Amyloidosis Classification

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:

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:===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.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.

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:

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