Amyloidosis classification: Difference between revisions
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{{Amyloidosis}} | |||
{{CMG}}{{AE}}{{SHH}} | |||
== 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]]:''' <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" | |||
! 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]] | |||
|} | |||
===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;" |[[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== | |||
{{reflist|2}} | |||
{{WH}} | |||
{{WS}} | |||
{{Amyloidosis}} | {{Amyloidosis}} | ||
{{CMG}}; {{AE}} {{SHH}} | {{CMG}}; {{AE}} {{SHH}} | ||
Revision as of 23:07, 25 October 2019
|
Amyloidosis Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
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: Shaghayegh Habibi, M.D.[2]
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 | 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:[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
- ↑ 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.
- ↑ 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)
- ↑ 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.
- ↑ 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.
|
Amyloidosis Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
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. [3]; Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[4]
Overview
Classification
Amyloidosis may be classified based on precursor of amyloidogenic protein into different subtypes, include:[1][2]
| 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
- ↑ Real de Asúa D, Costa R, Galván JM, Filigheddu MT, Trujillo D, Cadiñanos J (2014). "Systemic AA amyloidosis: epidemiology, diagnosis, and management". Clin Epidemiol. 6: 369–77. doi:10.2147/CLEP.S39981. PMC 4218891. PMID 25378951.
- ↑ Misumi Y, Ando Y (July 2014). "[Classification of amyloidosis]". Brain Nerve (in Japanese). 66 (7): 731–7. PMID 24998818.