Amyloidosis classification

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	Amyloidosis Microchapters
Home
Patient Information
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
Pathophysiology
Causes
Differentiating Amyloidosis from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X-ray
Echocardiography and Ultrasound
CT scan
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Amyloidosis classification On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Amyloidosis classification All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Amyloidosis classification
CDC on Amyloidosis classification
Amyloidosis classification in the news
Blogs on Amyloidosis classification
Directions to Hospitals Treating Psoriasis
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	Abbreviation	Amyloidogenic Protein/Fibril	Acquired/Inherited	Most Common Organ Involvement	Associated Conditions
Primary amyloidosis	AL	Light chains of immunoglobulines (most common type)	Acquired	Heart and kidneys	Monoclonal gammopathy
Secondary amyloidosis	AA	Serum amyloid A protein	Acquired	Kidneys (early), heart and liver (late)	Chronic inflammatory diseases: Rheumatoid arthritis Juvenile inflammatory arthritis Intravenous drug use Familial periodic fever syndromes
Fibrinogen A alpha-chain associated amyloidosis	AF	Mutant transthyretin A1-apolipoprotein, Gelsolin Fibrinogen	Hereditary		Familial polyneuropathy/cardiomyopathy/nephropathy
Senile systemic amyloidosis	ATTRwt	Wild-type transthyretin Abnormal TTR gene	Acquired or Hereditary	Heart and nerves (more common in hereditary type)	Senile restrictive cardiomyopathy/Transthyretin-related amyloidosis wild-type
β2-microglobulin-related amyloidosis	AH	ß2-microglobulin	Acquired or Hereditary	Nerves (peripheral and autonomic)	Long-term hemodialysis
Leucocyte cell–derived chemotaxin 2 related amyloidosis	ALect2
Abnormal Apolipoprotein A-I related amyloidosis	AApoA-I
Lysozyme amyloid related amyloidosis	ALys
Gelosin amyloid related amyloidosis	AGel

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

Classification	Subtypes	Causes	Clinical Features
Systemic amyloidosis	Primary amyloidosis (AL)	Aggregation and deposition of immunoglobulin 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 bleeding diathesis
	Secondary amyloidosis (AA)	Chronic inflammation (TB, familial mediterranean fever, rheumatoid arthritis and multiple myeloma)	Nephrotic syndrome Heart failure
	Hereditary amyloidosis	Amyloidogenic mutations and subsequently deposition of amyloids	Heart failure Arrhythmia
Organ-specific amyloidosis	Renal amyloidosis	Immunoglobulin light-chain amyloidosis (AL amyloidosis) Transthyretin-related amyloidosis (associated with familial/mutant or senile/wild-type TTR)	Proteinuria Nephrotic syndrome Chronic renal failure
	Cardiac amyloidosis		Systolic dysfunction Diastolic dysfunction Arrhythmia
	Hepatic amyloidosis		Hepatomegaly Elevated liver enzymes
	Amyloid neuropathy		Peripheral neuropathy and autonomic neuropathy Neurodegenerative disorders: Parkinson, Alzheimer, and Huntington's disease
	Gastrointestinal amyloidosis		Nonspecific findings: Dyspepsia, abdominal pain, diarrhea, malabsorption

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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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.

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[pmid28134587-1] 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.

[pmid30614283-2] 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)

[pmid26719234-3] 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.

[pmid27634125-4] 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.

[pmid25378951-5] 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.

[pmid24998818-6] Misumi Y, Ando Y (July 2014). "[Classification of amyloidosis]". Brain Nerve (in Japanese). 66 (7): 731–7. PMID 24998818.

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Amyloidosis classification

Overview

Classification

Classification Based on Precursor of Amyloidogenic Protein: [1][2]

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

Refrences

Overview

Classification

References

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Classification Based on Precursor of Amyloidogenic Protein: ^[1]^[2]

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