Amyloidosis classification: Difference between revisions

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{{Amyloidosis}}
{{Amyloidosis}}
{{CMG}}; {{AE}} {{SHH}}
 
{{CMG}}{{AE}}{{HK}}{{SHH}}


== Overview ==
== 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==


Amyloidosis may be classified according to [[precursor]] amyloidogenic [[protein]] into different subtypes, include:<ref name="pmid25378951">{{cite journal |vauthors=Real de Asúa D, Costa R, Galván JM, Filigheddu MT, Trujillo D, Cadiñanos J |title=Systemic AA amyloidosis: epidemiology, diagnosis, and management |journal=Clin Epidemiol |volume=6 |issue= |pages=369–77 |date=2014 |pmid=25378951 |pmc=4218891 |doi=10.2147/CLEP.S39981 |url=}}</ref><ref name="pmid24998818">{{cite journal |vauthors=Misumi Y, Ando Y |title=[Classification of amyloidosis] |language=Japanese |journal=Brain Nerve |volume=66 |issue=7 |pages=731–7 |date=July 2014 |pmid=24998818 |doi= |url=}}</ref>
=== '''Classification Based on [[Precursor]] of Amyloidogenic [[Protein]]:''' <ref name="pmid28134587">{{cite journal |vauthors=Khoor A, Colby TV |title=Amyloidosis of the Lung |journal=Arch. Pathol. Lab. Med. |volume=141 |issue=2 |pages=247–254 |date=February 2017 |pmid=28134587 |doi=10.5858/arpa.2016-0102-RA |url=}}</ref><ref name="pmid30614283">{{cite journal |vauthors=Benson MD, Buxbaum JN, Eisenberg DS, Merlini G, Saraiva MJM, Sekijima Y, Sipe JD, Westermark P |title=Amyloid nomenclature 2018: recommendations by the International Society of Amyloidosis (ISA) nomenclature committee |journal=Amyloid |volume=25 |issue=4 |pages=215–219 |date=December 2018 |pmid=30614283 |doi=10.1080/13506129.2018.1549825 |url=}}</ref> ===
{| class="wikitable"
{| class="wikitable"
!Abbreviation
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Type
!Amyloidogenic protein
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Abbreviation
!Clinical syndrome
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Amyloidogenic Protein/Fibril
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Acquired/Inherited
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Most Common Organ Involvement
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Associated Conditions
|-
|-
|AL
| style="background:#DCDCDC;" |'''Primary amyloidosis'''
|Light chains of immunoglobulines (most common type)
|AL  
|Monoclonal gammopathy
|
* [[Light chain|Light chains]] of [[Immunoglobulin|immunoglobulines]] (most common type)
|[[Acquired disorder|Acquired]]
|[[Heart]] and [[kidneys]]
|
* [[Monoclonal gammopathy]]
|-
|-
| style="background:#DCDCDC;" |'''Secondary amyloidosis'''
|AA
|AA
|Serum amyloid A protein
|
|Chronic inflammatory diseases
* [[Serum amyloid A|Serum amyloid A protein]]
|[[Acquired disorder|Acquired]]
|[[Kidneys]] (early), [[heart]] and [[liver]] (late)
|
* [[Chronic inflammation|Chronic inflammatory diseases:]]
** [[Rheumatoid arthritis]]
** [[Juvenile arthritis|Juvenile inflammatory arthritis]]
** [[Intravenous drug use]]
** Familial periodic fever syndromes
|-
| style="background:#DCDCDC;" |'''Senile systemic or wild-type amyloidosis'''
|ATTRwt/ATTRvar
|
*[[Transthyretin|Wild-type transthyretin (TTRwt)]]
* Abnormal TTR gene (TTRvar)
|[[Acquired disorder|Acquired]] (ATTRwt) or [[Hereditary]] (ATTRvar)
|[[Heart]] and [[nerves]] (more common in hereditary type)
|
* [[Senile]] [[restrictive cardiomyopathy]]/[[Transthyretin]]-related amyloidosis wild-type
*ATTRwt common in males and exhibits involvement of [[ligaments]] and tenosynovium
*ATTRvar may involve [[eyes]] and [[leptomeninges]]
|-
| style="background:#DCDCDC;" |'''β2-microglobulin related amyloidosis'''
|AH
|
*[[Beta-2 microglobulin|ß2-microglobulin]]
|[[Acquired disorder|Acquired]] or [[Hereditary]]
|[[Nerves]] ([[Peripheral nerve|peripheral]] and [[Autonomic nervous system|autonomic]])
|
* Long-term [[hemodialysis]]
|-
| style="background:#DCDCDC;" |'''Leucocyte cell–derived chemotaxin 2 related amyloidosis'''
|ALect2
|
* Leucocyte cell–derived chemotaxin 2
|[[Acquired disorder|Acquired]]
|[[Kidneys]] and [[liver]]
|
*[[Nephrotic syndrome]]
*[[Proteinuria]]
 
*
|-
|-
| style="background:#DCDCDC;" |'''Fibrinogen A alpha-chain associated amyloidosis'''
|AF
|AF
|Mutant transthyretin, A1-apolipoprotein, gelsolin, fibrinogen, etc.
|
|Familial polyneuropathy/cardiomyopathy/nephropathy
*[[Fibrinogen]]
|[[Hereditary]]
|[[Kidneys]] and [[liver]]
|
* Familial [[polyneuropathy]]/[[cardiomyopathy]]/[[nephropathy]]
|-
| style="background:#DCDCDC;" |'''Abnormal Apolipoprotein A-I, AII, and AIV related amyloidosis'''
|AApoA-I
|
* Aberrant [[apolipoprotein A-I]], [[Apolipoprotein A2|A-II]] and A-IV proteins
|[[Hereditary]]
|[[Kidneys]], [[liver]] and [[nerves]] ([[Peripheral nerve|peripheral]])
|
*[[Testicular]] involvement (possible relation to [[steroidogenic]] tissues)
|-
| style="background:#DCDCDC;" |'''Lysozyme amyloid related amyloidosis'''
|ALys
|
* Aberrant [[lysozyme]]
|[[Hereditary]]
|[[Liver]] and [[kidneys]]
|
*[[Gastrointestinal tract|Gastrointestinal]] involvement
|-
| style="background:#DCDCDC;" |'''Gelsolin related amyloidosis'''
|AGel
|
* Abnormal [[gelsolin]]
|[[Hereditary]]
|[[Kidneys]] and [[nerves]] ([[Peripheral nerve|peripheral]] and [[Cranial nerves|cranial]])
|
*[[Cranial nerves|Cranial]] neuropathy
|}
 
===Classification Based on Organ Involvement:<ref name="pmid26719234">{{cite journal |vauthors=Wechalekar AD, Gillmore JD, Hawkins PN |title=Systemic amyloidosis |journal=Lancet |volume=387 |issue=10038 |pages=2641–2654 |date=June 2016 |pmid=26719234 |doi=10.1016/S0140-6736(15)01274-X |url=}}</ref><ref name="pmid27634125">{{cite journal |vauthors=Falk RH, Alexander KM, Liao R, Dorbala S |title=AL (Light-Chain) Cardiac Amyloidosis: A Review of Diagnosis and Therapy |journal=J. Am. Coll. Cardiol. |volume=68 |issue=12 |pages=1323–41 |date=September 2016 |pmid=27634125 |doi=10.1016/j.jacc.2016.06.053 |url=}}</ref>===
{| 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;" + |Clinical Features
|-
! rowspan="3" style="background:#DCDCDC;" |Systemic amyloidosis
! style="background:#DCDCDC;" |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;" |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;" |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]]
|-
|-
|ATTRwt
! style="background:#DCDCDC;" |Amyloid neuropathy
|Wild-type transthyretin
|
|Senile restrictive cardiomyopathy _ Transthyretin-related amyloidosis wild-type
*[[Peripheral neuropathy]] and [[autonomic neuropathy]]
*[[Neurodegenerative disease|Neurodegenerative disorders]]:
**[[Parkinson's disease|Parkinson]], [[Alzheimer's disease|Alzheimer]], and [[Huntington's disease]]
|-
|-
|AH
! style="background:#DCDCDC;" |Gastrointestinal amyloidosis
|ß2-microglobulin
|
|Long-term hemodialysis
*Nonspecific findings:
**[[Dyspepsia]], [[abdominal pain]], [[diarrhea]], [[malabsorption]]
|}
|}


== References ==
==Refrences==
{{reflist|2}}
{{reflist|2}}
{{WH}}
{{WS}}


[[Category:Disease]]
ocrinology]]
[[Category:Rheumatology]]
[[Category:Cardiology]]
[[Category:Endocrinology]]


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Latest revision as of 21:52, 10 December 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]Shaghayegh Habibi, M.D.[3]

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: [1][2]

Type Abbreviation Amyloidogenic Protein/Fibril Acquired/Inherited Most Common Organ Involvement Associated Conditions
Primary amyloidosis AL Acquired Heart and kidneys
Secondary amyloidosis AA Acquired Kidneys (early), heart and liver (late)
Senile systemic or wild-type amyloidosis ATTRwt/ATTRvar Acquired (ATTRwt) or Hereditary (ATTRvar) Heart and nerves (more common in hereditary type)
β2-microglobulin related amyloidosis AH Acquired or Hereditary Nerves (peripheral and autonomic)
Leucocyte cell–derived chemotaxin 2 related amyloidosis ALect2
  • Leucocyte cell–derived chemotaxin 2
 Acquired Kidneys and liver
Fibrinogen A alpha-chain associated amyloidosis AF Hereditary Kidneys and liver
Abnormal Apolipoprotein A-I, AII, and AIV related amyloidosis AApoA-I Hereditary Kidneys, liver and nerves (peripheral)
Lysozyme amyloid related amyloidosis ALys Hereditary Liver and kidneys
Gelsolin related amyloidosis AGel Hereditary Kidneys and nerves (peripheral and cranial)

Classification Based on Organ Involvement:[3][4]

Classification Subtypes Causes Clinical Features
Systemic amyloidosis Primary amyloidosis (AL)
Secondary amyloidosis (AA)
Hereditary amyloidosis
Organ-specific amyloidosis Renal amyloidosis
Cardiac amyloidosis
Hepatic amyloidosis
Amyloid neuropathy
Gastrointestinal amyloidosis

Refrences

  1. ↑ Khoor A, Colby TV (February 2017). "Amyloidosis of the Lung". Arch. Pathol. Lab. Med. 141 (2): 247–254. doi:10.5858/arpa.2016-0102-RA. PMID 28134587.
  2. ↑ Benson MD, Buxbaum JN, Eisenberg DS, Merlini G, Saraiva M, Sekijima Y, Sipe JD, Westermark P (December 2018). "Amyloid nomenclature 2018: recommendations by the International Society of Amyloidosis (ISA) nomenclature committee". Amyloid. 25 (4): 215–219. doi:10.1080/13506129.2018.1549825. PMID 30614283. Vancouver style error: initials (help)
  3. ↑ Wechalekar AD, Gillmore JD, Hawkins PN (June 2016). "Systemic amyloidosis". Lancet. 387 (10038): 2641–2654. doi:10.1016/S0140-6736(15)01274-X. PMID 26719234.
  4. ↑ Falk RH, Alexander KM, Liao R, Dorbala S (September 2016). "AL (Light-Chain) Cardiac Amyloidosis: A Review of Diagnosis and Therapy". J. Am. Coll. Cardiol. 68 (12): 1323–41. doi:10.1016/j.jacc.2016.06.053. PMID 27634125.

Template:WH Template:WS

ocrinology]]

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