Nephrotic syndrome classification: Difference between revisions

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Revision as of 18:07, 15 May 2018

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. 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

Etiologic Classification

Nephrotic syndrome can be classified into primary or secondary depending on the underlying etiology.

Nephrotic
syndrome

Primary

Secondary

Primary (Idiopathic) Nephrotic Syndrome

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:^[1]

Different types of nephrotic syndromes		Specific type
Podocytopathies	Primary	Primary minimal change disease
	Primary	Primary FSGS
	Secondary	Infection: HIV
		Drugs/toxins: NSAIDs, interferon, pamironate, lithium, vaccins, envenomation
		Malignancies: hodgkin lymphoma, thymoma
		Genetics: nephrin mutations, podocin
Membranous nephropathies	Primary
	Secondary

Minimal change disease
Focal segmental glomerulosclerosis (FSGS)
Membranous nephropathy
Diabetic nephropathy
Glomerular deposition diseases
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 infections, malignancies, systemic conditions, and medications. The most common cause of secondary nephrotic syndrome is diabetes mellitus.

**Common Causes of Secondary Nephrotic Syndrome^[2]^[1]**
Cause	Characteristic Features
Diabetes Mellitus	Glucosuria Hyperglycemia Polyuria Polydipsia
Systemic Lupus Erythematosus (SLE)	Anemia Arthralgia Malar or discoid rash Photosensitivity Neurological, renal, immunologic disease Pleural effusion or ascites Positive ANA and anti-dsDNA antibodies
Viral Hepatitis (HBV and HCV)	Elevated liver function tests History of transfusions High-risk sexual encounters IV drug use Positive HBsAg or HCV RNA
NSAIDs	Associated with minimal change disease (MCD)
Amyloidosis	Cardiomyopathy Hepatomegaly Peripheral neuropathy
Multiple Myeloma	Abrnomal urine protein electrophoresis (UPEP) and serum protein electrophoresis (SPEP) Back pain Renal failure Anemia Hypercalcemia Positive serum beta-2-microglobulin
HIV	Reduced CD4 cell counts High-risk sexual encounters History of transfusions History of drug use Renal pathology similar to focal segmental glomerulosclerosis (FSGS)
Preeclampsia	High blood pressure, edema, and proteinuria during pregnancy

^{Adapted from Hull RP, Goldsmith DJ. Nephrotic syndrome in adults. BMJ. 2008; 336:1185-9}

References

↑ ^1.0 ^1.1 Kodner C (2009). "Nephrotic syndrome in adults: diagnosis and management". Am Fam Physician. 80 (10): 1129–34. PMID 19904897.
↑ Hull RP, Goldsmith DJ (2008). "Nephrotic syndrome in adults". BMJ. 336 (7654): 1185–9. doi:10.1136/bmj.39576.709711.80. PMC 2394708. PMID 18497417.CS1 maint: PMC format (link)

Template:WH Template:WS

[pmid19904897-1] 1.0 ^1.1 Kodner C (2009). "Nephrotic syndrome in adults: diagnosis and management". Am Fam Physician. 80 (10): 1129–34. PMID 19904897.

[pmid18497417-2] Hull RP, Goldsmith DJ (2008). "Nephrotic syndrome in adults". BMJ. 336 (7654): 1185–9. doi:10.1136/bmj.39576.709711.80. PMC 2394708. PMID 18497417.CS1 maint: PMC format (link)

[1]

[2]

@@ Line 17: / Line 17: @@
 </center>
 ====Primary (Idiopathic) Nephrotic Syndrome====
-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>:
+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"
+!Different types of nephrotic syndromes
+!
+!Specific type
+|-
+| rowspan="6" |Podocytopathies
+| rowspan="2" |Primary
+|Primary minimal change disease
+|-
+|Primary FSGS
+|-
+| rowspan="4" |Secondary
+|Infection: HIV
+|-
+|Drugs/toxins: NSAIDs, interferon, pamironate, lithium, vaccins, envenomation
+|-
+|Malignancies: hodgkin lymphoma, thymoma
+|-
+|Genetics: nephrin mutations, podocin
+|-
+|Membranous nephropathies
+|Primary
+|
+|-
+|
+|Secondary
+|
+|-
+|
+|
+|
+|}
+*
+*
+*
 *[[Minimal change disease]]
 *[[Focal segmental glomerulosclerosis]] (FSGS)
 *[[Membranous nephropathy]]
-*[[Membranoproliferative glomerulonephritis]]
+*[[Diabetic nephropathy]]
-*[[IgM nephropathy]]
+*Glomerular deposition diseases
-*C1q nephropathy
+**[[Light chain disease|Light chain deposition 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.
+**[[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====
@@ Line 31: / Line 69: @@
 {| 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="#d9ff54" |'''Cause''' || bgcolor="#d9ff54" |'''Characteristic Features'''
 |-
-| bgcolor="#ececec"|'''[[Diabetes Mellitus]]''' ||
+| bgcolor="#ececec" |'''[[Diabetes Mellitus]]''' ||
 *[[Glucosuria]]
 *[[Hyperglycemia]]
@@ Line 39: / Line 77: @@
 *[[Polydipsia]]
 |-
-| bgcolor="#ececec"|'''[[Systemic Lupus Erythematosus]] (SLE)''' ||
+| bgcolor="#ececec" |'''[[Systemic Lupus Erythematosus]] (SLE)''' ||
 *[[Anemia]]
 *[[Arthralgia]]
@@ Line 48: / Line 86: @@
 *Positive [[ANA]] and [[anti-dsDNA]] antibodies
 |-
-| bgcolor="#ececec"|'''[[Viral Hepatitis]] ([[HBV]] and [[HCV]])''' ||
+| bgcolor="#ececec" |'''[[Viral Hepatitis]] ([[HBV]] and [[HCV]])''' ||
 *Elevated [[liver function test]]s
 *History of transfusions
@@ Line 55: / Line 93: @@
 *Positive HBsAg or [[HCV]] RNA
 |-
-| bgcolor="#ececec"|'''[[NSAID]]s''' ||
+| bgcolor="#ececec" |'''[[NSAID]]s''' ||
 *Associated with minimal change disease (MCD)
 |-
-|bgcolor="#ececec"|'''[[Amyloidosis]]''' ||
+| bgcolor="#ececec" |'''[[Amyloidosis]]''' ||
 *[[Cardiomyopathy]]
@@ Line 64: / Line 102: @@
 *[[Peripheral neuropathy]]
 |-
-| bgcolor="#ececec"|'''[[Multiple Myeloma]]''' ||
+| bgcolor="#ececec" |'''[[Multiple Myeloma]]''' ||
 *Abrnomal [[urine protein electrophoresis]] (UPEP) and [[serum protein electrophoresis]] (SPEP)
 *[[Back pain]]
@@ Line 72: / Line 110: @@
 *Positive serum beta-2-microglobulin
 |-
-| bgcolor="#ececec"|'''[[HIV]]''' ||
+| bgcolor="#ececec" |'''[[HIV]]''' ||
 *Reduced [[CD4]] cell counts
 *High-risk sexual encounters
@@ Line 79: / Line 117: @@
 *Renal pathology similar to [[focal segmental glomerulosclerosis]] (FSGS)
 |-
-| bgcolor="#ececec"|'''[[Preeclampsia]]''' ||
+| bgcolor="#ececec" |'''[[Preeclampsia]]''' ||
 *[[High blood pressure]], [[edema]], and [[proteinuria]] during pregnancy
 |}