Amyloidosis classification: Difference between revisions

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** Familial periodic fever syndromes
** Familial periodic fever syndromes
|-
|-
| style="background:#DCDCDC;" |'''Senile systemic amyloidosis'''
| style="background:#DCDCDC;" |'''Senile systemic or wild-type amyloidosis'''
|ATTRwt
|ATTRwt/ATTRvar
|
|
*[[Transthyretin|Wild-type transthyretin (TTR)]]
*[[Transthyretin|Wild-type transthyretin (TTRwt)]]
* Abnormal TTR gene
* Abnormal TTR gene (TTRvar)
|[[Acquired disorder|Acquired]] or [[Hereditary]]
|[[Acquired disorder|Acquired]] (ATTRwt) or [[Hereditary]] (ATTRvar)
|[[Heart]] and [[nerves]] (more common in hereditary type)
|[[Heart]] and [[nerves]] (more common in hereditary type)
|
|
* [[Senile]] [[restrictive cardiomyopathy]]/[[Transthyretin]]-related amyloidosis wild-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'''
| style="background:#DCDCDC;" |'''β2-microglobulin related amyloidosis'''
|AH  
|AH  
|
|
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*[[Gastrointestinal tract|Gastrointestinal]] involvement
*[[Gastrointestinal tract|Gastrointestinal]] involvement
|-
|-
| style="background:#DCDCDC;" |'''Gelsolin amyloid related amyloidosis'''
| style="background:#DCDCDC;" |'''Gelsolin related amyloidosis'''
|AGel
|AGel
|
|
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{{Amyloidosis}}
ocrinology]]
{{CMG}}; {{AE}} {{SHH}}
 
== Overview ==
 
==Classification==
 
Amyloidosis may be classified based on [[precursor]] of 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>
{| class="wikitable"
!Type
!Amyloidogenic protein/ fibril
!Clinical syndrome
|-
|AL
|Light chains of immunoglobulines (most common type)
|Monoclonal gammopathy
|-
|AA
|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
|}
 
== References ==
{{reflist|2}}
 
[[Category:Disease]]
[[Category:Rheumatology]]
[[Category:Cardiology]]
[[Category:Endocrinology]]


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Latest revision as of 21:52, 10 December 2019

Amyloidosis Microchapters

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Overview

Historical Perspective

Classification

• Primary amyloidosis
• Secondary amyloidosis
• Familial amyloidosis
• Wild-type (senile) amyloidosis
• Cardiac amyloidosis
• Beta-2 microglobulin related amyloidosis
• Gelsolin related amyloidosis
• Lysozyme amyloid related amyloidosis
• Leucocyte cell-derived chemotaxin 2 related amyloidosis
• Fibrinogen A alpha-chain associated amyloidosis

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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.

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