]], type of [[antibodies]], [[Light microscope|light]], and [[Electron microscopy|electron microscopic]] features.  


==Differential Diagnosis==
==Differential Diagnosis==
Nephrotic syndrome should be differentiate from other causes of glomerular disease. The various types of glomerular diseases may be differentiated from each other based on associations, presence of [[pitting edema]], hemeturia, [[hypertension]], [[hemoptysis]], [[oliguria]], peri-orbital edema, [[hyperlipidemia]], type of [[antibodies]], [[Light microscope|light]] and [[Electron microscopy|electron microscopic]] features. The following table differentiates between various types of glumerular diseases:
<small>
{| class="wikitable"
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Glomerular diseases
! colspan="2" rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
! colspan="9" align="center" style="background:#4479BA; color: #FFFFFF;" + |History and Symtoms
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Laboratory Findings
! colspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Pathology
|-
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Systemic symptoms
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemeturia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Proteinuria
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hypertension
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Pitting edema
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Oliguria
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Nephrotic features
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Nephritic features
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hyperlipidemia and hypercholesterolemia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Auto-antibodies,
Complements
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Light microscope
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Electron microscope
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Immunoflourescence pattern
|-
! rowspan="13" align="center" style="background:#4479BA; color: #FFFFFF;" + |Acute Nephritic Syndromes
! colspan="2" |[[Poststreptococcal glomerulonephritis|Poststreptococcal Glomerulonephritis]]<ref name="pmid13022878">{{cite journal| author=GERMUTH FG| title=A comparative histologic and immunologic study in rabbits of induced hypersensitivity of the serum sickness type. | journal=J Exp Med | year= 1953 | volume= 97 | issue= 2 | pages= 257-82 | pmid=13022878 | doi= | pmc=PMC2136196 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13022878 }}</ref><ref name="pmid5031005">{{cite journal| author=Germuth FG, Senterfit LB, Dreesman GR| title=Immune complex disease. V. The nature of the circulating complexes associated with glomerular alterations in the chronic BSA-rabbit system. | journal=Johns Hopkins Med J | year= 1972 | volume= 130 | issue= 6 | pages= 344-57 | pmid=5031005 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5031005 }}</ref><ref name="pmid22895519">{{cite journal| author=Radhakrishnan J, Cattran DC| title=The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient. | journal=Kidney Int | year= 2012 | volume= 82 | issue= 8 | pages= 840-56 | pmid=22895519 | doi=10.1038/ki.2012.280 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22895519 }}</ref>
|
* [[Streptococcal infections|Streptococcal]] [[skin]] [[infections]]
* [[Streptococcus|Streptococcal]] [[pharyngitis]]
* 2-3 weeks after [[infection]]
|
* [[Fever]]
* [[Fatigue]]
* Skin [[rash]]
|<nowiki>+/-</nowiki>
| +
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|
* [[Anti-dsDNA antibody|Anti-dsDNA antibodies]]
* Anti-C1q antibodies
* [[Antineutrophil cytoplasmic antibodies]] ([[ANCA]])
|
* Hypercellular and [[inflamed]] [[glomeruli]]
|
* Sub-[[epithelial]] [[immune complex]] deposits
* Obliteration of epithelial cell foot processes
|
* [[Immune]] complex GN
* Granular deposit
|-
! colspan="2" |[[Renal]] disease due to [[Endocarditis|Subacute Bacterial Endocarditis]], or [[cardiac shunt]] (Atrioventricular)<ref name="pmid6380288">{{cite journal |vauthors=Neugarten J, Baldwin DS |title=Glomerulonephritis in bacterial endocarditis |journal=Am. J. Med. |volume=77 |issue=2 |pages=297–304 |date=August 1984 |pmid=6380288 |doi= |url=}}</ref><ref name="pmid6831779">{{cite journal |vauthors=Arze RS, Rashid H, Morley R, Ward MK, Kerr DN |title=Shunt nephritis: report of two cases and review of the literature |journal=Clin. Nephrol. |volume=19 |issue=1 |pages=48–53 |date=January 1983 |pmid=6831779 |doi= |url=}}</ref>
|
* History of infective endocarditis mostly due to ''[[Staphylococcus aureus|S. aureus]]''
* [[Cardiac shunt]]
|
* [[Fever]]
* [[Fatigue]]
* Weight loss
|<nowiki>+/-</nowiki>
| +
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|
* [[Myeloperoxidase (MPO)|ANCA (myeloperoxidase)]] positive in 1/3
* Activation of the [[alternative complement pathway]] (Decreased [[C3 (complement)|C3]], C4)
* Positive [[RF]]
* Positive [[Anti-glomerular basement membrane antibody|anti-GBM autoantibodies]]
|
* [[Rapidly progressive glomerulonephritis|Crescentic]] GN is the most common pathological features
* [[Membranoproliferative glomerulonephritis|Diffuse membranoproliferative glomerulonephritis]] features
* Focal proliferative GN
* Mesangial proliferative GN 
|
* [[Mesangial cell|Mesangial]] deposits,
* Subendothelial deposits
* Subepithelial "humps," in minority of cases
|
* Pauci-immune GN
|-
! colspan="2" |[[Lupus nephritis|Lupus Nephritis]]<ref name="pmid14717922">{{cite journal |vauthors=Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, Balow JE, Bruijn JA, Cook T, Ferrario F, Fogo AB, Ginzler EM, Hebert L, Hill G, Hill P, Jennette JC, Kong NC, Lesavre P, Lockshin M, Looi LM, Makino H, Moura LA, Nagata M |title=The classification of glomerulonephritis in systemic lupus erythematosus revisited |journal=Kidney Int. |volume=65 |issue=2 |pages=521–30 |date=February 2004 |pmid=14717922 |doi=10.1111/j.1523-1755.2004.00443.x |url=}}</ref>
|
* History of [[SLE]] features
|
* [[Systemic lupus erythematosus|Lupus]] criteria:
** [[Malar rash]]
** [[Arthritis]]
** [[Arthralgia]]
** [[Anemia]]
** Easy bruising
|<nowiki>+/-</nowiki>
| +
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|
* Anti-C1q antibodies
* [[Anti-dsDNA antibody|Anti-dsDNA]]
|
* Differs based on the disease classification
|
* Differs based on the disease classification
|
* Differs based on the disease classification, mostly immune complex GN
* Granular deposit
|-
! colspan="2" |[[Goodpasture syndrome|Antiglomerular Basement Membrane Disease]] [[Goodpasture syndrome|(Goodpasture's syndrome)]]<ref name="pmid8914046">{{cite journal |vauthors=Bolton WK |title=Goodpasture's syndrome |journal=Kidney Int. |volume=50 |issue=5 |pages=1753–66 |date=November 1996 |pmid=8914046 |doi= |url=}}</ref><ref name="pmid1090223">{{cite journal |vauthors=Mathew TH, Hobbs JB, Kalowski S, Sutherland PW, Kincaid-Smith P |title=Goodpasture's syndrome: normal renal diagnostic findings |journal=Ann. Intern. Med. |volume=82 |issue=2 |pages=215–8 |date=February 1975 |pmid=1090223 |doi= |url=}}</ref>
|
* Young adults
|
* Dry [[cough]]
* [[Hemoptysis]]
* [[Malaise]] 
* [[Fever]] and chills
* [[Arthralgia]] 
* [[Fatigue]]
* [[Lethargy]]
* [[Pallor]]
* [[Anorexia]]
* Easy bruising
|<nowiki>+</nowiki>
| +
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|
* [[Myeloperoxidase (MPO)|ANCA (myeloperoxidase)]]
* Positive [[Anti-glomerular basement membrane antibody|anti-GBM autoantibodies]]
|
* Hypercellular and [[inflamed]] [[glomeruli]] (Crescent formation) 
|Diffuse thickening of the [[glomerular basement membrane]] with absence of sub-[[Epithelial cells|epithelial]] and sub-[[endothelial]] deposits 
|
* Immune complex GN
* Linear deposit
|-
! colspan="2" |[[IgA nephropathy|IgA Nephropathy]]<ref name="pmid21949093">{{cite journal |vauthors=Suzuki H, Kiryluk K, Novak J, Moldoveanu Z, Herr AB, Renfrow MB, Wyatt RJ, Scolari F, Mestecky J, Gharavi AG, Julian BA |title=The pathophysiology of IgA nephropathy |journal=J. Am. Soc. Nephrol. |volume=22 |issue=10 |pages=1795–803 |date=October 2011 |pmid=21949093 |pmc=3892742 |doi=10.1681/ASN.2011050464 |url=}}</ref><ref name="pmid23782179">{{cite journal |vauthors=Wyatt RJ, Julian BA |title=IgA nephropathy |journal=N. Engl. J. Med. |volume=368 |issue=25 |pages=2402–14 |date=June 2013 |pmid=23782179 |doi=10.1056/NEJMra1206793 |url=}}</ref>
|
* Young children
* History of [[mucosal]] [[infections]] (e.g. [[gastroenteritis]]) and [[upper respiratory tract infection]]
* 2-3 days after [[infection]] (synpharyngitic)
|
* Low grade [[fever]]
* [[Flank pain]]
|<nowiki>+</nowiki>
| +/-
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
| -
|
* Immune complex deposition
|
* Crescent formation
|
* [[Mesangial cell|Mesangial]] proliferation
|
* Immune complex GN, granular deposite
|-
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Systemic symptoms
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemeturia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Proteinuria
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hypertension
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Pitting edema
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Oliguria
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Nephrotic features
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Nephritic features
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hyperlipidemia and hypercholesterolemia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Auto-antibodies,
Complements
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Light microscope
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Electron microscope
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Immunoflourescence pattern
|-
! rowspan="3" |[[Vasculitis|ANCA Small-Vessel Vasculitis]]<ref name="pmid8746284">{{cite journal |vauthors=Higgins RM, Goldsmith DJ, Connolly J, Scoble JE, Hendry BM, Ackrill P, Venning MC |title=Vasculitis and rapidly progressive glomerulonephritis in the elderly |journal=Postgrad Med J |volume=72 |issue=843 |pages=41–4 |date=January 1996 |pmid=8746284 |pmc=2398323 |doi= |url=}}</ref><ref name="pmid12631105">{{cite journal |vauthors=Jennette JC |title=Rapidly progressive crescentic glomerulonephritis |journal=Kidney Int. |volume=63 |issue=3 |pages=1164–77 |date=March 2003 |pmid=12631105 |doi=10.1046/j.1523-1755.2003.00843.x |url=}}</ref>
! colspan="1" |[[Granulomatosis with polyangiitis|Granulomatosis with Polyangiitis (Wegener's)]]<ref name="pmid18172777">{{cite journal |vauthors=Renaudineau Y, Le Meur Y |title=Renal involvement in Wegener's granulomatosis |journal=Clin Rev Allergy Immunol |volume=35 |issue=1-2 |pages=22–9 |date=October 2008 |pmid=18172777 |doi=10.1007/s12016-007-8066-6 |url=}}</ref><ref name="pmid6384024">{{cite journal |vauthors=Weiss MA, Crissman JD |title=Renal biopsy findings in Wegener's granulomatosis: segmental necrotizing glomerulonephritis with glomerular thrombosis |journal=Hum. Pathol. |volume=15 |issue=10 |pages=943–56 |date=October 1984 |pmid=6384024 |doi= |url=}}</ref><ref name="pmid18524109">{{cite journal |vauthors=Pagnoux C |title=[Wegener's granulomatosis and microscopic polyangiitis] |language=French |journal=Rev Prat |volume=58 |issue=5 |pages=522–32 |date=March 2008 |pmid=18524109 |doi= |url=}}</ref>
|
* Middle age male
|
* Constitutional symptoms
* [[Dyspnea]]
* [[Purpura]]
* [[Arthralgia|Arthralgias]]
* Neurologic dysfunction
* [[Rhinosinusitis|Sinusitis]]
* [[Otitis media]]
* [[Epistaxis]].
|<nowiki>+</nowiki>
| +
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
| -
|
* [[ANCA]]
|
* [[Necrotizing]] and [[Crescentic glomerulonephritis|crescentic]] glomerulonephritis
|
* Subendothelial [[edema]]
* Microthrombosis
* [[Degranulation]] of [[neutrophils]]
|
* Pauci-immune GN
|-
! colspan="1" |[[Microscopic polyangiitis|Microscopic Polyangiitis]]<ref name="pmid20688249">{{cite journal |vauthors=Chung SA, Seo P |title=Microscopic polyangiitis |journal=Rheum. Dis. Clin. North Am. |volume=36 |issue=3 |pages=545–58 |date=August 2010 |pmid=20688249 |pmc=2917831 |doi=10.1016/j.rdc.2010.04.003 |url=}}</ref>
|<nowiki>+/-</nowiki>
|
* Constitutional symptoms
* [[Dyspnea]]
* [[Purpura]]
* [[Arthralgia|Arthralgias]]
* Neurologic dysfunction
|<nowiki>+</nowiki>
| +
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
| +
|
|<nowiki>-</nowiki>
|
* [[P-ANCA]]
|
* Hypercellular and [[inflamed]] [[glomeruli]] (Crescent formation)
|
* Hypercellular and [[inflamed]] [[glomeruli]] (Crescent formation)
|
* Pauci-immune GN
|-
! colspan="1" |[[Eosinophilic granulomatosis with polyangiitis|Churg-Strauss Syndrome]]<ref name="pmid16632015">{{cite journal |vauthors=Sinico RA, Di Toma L, Maggiore U, Tosoni C, Bottero P, Sabadini E, Giammarresi G, Tumiati B, Gregorini G, Pesci A, Monti S, Balestrieri G, Garini G, Vecchio F, Buzio C |title=Renal involvement in Churg-Strauss syndrome |journal=Am. J. Kidney Dis. |volume=47 |issue=5 |pages=770–9 |date=May 2006 |pmid=16632015 |doi=10.1053/j.ajkd.2006.01.026 |url=}}</ref>
|<nowiki>+/-</nowiki>
|
*  [[Asthma]]
* [[Rhinosinusitis|Sinusitis]]
* [[Myalgia]] 
* [[Arthralgia]]
* [[Purpura]]
* [[Cardiac arrhythmia|Arrythmias]]
* [[Peripheral neuropathy]]
|<nowiki>+</nowiki>
| +
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|
|<nowiki>-</nowiki>
|
*  [[C-ANCA]]
|
* Hypercellular and [[inflamed]] [[glomeruli]] (Crescent formation)
|
* Hypercellular and [[inflamed]] [[glomeruli]] (Crescent formation)
|
* Pauci-immune GN
|-
! colspan="2" |[[Membranoproliferative glomerulonephritis|Membranoproliferative Glomerulonephritis]]<ref name="pmid19908070">{{cite journal |vauthors=Alchi B, Jayne D |title=Membranoproliferative glomerulonephritis |journal=Pediatr. Nephrol. |volume=25 |issue=8 |pages=1409–18 |date=August 2010 |pmid=19908070 |pmc=2887509 |doi=10.1007/s00467-009-1322-7 |url=}}</ref><ref name="pmid657595">{{cite journal |vauthors=Davis AE, Schneeberger EE, Grupe WE, McCluskey RT |title=Membranoproliferative glomerulonephritis (MPGN type I) and dense deposit disease (DDD) in children |journal=Clin. Nephrol. |volume=9 |issue=5 |pages=184–93 |date=May 1978 |pmid=657595 |doi= |url=}}</ref>
|
* [[Idiopathic]]
* [[Hepatitis B]] and [[Hepatitis C|C]] (Type 1)
* C3 nepritic factor (Type2)
|
* [[Hematuria]]
* [[Oliguria]]
* [[Periorbital edema]]
* [[Hypertension]]
|<nowiki>+</nowiki>
| +
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|
* Thick [[glomerular basement membrane]] (Tram-track appearance)
|
* [[Mesangial cell|Mesangial]] proliferation
* [[Leukocytes|Leukocyte]] infiltration
|
* Immune complex GN
* Granular deposite
|-
! colspan="2" |[[Henoch-Schönlein purpura]] <ref name="pmid8023818">{{cite journal |vauthors=Jennette JC, Falk RJ |title=The pathology of vasculitis involving the kidney |journal=Am. J. Kidney Dis. |volume=24 |issue=1 |pages=130–41 |date=July 1994 |pmid=8023818 |doi= |url=}}</ref>
|
* Most common in young male, following [[Upper respiratory tract|upper respiratory]]  infections
|
* '''Skin manifestations-''' Palpable [[purpura]] on buttocks
* '''Gastrointestinal''' '''manifestations-'''
** [[Abdominal pain]]
** [[Melena]]
** [[Dysentery|Bloody diarrhea]]
** [[Hematemesis]]
* '''Joints'''  '''manifestations-'''
** [[Arthralgia]]
|<nowiki>+</nowiki>
| +
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| -
|
* Diffuse mesangial IgA deposits often associated with mesangial hypercellularity
|
* Diffuse mesangial IgA deposits often associated with mesangial hypercellularity
|
* Immune complex GN, granular deposite
|-
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Systemic symptoms
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemeturia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Proteinuria
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hypertension
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Pitting edema
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Oliguria
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Nephrotic features
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Nephritic features
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hyperlipidemia and hypercholesterolemia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Auto-antibodies,
Complements
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Light microscope
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Electron microscope
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Immunoflourescence pattern
|-
! colspan="2" |[[Cryoglobulinemia]]<ref name="pmid26802335">{{cite journal |vauthors=Fogo AB, Lusco MA, Najafian B, Alpers CE |title=AJKD Atlas of Renal Pathology: Cryoglobulinemic Glomerulonephritis |journal=Am. J. Kidney Dis. |volume=67 |issue=2 |pages=e5–7 |date=February 2016 |pmid=26802335 |doi=10.1053/j.ajkd.2015.12.007 |url=}}</ref>
|Patients having cryoglobulinemia may have positive history of:
* [[Hepatitis C|Hepatitis C infection]]
* [[Hepatitis B|Hepatitis B infection]]
* Leg ulcers
* Recurrent [[thrombosis]]
|'''Pulmonary symptoms:'''
* [[Difficulty breathing]]
* Cough
'''Cutaneous symptoms:'''
* [[Purpura]] 
* [[Skin ulcer]]
'''Gastrointestinal symptoms:'''
* Abdominal pain
'''General symptoms:'''
* Fever
* [[Arthralgia]],
* [[Myalgia]]
* [[Fatigue]]
* [[Blurred vision|Blurring]]/loss of vision
* [[Diplopia]]
* [[Confusion]]
|<nowiki>+/-</nowiki>
| +
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|
* [[Membranoproliferative glomerulonephritis]]
|
* [[Mesangial cell|Mesangial]] and subendothelial deposits
|
* Prominent [[IgM]] and C3
|-
! rowspan="9" align="center" style="background:#4479BA; color: #FFFFFF;" + |Nephrotic Syndrome
! colspan="2" |[[Minimal change disease|Minimal Change Disease]]<ref name="pmid17195422">{{cite journal |vauthors=Saha TC, Singh H |title=Minimal change disease: a review |journal=South. Med. J. |volume=99 |issue=11 |pages=1264–70 |date=November 2006 |pmid=17195422 |doi=10.1097/01.smj.0000243183.87381.c2 |url=}}</ref><ref name="pmid27092244">{{cite journal |vauthors=Saleem MA, Kobayashi Y |title=Cell biology and genetics of minimal change disease |journal=F1000Res |volume=5 |issue= |pages= |date=2016 |pmid=27092244 |pmc=4821284 |doi=10.12688/f1000research.7300.1 |url=}}</ref>
| colspan="2" |
* Young children
* Recent [[infection]] and [[immunization]]
* [[Atopy]]
* [[Hodgkin's lymphoma|Hodgkin lymphoma]]
* [[Thrombosis]] (due to [[Urinary system|urinary]] loss of [[Antithrombin III|antithrombin-III]])
| -
| +
| -
| +
| +/-
| +
| -
| +
|
|
* Normal
|
* Fusion of [[podocytes]]
| -
|-
! colspan="2" |[[Focal segmental glomerulosclerosis|Focal Segmental Glomerulosclerosis]]<ref name="pmid28242845">{{cite journal |vauthors=Rosenberg AZ, Kopp JB |title=Focal Segmental Glomerulosclerosis |journal=Clin J Am Soc Nephrol |volume=12 |issue=3 |pages=502–517 |date=March 2017 |pmid=28242845 |pmc=5338705 |doi=10.2215/CJN.05960616 |url=}}</ref><ref name="pmid25168829">{{cite journal |vauthors=Jefferson JA, Shankland SJ |title=The pathogenesis of focal segmental glomerulosclerosis |journal=Adv Chronic Kidney Dis |volume=21 |issue=5 |pages=408–16 |date=September 2014 |pmid=25168829 |pmc=4149756 |doi=10.1053/j.ackd.2014.05.009 |url=}}</ref><ref name="pmid2429634">{{cite journal |vauthors=Gephardt GN, Tubbs RR, Popowniak KL, McMahon JT |title=Focal and segmental glomerulosclerosis. Immunohistologic study of 20 renal biopsy specimens |journal=Arch. Pathol. Lab. Med. |volume=110 |issue=10 |pages=902–5 |date=October 1986 |pmid=2429634 |doi= |url=}}</ref>
| colspan="2" |
* Idiopathic
* [[Human Immunodeficiency Virus (HIV)|HIV]]
* [[Heroine hydrochloride|Heroine]] use
* [[Sickle-cell disease|Sickle cell disease]]
* [[Interferon]]
* Severe [[obesity]]
* [[Cryoglobulinemia|Mixed cryoglobulinemia]] ([[Hepatitis C]])
|<nowiki>-</nowiki>
| +
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
| +
|<nowiki>-</nowiki>
| +
|
|
* Focal (some [[glomeruli]]) and segmental (only part of [[glomerulus]])
|
* Effacement of [[podocytes]]
|<nowiki>-</nowiki>
|-
! colspan="2" |[[Membranous glomerulonephritis|Membranous Glomerulonephritis]]<ref name="pmid25558821">{{cite journal |vauthors=Lai WL, Yeh TH, Chen PM, Chan CK, Chiang WC, Chen YM, Wu KD, Tsai TJ |title=Membranous nephropathy: a review on the pathogenesis, diagnosis, and treatment |journal=J. Formos. Med. Assoc. |volume=114 |issue=2 |pages=102–11 |date=February 2015 |pmid=25558821 |doi=10.1016/j.jfma.2014.11.002 |url=}}</ref><ref name="pmid10495797">{{cite journal |vauthors=Wasserstein AG |title=Membranous glomerulonephritis |journal=J. Am. Soc. Nephrol. |volume=8 |issue=4 |pages=664–74 |date=April 1997 |pmid=10495797 |doi= |url=}}</ref>
| colspan="2" |
* [[Idiopathic]]
* [[Hepatitis B]] and [[Hepatitis C|C]]
* [[Solid tumors]]
* [[Systemic lupus erythematosus]]
* Drugs ([[NSAIDS]], pencilamine, [[gold]], [[captopril]])
|<nowiki>-</nowiki>
| +
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
| +
|<nowiki>-</nowiki>
| +
|Immune complex deposition
|
* Thick [[glomerular basement membrane]]
|
* Sub-[[Epithelial cells|epithelial]] [[immune complex]] depositis with 'spike and dome' appearance
|Immune complex GN, granular deposite
|-
! colspan="2" |[[Diabetic nephropathy|Diabetic Nephropathy]]<ref name="pmid11978659">{{cite journal| author=Drummond K, Mauer M, International Diabetic Nephropathy Study Group| title=The early natural history of nephropathy in type 1 diabetes: II. Early renal structural changes in type 1 diabetes. | journal=Diabetes | year= 2002 | volume= 51 | issue= 5 |pages= 1580-7 | pmid=11978659 | doi= | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11978659  }}</ref><ref name="pmid3699305">{{cite journal| author=Hørlyck A, Gundersen HJ, Osterby R| title=The cortical distribution pattern of diabetic glomerulopathy. | journal=Diabetologia | year= 1986 | volume= 29| issue= 3 | pages= 146-50 | pmid=3699305 | doi= | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3699305  }}</ref><ref name="pmid21422926">{{cite journal| author=Alpers CE, Hudkins KL| title=Mouse models of diabetic nephropathy. | journal=Curr Opin Nephrol Hypertens | year= 2011 | volume= 20 | issue= 3 |pages= 278-84 | pmid=21422926 | doi=10.1097/MNH.0b013e3283451901 | pmc=PMC3658822 |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21422926  }}</ref><ref name="pmid19970254">{{cite journal| author=Kimmelstiel P, Wilson C| title=Intercapillary Lesions in the Glomeruli of the Kidney. | journal=Am J Pathol | year= 1936 | volume= 12 | issue= 1 |pages= 83-98.7 | pmid=19970254 | doi= | pmc=PMC1911022 |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19970254  }}</ref><ref name="pmid2766585">{{cite journal| author=Alpers CE, Biava CG| title=Idiopathic lobular glomerulonephritis (nodular mesangial sclerosis): a distinct diagnostic entity. | journal=Clin Nephrol | year= 1989 | volume= 32 | issue= 2 | pages= 68-74 | pmid=2766585 | doi= | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2766585  }}</ref><ref name="pmid17536064">{{cite journal| author=Toyoda M, Najafian B, Kim Y, Caramori ML, Mauer M| title=Podocyte detachment and reduced glomerular capillary endothelial fenestration in human type 1 diabetic nephropathy. | journal=Diabetes | year= 2007 | volume= 56 | issue= 8 | pages= 2155-60 |pmid=17536064 | doi=10.2337/db07-0019 | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17536064  }}</ref><ref name="pmid16565248">{{cite journal| author=Najafian B, Crosson JT, Kim Y, Mauer M| title=Glomerulotubular junction abnormalities are associated with proteinuria in type 1 diabetes. | journal=J Am Soc Nephrol | year= 2006 | volume= 17 | issue= 4 Suppl 2 | pages= S53-60| pmid=16565248 | doi=10.1681/ASN.2005121342 | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16565248  }}</ref><ref name="pmid12660325">{{cite journal| author=Najafian B, Kim Y, Crosson JT, Mauer M| title=Atubular glomeruli and glomerulotubular junction abnormalities in diabetic nephropathy. | journal=J Am Soc Nephrol | year= 2003 | volume= 14 | issue= 4 | pages= 908-17 | pmid=12660325 | doi= | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12660325  }}</ref><ref name="pmid21659756">{{cite journal| author=Najafian B, Alpers CE, Fogo AB| title=Pathology of human diabetic nephropathy. |journal=Contrib Nephrol | year= 2011 | volume= 170 | issue=  | pages= 36-47 | pmid=21659756 |doi=10.1159/000324942 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659756  }}</ref><ref name="pmid216597562">{{cite journal| author=Najafian B, Alpers CE, Fogo AB| title=Pathology of human diabetic nephropathy. |journal=Contrib Nephrol | year= 2011 | volume= 170 | issue=  | pages= 36-47 | pmid=21659756 |doi=10.1159/000324942 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659756  }}</ref>
| colspan="2" |'''''For more information on diabetes [[Diabetes mellitus|click here]]'''.''
|<nowiki>-</nowiki>
| +
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
| +
|<nowiki>-</nowiki>
| +
|
|
* Diffuse [[Mesangial cell|mesangial]] matrix expansion (nodular glomerulosclerosis)
* Increased [[Mesangial cell|mesangial]] hypercellularity
* Prominent glomerular basement membranes
* Thick [[basement membrane]] without any deposit
|
* Nodular glomerulosclerosis
| -
|-
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Systemic symptoms
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemeturia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Proteinuria
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hypertension
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Pitting edema
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Oliguria
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Nephrotic features
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Nephritic features
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hyperlipidemia and hypercholesterolemia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Auto-antibodies,
Complements
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Light microscope
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Electron microscope
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Immunoflourescence pattern
|-
! rowspan="4" | [[Glomerular deposition disease|Glomerular Deposition Diseases]] 
![[Light chain nephropathy|Light Chain Deposition Disease]]<ref name="pmid21511832">{{cite journal |vauthors=Hutchison CA, Cockwell P, Stringer S, Bradwell A, Cook M, Gertz MA, Dispenzieri A, Winters JL, Kumar S, Rajkumar SV, Kyle RA, Leung N |title=Early reduction of serum-free light chains associates with renal recovery in myeloma kidney |journal=J. Am. Soc. Nephrol. |volume=22 |issue=6 |pages=1129–36 |date=June 2011 |pmid=21511832 |pmc=3103732 |doi=10.1681/ASN.2010080857 |url=}}</ref>
|
* Occurs in the setting of high tumor burden
| -
|<nowiki>-</nowiki>
| +
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
| +
|<nowiki>-</nowiki>
| +
| -
|
* Light-chain deposits
|
* Granular deposits on electron microscopy
|
* Detection of light chain deposits using anti–light chain antibody
|-
![[Amyloidosis|Renal Amyloidosis]]<ref name="pmid23227278">{{cite journal |vauthors=Baker KR, Rice L |title=The amyloidoses: clinical features, diagnosis and treatment |journal=Methodist Debakey Cardiovasc J |volume=8 |issue=3 |pages=3–7 |date=2012 |pmid=23227278 |pmc=3487569 |doi= |url=}}</ref><ref name="pmid23979488">{{cite journal |vauthors=Gillmore JD, Hawkins PN |title=Pathophysiology and treatment of systemic amyloidosis |journal=Nat Rev Nephrol |volume=9 |issue=10 |pages=574–86 |date=October 2013 |pmid=23979488 |doi=10.1038/nrneph.2013.171 |url=}}</ref><ref name="pmid26155101">{{cite journal |vauthors=Jerzykowska S, Cymerys M, Gil LA, Balcerzak A, Pupek-Musialik D, Komarnicki MA |title=Primary systemic amyloidosis as a real diagnostic challenge - case study |journal=Cent Eur J Immunol |volume=39 |issue=1 |pages=61–6 |date=2014 |pmid=26155101 |pmc=4439975 |doi=10.5114/ceji.2014.42126 |url=}}</ref><ref name="pmid16409147">{{cite journal |vauthors=Pepys MB |title=Amyloidosis |journal=Annu. Rev. Med. |volume=57 |issue= |pages=223–41 |date=2006 |pmid=16409147 |doi=10.1146/annurev.med.57.121304.131243 |url=}}</ref>
|
* For the secondary causes of amloidosis:
* [[Tuberculosis]]
* [[Familial mediterranean fever|Familial Mediterranean fever]]
* [[Rheumatoid arthritis]]
* [[Multiple myeloma]]
|
* [[Dyspnea]]
* [[Lethargy]]
* Weight loss


{| border="1" style="border-collapse:collapse; text-align:left;" cellpadding="5" align="center"
* [[Hemorrhagic diathesis|Bleeding tendency]]
|+ '''''Common Differential Diagnoses of Nephrotic Syndrome'''''
|<nowiki>-</nowiki>
| bgcolor="#d9ff54"|'''Differential Diagnosis''' || bgcolor="#d9ff54"|'''Characteristic Features'''
| +
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
| +
|<nowiki>-</nowiki>
| +
| -
|
* Diffuse glomerular deposition of amorphous hyaline material (nodular pattern), in mesangium (weakly staining with periodic acid-Schiff (PAS)
|
* Nodular deposit
|
* AA amyloidosis type: negative for immunoglobulins and complement
* AL amyloidosis type: Positive for lambda or kappa light chains
|-
|-
| bgcolor="#ececec"|'''[[Congestive Heart Failure]]''' ||
!Fibrillary-Immunotactoid Glomerulopathy<ref name="pmid1996564">{{cite journal |vauthors=Korbet SM, Schwartz MM, Lewis EJ |title=Immunotactoid glomerulopathy |journal=Am. J. Kidney Dis. |volume=17 |issue=3 |pages=247–57 |date=March 1991 |pmid=1996564 |doi= |url=}}</ref>
*History of [[cardiovascular disease]]
|
*[[Dyspnea]], [[orthopnea]], [[paroxysmal nocturnal dyspnea]], [[pitting edema]], [[pulmonary edema]]
* [[Malignancy]]  
*Low [[ejection fraction]] on [[echocardiography]]
* [[Monoclonal gammopathy]]
*Elevated [[brain natriuretic peptide]] (BNP) levels
* [[Autoimmunity|Autoimmune]] disease
*Abnormal [[heart rate]]
| -
*[[Third heart sound]]
|<nowiki>+/-</nowiki>
*[[Lung crackles]] or [[wheezing]]
| +
*[[Elevated jugular venous pressure]]
|<nowiki>+/-</nowiki>
*[[Cardiomegaly]] with cardiothoracic ratio on [[X-ray > 0.5
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
| +
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
| -
|
* Diffuse sclerosing glomerulonephritis
 
* Diffuse proliferative glomerulonephritis
 
* Membranoproliferative glomerulonephritis
 
* Mesangioproliferative/sclerosing disease
 
* Membranous glomerulonephritis
|
* Large fibrillar deposits in the mesangium randomly
* Glomerular capillary walls different from amloidosis
* No staining with Congo red or thioflavine-T or with antibodies to a specific type
|
* Positive for immunoglobulin G (IgG), C3
* [[Light chain|Kappa]] and lambda (ie, polyclonal) light chains
|-
|-
| bgcolor="#ececec"|'''[[Liver Cirrhosis]]''' ||
![[Fabry's disease|Fabry's Disease]]<ref name="pmid12068025">{{cite journal |vauthors=Alroy J, Sabnis S, Kopp JB |title=Renal pathology in Fabry disease |journal=J. Am. Soc. Nephrol. |volume=13 Suppl 2 |issue= |pages=S134–8 |date=June 2002 |pmid=12068025 |doi= |url=}}</ref><ref name="pmid9918480">{{cite journal |author=Meikle PJ, Hopwood JJ, Clague AE, Carey WF |title=Prevalence of lysosomal storage disorders |journal=[[JAMA : the Journal of the American Medical Association]] |volume=281 |issue=3 |pages=249–54 |year=1999 |month=January |pmid=9918480 |doi= |url=}}</ref><ref name="pmid11889412">{{cite journal |author=Branton MH, Schiffmann R, Sabnis SG, ''et al.'' |title=Natural history of Fabry renal disease: influence of alpha-galactosidase A activity and genetic mutations on clinical course |journal=[[Medicine]] |volume=81 |issue=2 |pages=122–38 |year=2002 |month=March |pmid=11889412 |doi= |url=}}</ref>
*[[Gynecomastia]], [[spider angiomata]], [[testicular atrophy]], [[encephalopathy]]
|
*[[Pitting edema]] and [[ascites]]
* [[Family history]] suggestive of the disorder
*Normal/elevated liver [[transaminase]]s
|
*[[Prolonged INR]]
* [[Anhidrosis]] or decreased sweating
*[[Hypoalbuminemia]]
* [[Fatigue (medical)|Fatigue]]
*Shrunken liver on imaging
* [[Angiokeratoma|Angiokeratomas]]  
* Burning pain of the extremities
* Corneal opacities.
* [[Dysphagia]]
* [[Abdominal pain]]
* [[Steatorrhea]]
* [[Delayed puberty]]
* [[Raynaud's phenomenon]]
* [[Hearing loss]]
* [[Telangiectasis]]
* [[Ataxia]]
|<nowiki>-</nowiki>
| +
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
| +
|<nowiki>-</nowiki>
| +
| -
|
* Vacuolization of visceral glomerular epithelial cells (podocytes) and distal tubular epithelial cells
* Glycolipid accumulation
|
* Myeloid or zebra bodies: Gb3 deposition within enlarged secondary lysosomes as lamellated membrane structures
* Inclusions, composed of concentric layers (onion skin appearance)
|<nowiki>-</nowiki>
|-
|-
| bgcolor="#ececec"|'''[[Protein Losing Enteropathy]]''' ||
! rowspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Basement Membrane Syndrome
*[[Watery diarrhea]]
! colspan="2" |[[Alport syndrome|Alport's Syndrome]]<ref name="pmid111374282">{{cite journal| author=McCarthy PA, Maino DM| title=Alport syndrome: a review. | journal=Clin Eye Vis Care | year= 2000 | volume= 12 | issue= 3-4 | pages= 139-150 | pmid=11137428 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11137428 }}</ref><ref name="pmid84141532">{{cite journal| author=Chugh KS, Sakhuja V, Agarwal A, Jha V, Joshi K, Datta BN et al.| title=Hereditary nephritis (Alport's syndrome)--clinical profile and inheritance in 28 kindreds. | journal=Nephrol Dial Transplant | year= 1993 | volume= 8 | issue= 8 | pages= 690-5 | pmid=8414153 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8414153 }}</ref><ref name="pmid8414153">{{cite journal| author=Chugh KS, Sakhuja V, Agarwal A, Jha V, Joshi K, Datta BN et al.| title=Hereditary nephritis (Alport's syndrome)--clinical profile and inheritance in 28 kindreds. | journal=Nephrol Dial Transplant | year= 1993 | volume= 8 | issue= 8 | pages= 690-5 | pmid=8414153 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8414153 }}</ref><ref name="pmid11137428">{{cite journal| author=McCarthy PA, Maino DM| title=Alport syndrome: a review. | journal=Clin Eye Vis Care | year= 2000 | volume= 12 | issue= 3-4 | pages= 139-150 | pmid=11137428 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11137428 }}</ref><ref name="pmid7819734">{{cite journal| author=Amari F, Segawa K, Ando F| title=Lens coloboma and Alport-like glomerulonephritis. | journal=Eur J Ophthalmol | year= 1994 | volume= 4 | issue= 3 | pages= 181-3 | pmid=7819734 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7819734 }}</ref><ref name="pmid6871140">{{cite journal| author=Govan JA| title=Ocular manifestations of Alport's syndrome: a hereditary disorder of basement membranes? | journal=Br J Ophthalmol | year= 1983 | volume= 67 | issue= 8 | pages= 493-503 | pmid=6871140 | doi= | pmc=PMC1040106 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6871140 }}</ref>
*[[Abdominal pain]]
|
*[[Weight loss]]
* Positive family history
*[[Hypoalbuminemia]] and hypoglobulinemia
|'''Auditary:'''
*Stool alpha-1-antitrypsin
*Early [[Tinnitus]]
*Imaging and endoscopy findings depend on etiology of [[enteropathy]]
*[[Vertigo]]
*High-frequency progressive bilateral [[Hearing impairment|hearing loss]]
 
'''Occular problems:'''
* Refractory Error
 
*Arcus
*[[Glaucoma]]
*Band Keratopathy
*[[Lenticonus]]
*[[Weill-Marchesani syndrome|Spherophakia]]
*[[Cataract|Cataracts]]  
|<nowiki>-</nowiki>
| +
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
| +
|<nowiki>-</nowiki>
| +
| -
|
* Early stage: unremarkable
 
* Late stages: [[glomerulosclerosis]], [[interstitial fibrosis]], and interstitial [[foam cells]] (due to prolonged [[proteinuria]]).
|
* Within [[glomerular basement membrane]] ([[GBM]]): longitudinal splitting of the lamina densa
|
* Absence of staining of the alpha-3, alpha-4, and alpha-5 (IV) chains in the [[glomerular basement membrane]]
* Minimal binding to a glomerulus in indirect [[immunofluorescence]] microscopy with [[Anti-glomerular basement membrane antibody|anti-glomerular basement membrane]] antibodies
|-
|-
|bgcolor="#ececec"|'''[[Malignancy]]''' ||
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
*[[Weight loss]], [[fever]], [[fatigue]]
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History
*Pitting or non-pitting edema with serous exudation, [[ascites]], [[pleural effusion]], [[pulmonary edema]]
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Systemic symptoms
*Lymphatic obstruction
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemeturia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Proteinuria
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hypertension
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Pitting edema
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Oliguria
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Nephrotic features
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Nephritic features
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hyperlipidemia and hypercholesterolemia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Auto-antibodies,
Complements
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Light microscope
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Electron microscope
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Immunoflourescence pattern
|-
|-
|bgcolor="#ececec"|'''[[Lymphedema]]''' ||
! colspan="2" |[[Thin basement membrane disease|Thin Basement Membrane Disease]]<ref name="pmid12969134">{{cite journal |author=Savige J, Rana K, Tonna S, Buzza M, Dagher H, Wang YY |title=Thin basement membrane nephropathy |journal=Kidney Int. |volume=64 |issue=4 |pages=1169–78 |year=2003 |month=October |pmid=12969134 |doi=10.1046/j.1523-1755.2003.00234.x}}</ref><ref name="pmid17726307">{{cite journal |author=Hou P, Chen Y, Ding J, Li G, Zhang H |title=A novel mutation of COL4A3 presents a different contribution to Alport syndrome and thin basement membrane nephropathy |journal=Am. J. Nephrol. |volume=27 |issue=5 |pages=538–44 |year=2007 |pmid=17726307 |doi=10.1159/000107666 |url=http://content.karger.com/produktedb/produkte.asp?typ=fulltext&file=000107666}}</ref>
*History of lymph node resection and/or [[radiation therapy]], especially in [[breast cancer]]
|
*[[Fatigue]]
* Positive family history
*Swollen limb with localized fluid accumulation
 
*[[Skin discoloration]]
* Gross [[hematuria]] following [[upper respiratory tract infection]]
|<nowiki>-</nowiki>
| -
| +
| -/+
| -
|<nowiki>-/+</nowiki>
| -
| -/+
| -
| -
| -
|Diffuse thinning of the [[Glomerular basement membrane|glomerular basement membranes]] (GBM)
|<nowiki>-</nowiki>
|-
|-
|bgcolor="#ececec"|'''[[Venous Insufficiency]]''' ||
! colspan="2" |[[Nail-patella syndrome|Nail-Patella Syndrome]]<ref name="pmid28941488">{{cite journal |vauthors=Najafian B, Smith K, Lusco MA, Alpers CE, Fogo AB |title=AJKD Atlas of Renal Pathology: Nail-Patella Syndrome-Associated Nephropathy |journal=Am. J. Kidney Dis. |volume=70 |issue=4 |pages=e19–e20 |date=October 2017 |pmid=28941488 |doi=10.1053/j.ajkd.2017.08.001 |url=}}</ref><ref name="pmid1960197">{{cite journal |vauthors=Guidera KJ, Satterwhite Y, Ogden JA, Pugh L, Ganey T |title=Nail patella syndrome: a review of 44 orthopaedic patients |journal=J Pediatr Orthop |volume=11 |issue=6 |pages=737–42 |date=1991 |pmid=1960197 |doi= |url=}}</ref>
*Painful extremities
|
*Bluish skin discoloration
* Positive family history
*[[Edema]] mostly after prolonged standing
|
*[[Stasis dermatitis]]
* Poorly developed fingernails, toe nails, and patellae (kneecaps).
 
* [[Elbow]] deformities
* Abnormally shaped [[pelvis]] bone ([[hip]] bone)
* [[Knee]] may be small, deformed or absent
|<nowiki>+</nowiki>
| +
| -
| -
| -
| -
| -
| -
| -
|
* Mostly unremarkable changes
* Secondary FSGS
* Late stages:
 
** Global glomerulosclerosis,
** Tubulointerstitial fibrosis
|
* [[Glomerular basement membrane|Glomerular basement membranes (GBMs)]]: Focal or diffuse irregular thickening with electron-lucent areas (moth-eaten appearance) containing type III collagen bundles.
* Similar collagen fibrils can be seen in mesangial matrix.
* Podocytes: Segmental effacement of foot processes.
|
* [[Immunofluorescence]] studies are typically negative.
 
* Nonspecific [[IgM]] and C3 deposition may be seen in sclerotic glomeruli.
|-
|-
| bgcolor="#ececec"|'''[[Malnutrition]]''' ||
! rowspan="6" align="center" style="background:#4479BA; color: #FFFFFF;" + | Glomerular-Vascular Syndromes 
*[[Weight loss]], [[fatigue]], [[irritability]], [[dyspnea]], and [[depression]]
! colspan="2" |[[Hypertensive nephropathy|Hypertensive Nephrosclerosis]]<ref name="pmid24327566">{{cite journal |vauthors=Hughson MD, Puelles VG, Hoy WE, Douglas-Denton RN, Mott SA, Bertram JF |title=Hypertension, glomerular hypertrophy and nephrosclerosis: the effect of race |journal=Nephrol. Dial. Transplant. |volume=29 |issue=7 |pages=1399–409 |date=July 2014 |pmid=24327566 |pmc=4071048 |doi=10.1093/ndt/gft480 |url=}}</ref>
*Menstrual irregularities
|Chronic [[hypertension]]
*Poor wound healing and easy bruisibility
|
*[[Muscle atrophy]] and fat wasting
* [[Hypertensive retinopathy|Hypertensive retinal]] changes.
*[[Dry skin]] and [[hair loss]]
 
*[[Anemia]]
* High [[jugular venous pressure]]
*[[Hypoalbuminemia]]
 
* [[Rales]] from [[pulmonary edema]] on auscultation
* .Signs of [[left ventricular hypertrophy]]
 
* [[Left ventricle|Left ventricular]] heave
* Shifting of apex towards the left
 
* [[S3]] and [[gallop rhythm]]
* Loud S2
 
* [[Ascites]]
 
* [[Paralysis]] from [[stroke]] secondary to [[hypertension]]
 
* Inferior limb [[edema]]
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
| +
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
| -
| +/-
| -
| colspan="3" |
* Interstitial [[fibrosis]] and atrophy
* Medial thickening and intimal fibrosis of medium-sized and larger vessels
* Arteriolar thickening, and hyalinosis
* Chronic stages:
** Global sclerosis
** Focal segmental [[sclerosis]]
|-
! colspan="2" |[[Cholesterol emboli syndrome|Cholesterol Emboli]]<ref name="pmid27012950">{{cite journal |vauthors=Lusco MA, Najafian B, Alpers CE, Fogo AB |title=AJKD Atlas of Renal Pathology: Cholesterol Emboli |journal=Am. J. Kidney Dis. |volume=67 |issue=4 |pages=e23–4 |date=April 2016 |pmid=27012950 |doi=10.1053/j.ajkd.2016.02.034 |url=}}</ref>
|
* Atherosclerotic cardiovascular disease
* Anticoagulation therapy
* Cardiopulmonary resuscitation
* [[Hypertension]]
* [[Aortic aneurysm]]
* [[Hypercholesterolemia]]
* Smoking history
* Male sex
* Age over 55 years
* [[Vascular]] procedures
* Invasive [[angiography]]
* [[Aortic aneurysm]] rupture or surgery
* [[Vascular]] surgery
|
* Depends on the organ involved
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
| +
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
| -
| +/-
| -
|
* Atheroemboli are seen in interlobular and arcuate arteries, as lance-shaped clefts, due to dissolution of [[cholesterol]] crystals
* Acute lesions:
** Atheroemboli are surrounded by red blood cells, fibrin, and leukocytes, with multinucleated giant cell reactions 
* Chronic lesions:
** Cholesterol clefts are surrounded by intimal fibrosis
** Vessel recanalization of chronic lesions can occur.
* Global and segmental sclerosis of glomeruli may be present.
|
* Extensive foot process effacement can be seen
|
* Not specific changes
|-
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Systemic symptoms
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemeturia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Proteinuria
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hypertension
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Pitting edema
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Oliguria
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Nephrotic features
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Nephritic features
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hyperlipidemia and hypercholesterolemia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Auto-antibodies,
Complements
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Light microscope
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Electron microscope
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Immunoflourescence pattern
|-
! colspan="2" |[[Sickle-cell disease|Sickle Cell Disease]]<ref name="pmid12028473">{{cite journal |vauthors=Wesson DE |title=The initiation and progression of sickle cell nephropathy |journal=Kidney Int. |volume=61 |issue=6 |pages=2277–86 |date=June 2002 |pmid=12028473 |doi=10.1046/j.1523-1755.2002.00363.x |url=}}</ref>
|
* Positive family history
|
* [[Pain and nociception|Pain]] and/or [[bone pain]]
* [[Dactylitis]]
* [[Blurry vision]]
* [[Priapism|Persistent and painful erection]]
* [[Numbness]]
* [[Tingling]]
* Motor skill loss
* [[Aphasia|Speech deficits]]
* [[Gait disturbance]]
* Leg ulceration
* [[Jaundice]]
|<nowiki>+/-</nowiki>
|<nowiki>+/-</nowiki>
| +/-
| -
| -
| -
| -
| -
| -
| colspan="3" |
* [[Glomerulus|Glomerular]] hypertrophy
* [[Hemosiderin]] deposits
* Focal areas of hemorrhage or necrosis
* Chronic stage: interstitial inflammation, edema, fibrosis, tubular atrophy, and [[papillary]] infarcts
 
* Glomerular enlargement and focal segmental glomerulosclerosis ([[Focal segmental glomerulosclerosis|FSGS]])
|-
! colspan="2" |[[Thrombotic microangiopathies|Thrombotic Microangiopathies]]<ref name="pmid27884283">{{cite journal |vauthors=Lusco MA, Fogo AB, Najafian B, Alpers CE |title=AJKD Atlas of Renal Pathology: Thrombotic Microangiopathy |journal=Am. J. Kidney Dis. |volume=68 |issue=6 |pages=e33–e34 |date=December 2016 |pmid=27884283 |doi=10.1053/j.ajkd.2016.10.006 |url=}}</ref>
| colspan="2" |'''''Click for more information on [[Thrombotic microangiopathies|Thrombotic Microangiopathies]].'''''
| +
|<nowiki>+/-</nowiki>
| +
| +/-
| +/-
| +/-
| -
| -
| -
|
* Acute stage:
** Inravasculr fibrin thrombi
* Chronic stage:
** Endocapillary hypercellularity.
** Intimal proliferation of [[Arteriole|arterioles]]
|
* Swollen glomerular endothelial cells with loss of [[Fenestration|fenestrations]]
* Chronic stage: interposed cells with new [[GBM]] matrix material deposition.
|
* [[Thrombus|Thrombi]] stain positive for [[fibrinogen]]
|-
! colspan="2" |[[Antiphospholipid syndrome|Antiphospholipid Antibody Syndrome]] <ref name="pmid24684307">{{cite journal| author=Jayakody Arachchillage D, Greaves M| title=The chequered history of the antiphospholipid syndrome. | journal=Br J Haematol | year= 2014 | volume= 165 | issue= 5 | pages= 609-17 | pmid=24684307 | doi=10.1111/bjh.12848 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24684307  }}</ref><ref name="pmid246843072">{{cite journal| author=Jayakody Arachchillage D, Greaves M| title=The chequered history of the antiphospholipid syndrome. | journal=Br J Haematol | year= 2014 | volume= 165 | issue= 5 | pages= 609-17 | pmid=24684307 | doi=10.1111/bjh.12848 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24684307  }}</ref><ref name="pmid18714484">{{cite journal| author=Popa A, Voinea L, Pop M, Stana D, Dascalu AM, Alexandrescu C et al.| title=[Primary antiphospholipid syndrome]. | journal=Oftalmologia | year= 2008 | volume= 52 | issue= 1 | pages= 13-7 | pmid=18714484 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18714484  }}</ref>
|
* [[Thrombosis]]
* [[Miscarriage]] 
* History of [[nephropathy]]
* History of [[Hematology|hematologic]] abnormalities
* Nonthrombotic neurologic symptoms, such as [[migraine]] headaches
* [[Pulmonary hypertension]]
|
*Fatigue
*Fever
*Weight loss
 
*[[Venous thrombosis]]
*[[Arterial thrombosis]]
*[[Thrombocytopenia]]
*Recurrent fetal loss     
| +
|<nowiki>+/-</nowiki>
| +
| +/-
| +/-
| +/-
| -
| -
| -
|
* Acute stage:
** Inravasculr fibrin [[Thrombus|thrombi]]
* Chronic stage:
** [[Endocapillary proliferative glomerulonephritis|Endocapillary]] hypercellularity.
** Intimal proliferation of [[Arteriole|arterioles]]
|
* Swollen glomerular endothelial cells with loss of fenestrations
* Chronic stage: interposed cells with new GBM matrix material deposition.
|
* [[Thrombus|Thrombi]] stain positive for [[fibrinogen]]
|}
|}
<small>


'''Some infectious diseases such as [[HIV]], [[Hepatitis B virus|HBV]], [[Hepatitis C|HCV]], [[syphilis]], [[leprosy]], [[malaria]], and [[schistosomiasis]] may cause glomerular diseases.'''
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
[[Category:Kidney diseases]]
 
[[Category:Medicine]]
[[Category:Nephrology]]
[[Category:Nephrology]]
[[Category:Pediatrics]]
[[Category:Urology]]
[[Category:Disease]]
[[Category:Up-To-Date]]
[[Category:Syndromes]]
[[Category:Emergency medicine]]
[[Category:Needs overview]]
[[Category:Primary care]]
{{WH}}
{{WS}}

Latest revision as of 22:57, 29 July 2020

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2]

Overview

Nephrotic syndrome should be differentiate from other causes of glomerular disease such as Fabry's disease, post-streptococcal glomerulonephritis, lupus nephritis, antiglomerular basement membrane disease (goodpasture's syndrome), Cryoglobulinemia, Henoch-Schönlein purpura, amyloidosis, pulmonary-renal syndromes (vasculitis), thin basement membrane disease, Alport's Syndrome, anti-GBM Disease, hypertensive nephrosclerosis, and subacute bacterial endocarditis. The various types of glomerular diseases may be differentiated from each other based on associations, presence of pitting edema, hematuria, hypertension, hemoptysis, oliguria, peri-orbital edema, hyperlipidemia, type of antibodies, light, and electron microscopic features.

Differential Diagnosis

Nephrotic syndrome should be differentiate from other causes of glomerular disease. The various types of glomerular diseases may be differentiated from each other based on associations, presence of pitting edema, hemeturia, hypertension, hemoptysis, oliguria, peri-orbital edema, hyperlipidemia, type of antibodies, light and electron microscopic features. The following table differentiates between various types of glumerular diseases:

Glomerular diseases Disease History and Symtoms Laboratory Findings Pathology
History Systemic symptoms Hemeturia Proteinuria Hypertension Pitting edema Oliguria Nephrotic features Nephritic features Hyperlipidemia and hypercholesterolemia Auto-antibodies,

Complements

Light microscope Electron microscope Immunoflourescence pattern
Acute Nephritic Syndromes Poststreptococcal Glomerulonephritis[1][2][3] +/- + +/- +/- +/- +/- +/- +/-
  • Immune complex GN
  • Granular deposit
Renal disease due to Subacute Bacterial Endocarditis, or cardiac shunt (Atrioventricular)[4][5] +/- + +/- +/- +/- +/- +/- +/-
  • Crescentic GN is the most common pathological features
  • Mesangial deposits,
  • Subendothelial deposits
  • Subepithelial "humps," in minority of cases
  • Pauci-immune GN
Lupus Nephritis[6]
  • History of SLE features
 +/- + +/- +/- +/- +/- +/- +/-
  • Differs based on the disease classification
  • Differs based on the disease classification
  • Differs based on the disease classification, mostly immune complex GN
  • Granular deposit
Antiglomerular Basement Membrane Disease (Goodpasture's syndrome)[7][8]
  • Young adults
 + + + + + + - - Diffuse thickening of the glomerular basement membrane with absence of sub-epithelial and sub-endothelial deposits 
  • Immune complex GN
  • Linear deposit
IgA Nephropathy[9][10] + +/- + +/- + - + -
  • Immune complex deposition
  • Crescent formation
  • Immune complex GN, granular deposite
Disease History Systemic symptoms Hemeturia Proteinuria Hypertension Pitting edema Oliguria Nephrotic features Nephritic features Hyperlipidemia and hypercholesterolemia Auto-antibodies,

Complements

Light microscope Electron microscope Immunoflourescence pattern
ANCA Small-Vessel Vasculitis[11][12] Granulomatosis with Polyangiitis (Wegener's)[13][14][15]
  • Middle age male
 + + + +/- + - + -
  •  Pauci-immune GN
Microscopic Polyangiitis[16] +/- + + + + + + -
  •  Pauci-immune GN
Churg-Strauss Syndrome[17] +/- + + + + + + -
  •  Pauci-immune GN
Membranoproliferative Glomerulonephritis[18][19] + + + +/- + + - - -
  • Immune complex GN
  • Granular deposite
Henoch-Schönlein purpura [20] + + + +/- + + - - -
  • Diffuse mesangial IgA deposits often associated with mesangial hypercellularity
  • Diffuse mesangial IgA deposits often associated with mesangial hypercellularity
  • Immune complex GN, granular deposite
Disease History Systemic symptoms Hemeturia Proteinuria Hypertension Pitting edema Oliguria Nephrotic features Nephritic features Hyperlipidemia and hypercholesterolemia Auto-antibodies,

Complements

Light microscope Electron microscope Immunoflourescence pattern
Cryoglobulinemia[21] Patients having cryoglobulinemia may have positive history of: Pulmonary symptoms:
  • Cough

Cutaneous symptoms:

Gastrointestinal symptoms:

  • Abdominal pain

General symptoms:

+/- + +/- + +/- +/- +/- +/- +/-
  • Prominent IgM and C3
Nephrotic Syndrome Minimal Change Disease[22][23] - + - + +/- + - +
  • Normal
 -
Focal Segmental Glomerulosclerosis[24][25][26] - + - + +/- + - + -
Membranous Glomerulonephritis[27][28] - + - + +/- + - + Immune complex deposition Immune complex GN, granular deposite
Diabetic Nephropathy[29][30][31][32][33][34][35][36][37][38] For more information on diabetes click here. - + - + +/- + - +
  • Diffuse mesangial matrix expansion (nodular glomerulosclerosis)
  • Increased mesangial hypercellularity
  • Prominent glomerular basement membranes
  • Thick basement membrane without any deposit
  • Nodular glomerulosclerosis
 -
Disease History Systemic symptoms Hemeturia Proteinuria Hypertension Pitting edema Oliguria Nephrotic features Nephritic features Hyperlipidemia and hypercholesterolemia Auto-antibodies,

Complements

Light microscope Electron microscope Immunoflourescence pattern
 Glomerular Deposition Diseases  Light Chain Deposition Disease[39]
  • Occurs in the setting of high tumor burden
 - - + - + +/- + - + -
  • Light-chain deposits
  • Granular deposits on electron microscopy
  • Detection of light chain deposits using anti–light chain antibody
Renal Amyloidosis[40][41][42][43] - + - + +/- + - + -
  • Diffuse glomerular deposition of amorphous hyaline material (nodular pattern), in mesangium (weakly staining with periodic acid-Schiff (PAS)
  • Nodular deposit
  • AA amyloidosis type: negative for immunoglobulins and complement
  • AL amyloidosis type: Positive for lambda or kappa light chains
Fibrillary-Immunotactoid Glomerulopathy[44] - +/- + +/- +/- +/- + +/- +/- -
  • Diffuse sclerosing glomerulonephritis
  • Diffuse proliferative glomerulonephritis
  • Membranoproliferative glomerulonephritis
  • Mesangioproliferative/sclerosing disease
  • Membranous glomerulonephritis
  • Large fibrillar deposits in the mesangium randomly
  • Glomerular capillary walls different from amloidosis
  • No staining with Congo red or thioflavine-T or with antibodies to a specific type
  • Positive for immunoglobulin G (IgG), C3
  • Kappa and lambda (ie, polyclonal) light chains
Fabry's Disease[45][46][47] - + - + +/- + - + -
  • Vacuolization of visceral glomerular epithelial cells (podocytes) and distal tubular epithelial cells
  • Glycolipid accumulation
  • Myeloid or zebra bodies: Gb3 deposition within enlarged secondary lysosomes as lamellated membrane structures
  • Inclusions, composed of concentric layers (onion skin appearance)
 -
Basement Membrane Syndrome Alport's Syndrome[48][49][50][51][52][53]
  • Positive family history
 Auditary:

Occular problems:

  • Refractory Error
 - + - + +/- + - + -
  • Early stage: unremarkable
Disease History Systemic symptoms Hemeturia Proteinuria Hypertension Pitting edema Oliguria Nephrotic features Nephritic features Hyperlipidemia and hypercholesterolemia Auto-antibodies,

Complements

Light microscope Electron microscope Immunoflourescence pattern
Thin Basement Membrane Disease[54][55]
  • Positive family history
 - - + -/+ - -/+ - -/+ - - - Diffuse thinning of the glomerular basement membranes (GBM) -
Nail-Patella Syndrome[56][57]
  • Positive family history
  • Poorly developed fingernails, toe nails, and patellae (kneecaps).
  • Elbow deformities
  • Abnormally shaped pelvis bone (hip bone)
  • Knee may be small, deformed or absent
 + + - - - - - - -
  • Mostly unremarkable changes
  • Secondary FSGS
  • Late stages:
    • Global glomerulosclerosis,
    • Tubulointerstitial fibrosis
  • Glomerular basement membranes (GBMs): Focal or diffuse irregular thickening with electron-lucent areas (moth-eaten appearance) containing type III collagen bundles.
  • Similar collagen fibrils can be seen in mesangial matrix.
  • Podocytes: Segmental effacement of foot processes.
  • Nonspecific IgM and C3 deposition may be seen in sclerotic glomeruli.
 Glomerular-Vascular Syndromes  Hypertensive Nephrosclerosis[58] Chronic hypertension +/- +/- + +/- +/- +/- - +/- -
  • Interstitial fibrosis and atrophy
  • Medial thickening and intimal fibrosis of medium-sized and larger vessels
  • Arteriolar thickening, and hyalinosis
  • Chronic stages:
Cholesterol Emboli[59]
  • Depends on the organ involved
 +/- +/- + +/- +/- +/- - +/- -
  • Atheroemboli are seen in interlobular and arcuate arteries, as lance-shaped clefts, due to dissolution of cholesterol crystals
  • Acute lesions:
    • Atheroemboli are surrounded by red blood cells, fibrin, and leukocytes, with multinucleated giant cell reactions
  • Chronic lesions:
    • Cholesterol clefts are surrounded by intimal fibrosis
    • Vessel recanalization of chronic lesions can occur.
  • Global and segmental sclerosis of glomeruli may be present.
  • Extensive foot process effacement can be seen
  • Not specific changes
Disease History Systemic symptoms Hemeturia Proteinuria Hypertension Pitting edema Oliguria Nephrotic features Nephritic features Hyperlipidemia and hypercholesterolemia Auto-antibodies,

Complements

Light microscope Electron microscope Immunoflourescence pattern
Sickle Cell Disease[60]
  • Positive family history
 +/- +/- +/- - - - - - -
  • Glomerular hypertrophy
  • Hemosiderin deposits
  • Focal areas of hemorrhage or necrosis
  • Chronic stage: interstitial inflammation, edema, fibrosis, tubular atrophy, and papillary infarcts
  • Glomerular enlargement and focal segmental glomerulosclerosis (FSGS)
Thrombotic Microangiopathies[61] Click for more information on Thrombotic Microangiopathies. + +/- + +/- +/- +/- - - -
  • Acute stage:
    • Inravasculr fibrin thrombi
  • Chronic stage:
    • Endocapillary hypercellularity.
    • Intimal proliferation of arterioles
  • Swollen glomerular endothelial cells with loss of fenestrations
  • Chronic stage: interposed cells with new GBM matrix material deposition.
Antiphospholipid Antibody Syndrome [62][63][64]
  • Fatigue
  • Fever
  • Weight loss
 + +/- + +/- +/- +/- - - -
  • Swollen glomerular endothelial cells with loss of fenestrations
  • Chronic stage: interposed cells with new GBM matrix material deposition.


Some infectious diseases such as HIV, HBV, HCV, syphilis, leprosy, malaria, and schistosomiasis may cause glomerular diseases.

References

  1. ↑ GERMUTH FG (1953). "A comparative histologic and immunologic study in rabbits of induced hypersensitivity of the serum sickness type". J Exp Med. 97 (2): 257–82. PMC 2136196. PMID 13022878.
  2. ↑ Germuth FG, Senterfit LB, Dreesman GR (1972). "Immune complex disease. V. The nature of the circulating complexes associated with glomerular alterations in the chronic BSA-rabbit system". Johns Hopkins Med J. 130 (6): 344–57. PMID 5031005.
  3. ↑ Radhakrishnan J, Cattran DC (2012). "The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient". Kidney Int. 82 (8): 840–56. doi:10.1038/ki.2012.280. PMID 22895519.
  4. ↑ Neugarten J, Baldwin DS (August 1984). "Glomerulonephritis in bacterial endocarditis". Am. J. Med. 77 (2): 297–304. PMID 6380288.
  5. ↑ Arze RS, Rashid H, Morley R, Ward MK, Kerr DN (January 1983). "Shunt nephritis: report of two cases and review of the literature". Clin. Nephrol. 19 (1): 48–53. PMID 6831779.
  6. ↑ Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, Balow JE, Bruijn JA, Cook T, Ferrario F, Fogo AB, Ginzler EM, Hebert L, Hill G, Hill P, Jennette JC, Kong NC, Lesavre P, Lockshin M, Looi LM, Makino H, Moura LA, Nagata M (February 2004). "The classification of glomerulonephritis in systemic lupus erythematosus revisited". Kidney Int. 65 (2): 521–30. doi:10.1111/j.1523-1755.2004.00443.x. PMID 14717922.
  7. ↑ Bolton WK (November 1996). "Goodpasture's syndrome". Kidney Int. 50 (5): 1753–66. PMID 8914046.
  8. ↑ Mathew TH, Hobbs JB, Kalowski S, Sutherland PW, Kincaid-Smith P (February 1975). "Goodpasture's syndrome: normal renal diagnostic findings". Ann. Intern. Med. 82 (2): 215–8. PMID 1090223.
  9. ↑ Suzuki H, Kiryluk K, Novak J, Moldoveanu Z, Herr AB, Renfrow MB, Wyatt RJ, Scolari F, Mestecky J, Gharavi AG, Julian BA (October 2011). "The pathophysiology of IgA nephropathy". J. Am. Soc. Nephrol. 22 (10): 1795–803. doi:10.1681/ASN.2011050464. PMC 3892742. PMID 21949093.
  10. ↑ Wyatt RJ, Julian BA (June 2013). "IgA nephropathy". N. Engl. J. Med. 368 (25): 2402–14. doi:10.1056/NEJMra1206793. PMID 23782179.
  11. ↑ Higgins RM, Goldsmith DJ, Connolly J, Scoble JE, Hendry BM, Ackrill P, Venning MC (January 1996). "Vasculitis and rapidly progressive glomerulonephritis in the elderly". Postgrad Med J. 72 (843): 41–4. PMC 2398323. PMID 8746284.
  12. ↑ Jennette JC (March 2003). "Rapidly progressive crescentic glomerulonephritis". Kidney Int. 63 (3): 1164–77. doi:10.1046/j.1523-1755.2003.00843.x. PMID 12631105.
  13. ↑ Renaudineau Y, Le Meur Y (October 2008). "Renal involvement in Wegener's granulomatosis". Clin Rev Allergy Immunol. 35 (1–2): 22–9. doi:10.1007/s12016-007-8066-6. PMID 18172777.
  14. ↑ Weiss MA, Crissman JD (October 1984). "Renal biopsy findings in Wegener's granulomatosis: segmental necrotizing glomerulonephritis with glomerular thrombosis". Hum. Pathol. 15 (10): 943–56. PMID 6384024.
  15. ↑ Pagnoux C (March 2008). "[Wegener's granulomatosis and microscopic polyangiitis]". Rev Prat (in French). 58 (5): 522–32. PMID 18524109.
  16. ↑ Chung SA, Seo P (August 2010). "Microscopic polyangiitis". Rheum. Dis. Clin. North Am. 36 (3): 545–58. doi:10.1016/j.rdc.2010.04.003. PMC 2917831. PMID 20688249.
  17. ↑ Sinico RA, Di Toma L, Maggiore U, Tosoni C, Bottero P, Sabadini E, Giammarresi G, Tumiati B, Gregorini G, Pesci A, Monti S, Balestrieri G, Garini G, Vecchio F, Buzio C (May 2006). "Renal involvement in Churg-Strauss syndrome". Am. J. Kidney Dis. 47 (5): 770–9. doi:10.1053/j.ajkd.2006.01.026. PMID 16632015.
  18. ↑ Alchi B, Jayne D (August 2010). "Membranoproliferative glomerulonephritis". Pediatr. Nephrol. 25 (8): 1409–18. doi:10.1007/s00467-009-1322-7. PMC 2887509. PMID 19908070.
  19. ↑ Davis AE, Schneeberger EE, Grupe WE, McCluskey RT (May 1978). "Membranoproliferative glomerulonephritis (MPGN type I) and dense deposit disease (DDD) in children". Clin. Nephrol. 9 (5): 184–93. PMID 657595.
  20. ↑ Jennette JC, Falk RJ (July 1994). "The pathology of vasculitis involving the kidney". Am. J. Kidney Dis. 24 (1): 130–41. PMID 8023818.
  21. ↑ Fogo AB, Lusco MA, Najafian B, Alpers CE (February 2016). "AJKD Atlas of Renal Pathology: Cryoglobulinemic Glomerulonephritis". Am. J. Kidney Dis. 67 (2): e5–7. doi:10.1053/j.ajkd.2015.12.007. PMID 26802335.
  22. ↑ Saha TC, Singh H (November 2006). "Minimal change disease: a review". South. Med. J. 99 (11): 1264–70. doi:10.1097/01.smj.0000243183.87381.c2. PMID 17195422.
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  34. ↑ Toyoda M, Najafian B, Kim Y, Caramori ML, Mauer M (2007). "Podocyte detachment and reduced glomerular capillary endothelial fenestration in human type 1 diabetic nephropathy". Diabetes. 56 (8): 2155–60. doi:10.2337/db07-0019. PMID 17536064.
  35. ↑ Najafian B, Crosson JT, Kim Y, Mauer M (2006). "Glomerulotubular junction abnormalities are associated with proteinuria in type 1 diabetes". J Am Soc Nephrol. 17 (4 Suppl 2): S53–60. doi:10.1681/ASN.2005121342. PMID 16565248.
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  44. ↑ Korbet SM, Schwartz MM, Lewis EJ (March 1991). "Immunotactoid glomerulopathy". Am. J. Kidney Dis. 17 (3): 247–57. PMID 1996564.
  45. ↑ Alroy J, Sabnis S, Kopp JB (June 2002). "Renal pathology in Fabry disease". J. Am. Soc. Nephrol. 13 Suppl 2: S134–8. PMID 12068025.
  46. ↑ Meikle PJ, Hopwood JJ, Clague AE, Carey WF (1999). "Prevalence of lysosomal storage disorders". JAMA : the Journal of the American Medical Association. 281 (3): 249–54. PMID 9918480. Unknown parameter |month= ignored (help)
  47. ↑ Branton MH, Schiffmann R, Sabnis SG; et al. (2002). "Natural history of Fabry renal disease: influence of alpha-galactosidase A activity and genetic mutations on clinical course". Medicine. 81 (2): 122–38. PMID 11889412. Unknown parameter |month= ignored (help)
  48. ↑ McCarthy PA, Maino DM (2000). "Alport syndrome: a review". Clin Eye Vis Care. 12 (3–4): 139–150. PMID 11137428.
  49. ↑ Chugh KS, Sakhuja V, Agarwal A, Jha V, Joshi K, Datta BN; et al. (1993). "Hereditary nephritis (Alport's syndrome)--clinical profile and inheritance in 28 kindreds". Nephrol Dial Transplant. 8 (8): 690–5. PMID 8414153.
  50. ↑ Chugh KS, Sakhuja V, Agarwal A, Jha V, Joshi K, Datta BN; et al. (1993). "Hereditary nephritis (Alport's syndrome)--clinical profile and inheritance in 28 kindreds". Nephrol Dial Transplant. 8 (8): 690–5. PMID 8414153.
  51. ↑ McCarthy PA, Maino DM (2000). "Alport syndrome: a review". Clin Eye Vis Care. 12 (3–4): 139–150. PMID 11137428.
  52. ↑ Amari F, Segawa K, Ando F (1994). "Lens coloboma and Alport-like glomerulonephritis". Eur J Ophthalmol. 4 (3): 181–3. PMID 7819734.
  53. ↑ Govan JA (1983). "Ocular manifestations of Alport's syndrome: a hereditary disorder of basement membranes?". Br J Ophthalmol. 67 (8): 493–503. PMC 1040106. PMID 6871140.
  54. ↑ Savige J, Rana K, Tonna S, Buzza M, Dagher H, Wang YY (2003). "Thin basement membrane nephropathy". Kidney Int. 64 (4): 1169–78. doi:10.1046/j.1523-1755.2003.00234.x. PMID 12969134. Unknown parameter |month= ignored (help)
  55. ↑ Hou P, Chen Y, Ding J, Li G, Zhang H (2007). "A novel mutation of COL4A3 presents a different contribution to Alport syndrome and thin basement membrane nephropathy". Am. J. Nephrol. 27 (5): 538–44. doi:10.1159/000107666. PMID 17726307.
  56. ↑ Najafian B, Smith K, Lusco MA, Alpers CE, Fogo AB (October 2017). "AJKD Atlas of Renal Pathology: Nail-Patella Syndrome-Associated Nephropathy". Am. J. Kidney Dis. 70 (4): e19–e20. doi:10.1053/j.ajkd.2017.08.001. PMID 28941488.
  57. ↑ Guidera KJ, Satterwhite Y, Ogden JA, Pugh L, Ganey T (1991). "Nail patella syndrome: a review of 44 orthopaedic patients". J Pediatr Orthop. 11 (6): 737–42. PMID 1960197.
  58. ↑ Hughson MD, Puelles VG, Hoy WE, Douglas-Denton RN, Mott SA, Bertram JF (July 2014). "Hypertension, glomerular hypertrophy and nephrosclerosis: the effect of race". Nephrol. Dial. Transplant. 29 (7): 1399–409. doi:10.1093/ndt/gft480. PMC 4071048. PMID 24327566.
  59. ↑ Lusco MA, Najafian B, Alpers CE, Fogo AB (April 2016). "AJKD Atlas of Renal Pathology: Cholesterol Emboli". Am. J. Kidney Dis. 67 (4): e23–4. doi:10.1053/j.ajkd.2016.02.034. PMID 27012950.
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  62. ↑ Jayakody Arachchillage D, Greaves M (2014). "The chequered history of the antiphospholipid syndrome". Br J Haematol. 165 (5): 609–17. doi:10.1111/bjh.12848. PMID 24684307.
  63. ↑ Jayakody Arachchillage D, Greaves M (2014). "The chequered history of the antiphospholipid syndrome". Br J Haematol. 165 (5): 609–17. doi:10.1111/bjh.12848. PMID 24684307.
  64. ↑ Popa A, Voinea L, Pop M, Stana D, Dascalu AM, Alexandrescu C; et al. (2008). "[Primary antiphospholipid syndrome]". Oftalmologia. 52 (1): 13–7. PMID 18714484.
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