Amyloidosis classification
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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 | Abbreviation | Amyloidogenic Protein/Fibril | Acquired/Inherited | Most Common Organ Involvement | Associated Conditions |
|---|---|---|---|---|---|
| Primary amyloidosis | AL |
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Acquired | Heart and kidneys | |
| Secondary amyloidosis | AA | Acquired | Kidneys (early), heart and liver (late) |
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| Senile systemic amyloidosis | ATTRwt |
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Acquired or Hereditary | Heart and nerves (more common in hereditary type) |
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| β2-microglobulin-related amyloidosis | AH | Acquired or Hereditary | Nerves (peripheral and autonomic) |
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| Leucocyte cell–derived chemotaxin 2 related amyloidosis | ALect2 |
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Acquired | Kidneys and liver |
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| Fibrinogen A alpha-chain associated amyloidosis | AF | Hereditary | Kidneys and liver |
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| Abnormal Apolipoprotein A-I, AII, and AIV related amyloidosis | AApoA-I |
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Hereditary | Kidneys, liver and nerves (peripheral) |
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| Lysozyme amyloid related amyloidosis | ALys |
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Hereditary | Liver and kidneys |
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| Gelsolin amyloid related amyloidosis | AGel |
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Hereditary | Kidneys and nerves (peripheral and cranial) |
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Classification Based on Organ Involvement:[3][4]
| Classification | Subtypes | Causes | Clinical Features |
|---|---|---|---|
| Systemic amyloidosis | Primary amyloidosis (AL) |
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| Secondary amyloidosis (AA) |
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| Hereditary amyloidosis | |||
| Organ-specific amyloidosis | Renal amyloidosis |
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| Cardiac amyloidosis | |||
| Hepatic amyloidosis |
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| Amyloid neuropathy | |||
| Gastrointestinal amyloidosis |
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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.
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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.