Amyloidosis Classification: Difference between revisions
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== 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== | |||
===Amyloidosis may be classified based on [[precursor]] of amyloidogenic [[protein]] into different subtypes, include:=== | |||
{| 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;" + |Clinical syndrome | |||
|- | |||
| style="background:#DCDCDC;" |[[Primary amyloidosis|'''AL (primary amyloidosis)''']] | |||
|[[Light chain|Light chains]] of [[Immunoglobulin|immunoglobulines]] (most common type) | |||
|[[Monoclonal gammopathy]] | |||
|- | |||
| style="background:#DCDCDC;" |[[AA amyloidosis|'''AA (secondary amyloidosis)''']] | |||
|[[Serum amyloid A|Serum amyloid A protein]] | |||
|[[Chronic inflammation|Chronic inflammatory diseases]] | |||
|- | |||
| style="background:#DCDCDC;" |'''AF''' | |||
|Mutant [[transthyretin]], [[Apolipoprotein A1|A1-apolipoprotein,]] [[gelsolin]], [[fibrinogen]], etc. | |||
|Familial [[polyneuropathy]]/[[cardiomyopathy]]/[[nephropathy]] | |||
|- | |||
| style="background:#DCDCDC;" |'''ATTRwt''' | |||
|Wild-type [[transthyretin]] | |||
|[[Senile]] [[restrictive cardiomyopathy]] _ [[Transthyretin]]-related amyloidosis wild-type | |||
|- | |||
| style="background:#DCDCDC;" |'''AH''' | |||
|ß2-microglobulin | |||
|Long-term [[hemodialysis]] | |||
|} | |||
===Amyloidosis also may classified by their extension of organ involvement as below:=== | |||
{| 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;" + |Causes | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Important clinical findings | |||
|- | |||
! rowspan="3" style="background:#DCDCDC;" |Systemic amyloidosis | |||
! style="background:#DCDCDC;" |[[AL amyloidosis|Primary amyloidosis (AL)]] | |||
| | |||
*Aggregation and deposition of [[immunoglobulin]] [[Light chain|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 [[Hemorrhagic diathesis|bleeding diathesis]] | |||
|- | |||
! style="background:#DCDCDC;" |[[AA amyloidosis|Secondary amyloidosis (AA)]] | |||
| | |||
*Chronic [[inflammation]] ([[Tuberculosis|TB]], [[familial mediterranean fever]], [[rheumatoid arthritis]] and [[multiple myeloma]]) | |||
| | |||
*[[Nephrotic syndrome]] | |||
*[[Congestive heart failure|Heart failure]] | |||
|- | |||
! style="background:#DCDCDC;" |[[Heredity|Hereditary]] amyloidosis | |||
| | |||
*Amyloidogenic [[Mutation|mutations]] and subsequently deposition of [[Amyloid|amyloids]] | |||
| | |||
*[[Congestive heart failure|Heart failure]] | |||
*[[Cardiac arrhythmia|Arrhythmia]] | |||
|- | |||
! rowspan="5" style="background:#DCDCDC;" |Organ-specific amyloidosis | |||
! style="background:#DCDCDC;" |[[Renal amyloidosis]] | |||
| rowspan="5" | | |||
*[[AL amyloidosis|Immunoglobulin light-chain amyloidosis (AL amyloidosis)]] | |||
*[[Transthyretin-related hereditary amyloidosis|Transthyretin-related amyloidosis]] (associated with familial/mutant or senile/wild-type [[Transthyretin|TTR]]) | |||
| | |||
*[[Proteinuria]] | |||
*[[Nephrotic syndrome]] | |||
*[[Chronic renal failure]] | |||
|- | |||
! style="background:#DCDCDC;" |[[Cardiac amyloidosis]] | |||
| | |||
*[[Systolic dysfunction]] | |||
*[[Diastolic dysfunction]] | |||
*[[Cardiac arrhythmia|Arrhythmia]] | |||
|- | |||
! style="background:#DCDCDC;" |[[Hepatic amyloidosis with intrahepatic cholestasis|Hepatic amyloidosis]] | |||
| | |||
*[[Hepatomegaly]] | |||
*Elevated [[liver enzymes]] | |||
|- | |||
! style="background:#DCDCDC;" |Amyloid neuropathy | |||
| | |||
*[[Peripheral neuropathy]] and [[autonomic neuropathy]] | |||
*[[Neurodegenerative disease|Neurodegenerative disorders]] | |||
**[[Parkinson's disease|Parkinson]], [[Alzheimer's disease|Alzheimer]], and [[Huntington's disease]] | |||
|- | |||
! style="background:#DCDCDC;" |Gastrointestinal amyloidosis | |||
| | |||
*Nonspecific findings | |||
**[[Dyspepsia]], [[abdominal pain]], [[diarrhea]], [[malabsorption]] | |||
|} | |||
==Refrences== | ==Refrences== |
Revision as of 17:01, 24 October 2019
Amyloidosis Microchapters |
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Amyloidosis Classification On the Web |
American Roentgen Ray Society Images of Amyloidosis Classification |
Risk calculators and risk factors for Amyloidosis Classification |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Template:Shamila
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
Amyloidosis may be classified based on precursor of amyloidogenic protein into different subtypes, include:
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 |
Amyloidosis also may classified by their extension of organ involvement as below:
Classification | subtypes | Causes | Important clinical findings |
---|---|---|---|
Systemic amyloidosis | Primary amyloidosis (AL) |
|
|
Secondary amyloidosis (AA) |
|
||
Hereditary amyloidosis | |||
Organ-specific amyloidosis | Renal amyloidosis |
|
|
Cardiac amyloidosis | |||
Hepatic amyloidosis |
| ||
Amyloid neuropathy | |||
Gastrointestinal amyloidosis |
|