Amyloidosis causes

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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
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Amyloidosis can be systemic or organ specific. In systemic amyloidosis, the secondary form (AL, AA) is much more common than the primary form.

Causes

Systemic Amyloidosis

Primary/Hereditary Amyloidosis

These rare hereditary disorders are usually due to point mutations in precursor proteins, and are also usually autosomal dominantly transmitted. The precursor proteins are:

• Transthyretin-the most commonly implicated protein.
• Lysozyme
• Apolipoprotein B
• Fibrinogen
• Apolipoprotein A1
• Gelsolin

Secondary Amyloidosis

These are far more common than the primary amyloidoses.

• AL amyloidosis: (immunoglobulin light chains are the precursor protein, overproduced in multiple myeloma). This is sometimes, confusingly and erroneously, called 'primary amyloidosis'.
• AA amyloidosis: (the precursor protein is serum amyloid A protein (SAA), an acute-phase protein due to chronic inflammation). In contrast to AL amyloid, this has previously been termed 'secondary amyloidosis'. These occur with a wide variety of diseases associated with chronic inflammation, such as rheumatoid arthritis, familial Mediterranean fever or chronic infection.
• Dialysis related amyloidosis: (the precursor protein is beta-2-microglobulin which is not removed with dialysis, and thus accumulates in patients with end stage renal failure on dialysis).

Organ-Specific Amyloidosis

In almost all of the organ-specific pathologies, there is significant debate as to whether the amyloid plaques are the causal agent of the disease or instead a downstream consequence of a common idiopathic agent. The associated proteins are indicated in parentheses.

Neurological Amyloid

• Alzheimer's disease (Aβ 39-43)
• Parkinson's disease (alpha-synuclein)
• Huntington's disease (huntingtin)
• Transmissible spongiform encephalopathies caused by prion protein (PrP) were sometimes classed as amyloidoses, as one of the four pathological features in diseased tissue is the presence of amyloid plaques. These diseases include
  • Creutzfeldt-Jakob disease (PrP in cerebrum)
  • Kuru (diffuse PrP deposits in brain)
  • Fatal Familial Insomnia (PrP in thalamus)
  • Bovine spongiform encephalopathy (PrP in cerebrum of cows)

Cardiovascular amyloid

• Cardiac amyloidosis
• See the chapter on CMR in Cardiac Amyloidosis
  • Senile cardiac amyloidosis-may cause heart failure
• Congophilic angiopathy

Other

• Amylin deposition can occur in the pancreas in some cases of type 2 diabetes mellitus