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

[edit | edit source]

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

[edit | edit source]

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

[edit | edit source]

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]

[edit | edit source]

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

[edit | edit source]

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.

Template:WH Template:WS