What is the classification of amyloidosis?

The four main types of systemic amyloidosis are light-chain amyloidosis, reactive amyloid A amyloidosis due to chronic inflammatory diseases, β2-microglobulin amyloidosis associated with long-term hemodialysis, and hereditary transthyretin-related amyloidosis caused by mutations in the transthyretin gene.

What are the 4 types of amyloidosis?

Types of amyloidosis include:
  • AL amyloidosis (immunoglobulin light chain amyloidosis). This is the most common type of amyloidosis in developed countries. …
  • AA amyloidosis. This type is also known as secondary amyloidosis. …
  • Hereditary amyloidosis (familial amyloidosis). …
  • Wild-type amyloidosis. …
  • Localized amyloidosis.

What are the 2 types of amyloidosis?

The most common types of amyloidosis are: AL (Primary) Amyloidosis. AA (Secondary) Amyloidosis.

What is the difference between primary and secondary amyloidosis?

Secondary means it occurs because of another disease or situation. For example, this condition usually occurs due to long-term (chronic) infection or inflammation. In contrast, primary amyloidosis means there is no other disease that is causing the condition. Systemic means that the disease affects the entire body.

What is the main cause of amyloidosis?

AA amyloidosis is caused by fragments of amyloid A protein, and affects the kidneys in about 80 percent of cases. It can complicate chronic diseases characterized by inflammation, such as rheumatoid arthritis (RA) or inflammatory bowel disease (IBS).

What is the difference between localized and systemic amyloidosis?

In systemic amyloidosis, the heart, kidneys, and nerves are most commonly affected, resulting in congestive heart failure, arrhythmia, nephrotic syndrome, renal failure, and peripheral and autonomic neuropathies. In localized amyloidosis, amyloid deposits at the site of production, so only one organ is disrupted.

What is primary systemic amyloidosis?

Primary amyloidosis is a rare disorder in which abnormal proteins build up in tissues and organs. Clumps of the abnormal proteins are called amyloid deposits.

How many forms of amyloidosis are there?

There are about 36 different types of amyloidosis, each due to a specific protein misfolding. Within these 36 proteins, 19 are grouped into localized forms, 14 are grouped as systemic forms, and 3 proteins can identify as either.

What kind of protein is amyloid?

Amyloids are aggregates of proteins characterised by a fibrillar morphology of 7–13 nm in diameter, a beta sheet (β-sheet) secondary structure (known as cross-β) and ability to be stained by particular dyes, such as Congo red. In the human body, amyloids have been linked to the development of various diseases.

How fast does amyloidosis progress?

How long that takes depends on the patient and the affected organ. Typically, 12 to 18 months will pass before amyloid buildup in the heart becomes fatal, while a patient with an affected kidney could live for 5 to 10 years, he says.

What is the best treatment for amyloidosis?

Recent studies have shown that people with newly diagnosed AL amyloidosis, the four-drug combination of subcutaneous daratumumab, bortezomib, cyclophosphamide, and dexamethasone is safe and effective. This treatment is now considered standard of care for most patients.

How long can you live with amyloidosis?

Amyloidosis has a poor prognosis, and the median survival without treatment is only 13 months. Cardiac involvement has the worst prognosis and results in death in about 6 months after onset of congestive heart failure. Only 5% of the patients with primary amyloidosis survive beyond 10 years.

Can you reverse amyloidosis?

There is not currently a cure for amyloidosis. The amyloid deposits cannot be directly removed. But there are treatments to stop more of the abnormal proteins being produced and treat your symptoms. These treatments can give your body time to gradually clear the deposits before they build up again.

What blood test shows amyloidosis?

Amyloidosis can be difficult to diagnose. There is no specific blood test and results of investigations vary greatly from patient to patient. The diagnosis of amyloidosis starts when a doctor becomes suspicious of the patient’s symptoms. A definitive diagnosis of amyloidosis can only be made through a biopsy.

What are the symptoms of end stage amyloidosis?

Common signs and symptoms of amyloid involvement in the heart include:
  • Dizziness.
  • Fainting.
  • Fatigue.
  • Fluid retention.
  • Low blood pressure.
  • Shortness of breath.