Nephrotic syndrome classification: Difference between revisions

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__NOTOC__
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{{Nephrotic syndrome}}
{{Nephrotic syndrome}}
{{CMG}} {{APM}}; {{AE}} {{OO}} [[User:YazanDaaboul|Yazan Daaboul]], [[User:Sergekorjian|Serge Korjian]]
{{CMG}}, {{APM}}; {{AE}} {{OO}}, [[User:YazanDaaboul|Yazan Daaboul]], [[User:Sergekorjian|Serge Korjian]]


==Overview==
==Overview==
Nephrotic syndrome can be classified into primary or secondary depending on the underlying etiology.  Primary (idiopathic) nephrotic syndrome is defined as nephrotic syndrome due to a primary [[glomerular]] disease. Secondary nephrotic syndrome is defined as nephrotic syndrome due to a primary etiology other than glomerular disorders, such as [[infection]]s, [[malignancies]], systemic conditions, and [[medication]]s.
Nephrotic syndrome can be classified into primary or secondary depending on the underlying etiology.  Primary (idiopathic) nephrotic syndrome is defined as nephrotic syndrome due to a primary [[glomerular]] disease. The secondary nephrotic syndrome is defined as nephrotic syndrome due to a primary etiology other than glomerular disorders, such as [[infection]]s, [[malignancies]], systemic conditions, and [[medication]]s.


==Classification==
==Classification==
===Etiologic Classification===
* Nephrotic syndrome can be classified into primary or secondary depending on the underlying etiology.<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref>
Nephrotic syndrome can be classified into primary or secondary depending on the underlying etiology.
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====Primary (Idiopathic) Nephrotic Syndrome====
* Below table lists different types of nephrotic syndromes:<ref name="pmid10793022">{{cite journal |vauthors=Braden GL, Mulhern JG, O'Shea MH, Nash SV, Ucci AA, Germain MJ |title=Changing incidence of glomerular diseases in adults |journal=Am. J. Kidney Dis. |volume=35 |issue=5 |pages=878–83 |date=May 2000 |pmid=10793022 |doi= |url=}}</ref><ref name="pmid15327377">{{cite journal |vauthors=Heaf J |title=The Danish Renal Biopsy Register |journal=Kidney Int. |volume=66 |issue=3 |pages=895–7 |date=September 2004 |pmid=15327377 |doi=10.1111/j.1523-1755.2004.00832.x |url=}}</ref><ref name="pmid28392820">{{cite journal |vauthors=Ha TS |title=Genetics of hereditary nephrotic syndrome: a clinical review |journal=Korean J Pediatr |volume=60 |issue=3 |pages=55–63 |date=March 2017 |pmid=28392820 |pmc=5383633 |doi=10.3345/kjp.2017.60.3.55 |url=}}</ref>
Primary (idiopathic) nephrotic syndrome is defined as nephrotic syndrome due to a primary glomerular disease. It is a common diagnosis in children. Etiologies of idiopathic nephrotic syndrome include:<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref>
{|  
{| class="wikitable"
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Different types of nephrotic syndromes
! colspan="2" |Different types of nephrotic syndromes
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease name
!Disease name
|-
|-
| rowspan="6" |Podocytopathies
! rowspan="6" align="center" style="background:#DCDCDC;" + |Podocytopathies
| rowspan="2" |Primary  
| rowspan="2" align="left" style="background:#F5F5F5;" + |Primary  
|Primary minimal change disease
| align="left" style="background:#F5F5F5;" + |[[Minimal change disease]]
|-
|-
|Primary FSGS
| align="left" style="background:#F5F5F5;" + |[[Focal segmental glomerulosclerosis]] ([[Focal segmental glomerulosclerosis|FSGS]])
|-
|-
| rowspan="4" |Secondary
| rowspan="4" align="left" style="background:#F5F5F5;" + |Secondary
|Infection: HIV
| align="left" style="background:#F5F5F5;" + |Infection such as:  
* [[Human Immunodeficiency Virus (HIV)|HIV]]
|-
|-
|Drugs/toxins: NSAIDs, interferon, pamironate, lithium, vaccins, envenomation
| align="left" style="background:#F5F5F5;" + |Drugs/toxins such as:
* [[Non-steroidal anti-inflammatory drug|NSAIDs]]
* Interferon
* Pamidronate
* Lithium
* Vaccins
* Envenomation
|-
|-
|Malignancies: hodgkin lymphoma, thymoma
| align="left" style="background:#F5F5F5;" + |Malignancies such as:
* [[Hodgkin's lymphoma|Hodgkin lymphoma]]
* [[Thymoma]]
|-
|-
|Genetics: nephrin mutations, podocin
| align="left" style="background:#F5F5F5;" + |Genetic disorders such as:
* [[Nephrin]] mutations
* [[Podocin]] gene mutations
|-
|-
| rowspan="2" |Membranous nephropathies
! rowspan="2" align="center" style="background:#DCDCDC;" + |[[Membranous glomerulonephritis]]
|Primary  
| align="left" style="background:#F5F5F5;" + |Primary  
|Primary membranous nephropathy
| align="left" style="background:#F5F5F5;" + |[[Membranous nephritis|Membranous nephropathy]]
|-
|-
|Secondary
| align="left" style="background:#F5F5F5;" + |Secondary
|Drugs, toxins, malignancies
| align="left" style="background:#F5F5F5;" + |
* [[Drugs]]
* [[Toxin|Toxins]]
* [[Cancer|Malignancies]]
|-
|-
|Membranoproliferative glomerulonephritis
! align="center" style="background:#DCDCDC;" + |[[Membranoproliferative glomerulonephritis]]
|Secondary
| align="left" style="background:#F5F5F5;" + |Secondary
|
| align="left" style="background:#F5F5F5;" + |
* Autoimmune disorders: SLE, aquired C3 glomerulopathy
* Autoimmune disorders such as:
* Infections: HCV, endocarditis
** [[SLE]]
* Genetics
** Aquired [[C3 glomerulopathy]]
* Malignancies  
* Infections such as:
* Thrombotic microangiopathy
** [[Hepatitis C|HCV]]
** [[Endocarditis]]
* [[Genetics]]
* [[Malignancies]]
* [[Thrombotic microangiopathy]]
|-
|-
| colspan="2" |Glomerular deposition diseases
! align="center" style="background:#DCDCDC;" + |Hereditary Nephropathy
|Diabetes mellitus
| colspan="2" align="left" style="background:#F5F5F5;" + |
Amyloidosis
* [[Alport syndrome]]
 
* [[IgA nephropathy]]
Monoclonal deposition disease
* C1q nephropathy
 
Nodular glomeruloneohritis associated with heavy smoking
|-
|-
|Other lesions
! align="center" style="background:#DCDCDC;" + |[[Glomerular deposition disease|Glomerular deposition diseases]]
|
| colspan="2" align="left" style="background:#F5F5F5;" + |
|
* [[Diabetes mellitus]]
* [[Amyloidosis]]
* [[Monoclonal antibodies|Monoclonal]] deposition disease
* Nodular glomeruloneohritis associated with heavy [[smoking]]
|-
|-
|
! align="center" style="background:#DCDCDC;" + |Others
|
| colspan="2" align="left" style="background:#F5F5F5;" + |
|
* [[Fabry's disease|Fabry disease]]
|-
* [[Nail-patella syndrome]]
|
* [[Lipodystrophy|Partial lipodystrophy]]
|
* [[Pre-eclampsia|Preeclampsia]]
|
|-
|
|
|
|}
|}
*
*
*
*[[Minimal change disease]]
*[[Focal segmental glomerulosclerosis]] (FSGS)
*[[Membranous nephropathy]]
*[[Diabetic nephropathy]]
*Glomerular deposition diseases
**[[Light chain disease|Light chain deposition disease]]
**[[Amyloidosis|Renal amyloidosis]]
**[[Fibrillary-Immunotactoid Glomerulopathy]]
**[[Fabry's disease|Fabry's Disease]]
*Hereditary Nephropathies: are group of disorders that present with both the features of nephrotic and nephritic syndromes. When features of nephritic syndrome are associated with sensorineural deafness, it is called Alport’s syndrome. Other hereditary nephropathies include IgA Nephropathy, C1q Nephropathy, etc.
====Secondary Nephrotic Syndrome====
Secondary nephrotic syndrome is defined as nephrotic syndrome due to a primary etiology other than glomerular disorders, such as [[infection]]s, [[malignancies]], systemic conditions, and [[medication]]s. The most common cause of secondary nephrotic syndrome is [[diabetes mellitus]].
{| border="1" style="border-collapse:collapse; text-align:left;" cellpadding="5" align="center"
|+ '''''Common Causes of Secondary Nephrotic Syndrome<ref name="pmid18497417">{{cite journal| author=Hull RP, Goldsmith DJ| title=Nephrotic syndrome in adults. | journal=BMJ | year= 2008 | volume= 336 | issue= 7654 | pages= 1185-9 | pmid=18497417 | doi=10.1136/bmj.39576.709711.80 | pmc=PMC2394708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18497417 }} </ref><ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref>'''''
| bgcolor="#d9ff54" |'''Cause''' || bgcolor="#d9ff54" |'''Characteristic Features'''
|-
| bgcolor="#ececec" |'''[[Diabetes Mellitus]]''' ||
*[[Glucosuria]]
*[[Hyperglycemia]]
*[[Polyuria]]
*[[Polydipsia]]
|-
| bgcolor="#ececec" |'''[[Systemic Lupus Erythematosus]] (SLE)''' ||
*[[Anemia]]
*[[Arthralgia]]
*[[Malar rash|Malar]] or discoid rash
*[[Photosensitivity]]
*Neurological, renal, immunologic disease
*[[Pleural effusion]] or [[ascites]]
*Positive [[ANA]] and [[anti-dsDNA]] antibodies
|-
| bgcolor="#ececec" |'''[[Viral Hepatitis]] ([[HBV]] and [[HCV]])''' ||
*Elevated [[liver function test]]s
*History of transfusions
*High-risk sexual encounters
*IV drug use
*Positive HBsAg or [[HCV]] RNA
|-
| bgcolor="#ececec" |'''[[NSAID]]s''' ||
*Associated with minimal change disease (MCD)
|-
| bgcolor="#ececec" |'''[[Amyloidosis]]''' ||
*[[Cardiomyopathy]]
*[[Hepatomegaly]]
*[[Peripheral neuropathy]]
|-
| bgcolor="#ececec" |'''[[Multiple Myeloma]]''' ||
*Abrnomal [[urine protein electrophoresis]] (UPEP) and [[serum protein electrophoresis]] (SPEP)
*[[Back pain]]
*[[Renal failure]]
*[[Anemia]]
*[[Hypercalcemia]]
*Positive serum beta-2-microglobulin
|-
| bgcolor="#ececec" |'''[[HIV]]''' ||
*Reduced [[CD4]] cell counts
*High-risk sexual encounters
*History of transfusions
*History of drug use
*Renal pathology similar to [[focal segmental glomerulosclerosis]] (FSGS)
|-
| bgcolor="#ececec" |'''[[Preeclampsia]]''' ||
*[[High blood pressure]], [[edema]], and [[proteinuria]] during pregnancy
|}
<center><sup>Adapted from Hull RP, Goldsmith DJ. Nephrotic syndrome in adults. BMJ. 2008; 336:1185-9</sup></center>
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
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Latest revision as of 22:57, 29 July 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Ali Poyan Mehr, M.D. [2]; Associate Editor(s)-in-Chief: Olufunmilola Olubukola M.D.[3], Yazan Daaboul, Serge Korjian

Overview

Nephrotic syndrome can be classified into primary or secondary depending on the underlying etiology. Primary (idiopathic) nephrotic syndrome is defined as nephrotic syndrome due to a primary glomerular disease. The secondary nephrotic syndrome is defined as nephrotic syndrome due to a primary etiology other than glomerular disorders, such as infections, malignancies, systemic conditions, and medications.

Classification

  • Nephrotic syndrome can be classified into primary or secondary depending on the underlying etiology.[1]
 
 
 
Nephrotic
syndrome
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Primary
 
 
 
Secondary
  • Below table lists different types of nephrotic syndromes:[2][3][4]
Different types of nephrotic syndromes Disease name
Podocytopathies Primary Minimal change disease
Focal segmental glomerulosclerosis (FSGS)
Secondary Infection such as:
Drugs/toxins such as:
  • NSAIDs
  • Interferon
  • Pamidronate
  • Lithium
  • Vaccins
  • Envenomation
Malignancies such as:
Genetic disorders such as:
Membranous glomerulonephritis Primary Membranous nephropathy
Secondary
Membranoproliferative glomerulonephritis Secondary
Hereditary Nephropathy
Glomerular deposition diseases
Others

References

  1. Kodner C (2009). "Nephrotic syndrome in adults: diagnosis and management". Am Fam Physician. 80 (10): 1129–34. PMID 19904897.
  2. Braden GL, Mulhern JG, O'Shea MH, Nash SV, Ucci AA, Germain MJ (May 2000). "Changing incidence of glomerular diseases in adults". Am. J. Kidney Dis. 35 (5): 878–83. PMID 10793022.
  3. Heaf J (September 2004). "The Danish Renal Biopsy Register". Kidney Int. 66 (3): 895–7. doi:10.1111/j.1523-1755.2004.00832.x. PMID 15327377.
  4. Ha TS (March 2017). "Genetics of hereditary nephrotic syndrome: a clinical review". Korean J Pediatr. 60 (3): 55–63. doi:10.3345/kjp.2017.60.3.55. PMC 5383633. PMID 28392820.