Amyloidosis Classification: Difference between revisions

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Revision as of 15:28, 25 October 2019

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

Overview

Amyloidosis may be classified on the basis of type of amyloidogenic protein and associated clinical syndromes into primary (AL) amyloidosis, secondary (AA) amyloidosis, familial (AF) amyloidosis, transthyretin (ATTRwt) amyloidosis and dialysis-associated (AH) amyloidosis. It can also be classified based on extent of organ system involvement.

Classification

Classification Based on Precursor of Amyloidogenic Protein:

Type	Amyloidogenic Protein/Fibril	Clinical Syndrome
AL (primary amyloidosis)	Light chains of immunoglobulines (most common type)	Monoclonal gammopathy
AA (secondary amyloidosis)	Serum amyloid A protein	Chronic inflammatory diseases
AF	Mutant transthyretin, A1-apolipoprotein, gelsolin, fibrinogen, etc.	Familial polyneuropathy/cardiomyopathy/nephropathy
ATTRwt	Wild-type transthyretin	Senile restrictive cardiomyopathy _ Transthyretin-related amyloidosis wild-type
AH	ß2-microglobulin	Long-term hemodialysis

Classification Based on Organ Involvement:

Classification	Subtypes	Causes	Clinical Features
Systemic amyloidosis	Primary amyloidosis (AL)	Aggregation and deposition of immunoglobulin light chains that usually produced by plasma cell clones	Nephrotic syndrome Restrictive cardiomyopathy Peripheral neuropathy Hepatomegaly with elevated liver enzymes Macroglossia Purpura and an unexplained bleeding diathesis
	Secondary amyloidosis (AA)	Chronic inflammation (TB, familial mediterranean fever, rheumatoid arthritis and multiple myeloma)	Nephrotic syndrome Heart failure
	Hereditary amyloidosis	Amyloidogenic mutations and subsequently deposition of amyloids	Heart failure Arrhythmia
Organ-specific amyloidosis	Renal amyloidosis	Immunoglobulin light-chain amyloidosis (AL amyloidosis) Transthyretin-related amyloidosis (associated with familial/mutant or senile/wild-type TTR)	Proteinuria Nephrotic syndrome Chronic renal failure
	Cardiac amyloidosis		Systolic dysfunction Diastolic dysfunction Arrhythmia
	Hepatic amyloidosis		Hepatomegaly Elevated liver enzymes
	Amyloid neuropathy		Peripheral neuropathy and autonomic neuropathy Neurodegenerative disorders: Parkinson, Alzheimer, and Huntington's disease
	Gastrointestinal amyloidosis		Nonspecific findings: Dyspepsia, abdominal pain, diarrhea, malabsorption

Refrences

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-{{CMG}}; {{AE}} {{Shamila}}
+{{CMG}}; {{AE}}
 == Overview ==
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 ==Classification==
-===Amyloidosis may be classified based on [[precursor]] of amyloidogenic [[protein]] into different subtypes, include:===
+===Classification Based on [[Precursor]] of Amyloidogenic [[Protein]]:===
 {| class="wikitable"
 ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Type
-! align="center" style="background:#4479BA; color: #FFFFFF;" + |Amyloidogenic protein/ fibril
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Amyloidogenic Protein/Fibril
-! align="center" style="background:#4479BA; color: #FFFFFF;" + |Clinical syndrome
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Clinical Syndrome
 |-
 | style="background:#DCDCDC;" |[[Primary amyloidosis|'''AL (primary amyloidosis)''']]
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 |Long-term [[hemodialysis]]
 |}
-===Amyloidosis also may classified by their extension of organ involvement as below:===
+===Classification Based on Organ Involvement:===
 {| class="wikitable"
 ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Classification
-! align="center" style="background:#4479BA; color: #FFFFFF;" + |subtypes
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Subtypes
 ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Causes
-! align="center" style="background:#4479BA; color: #FFFFFF;" + |Important clinical findings
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Clinical Features
 |-
 ! rowspan="3" style="background:#DCDCDC;" |Systemic amyloidosis
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 |
 *[[Peripheral neuropathy]] and [[autonomic neuropathy]]
-*[[Neurodegenerative disease|Neurodegenerative disorders]]
+*[[Neurodegenerative disease|Neurodegenerative disorders]]:
 **[[Parkinson's disease|Parkinson]], [[Alzheimer's disease|Alzheimer]], and [[Huntington's disease]]
 |-
 ! style="background:#DCDCDC;" |Gastrointestinal amyloidosis
 |
-*Nonspecific findings
+*Nonspecific findings:
 **[[Dyspepsia]], [[abdominal pain]], [[diarrhea]], [[malabsorption]]
 |}