Causes, Symptoms, and Types of Amyloidosis

Causes, Symptoms, and Types of Amyloidosis

The term amyloidosis refers to a broad group of diseases in which a particular protein (amyloid fibrils) builds up in one’s organs and tissues, affecting their function and appearance. It can affect any organ or part of the body and can result in a threat to life due to organ failure. There is no single treatment for this disease and the outcomes depend on the type and concentration of serum amyloid protein in the system.

Till the 1970s, the disease was thought to be correlated with a single amyloid substance and the classification was based on organ distribution and certain clinical observations. At present, a new system of classification is used wherein letters of the alphabet designate the protein that makes the majority of deposits. While the deposit patterns may differ between people, it usually consists of a single protein.

There are several different types of amyloidosis, such as the ones given below:

1. AL amyloidosis
This is the most frequently-occurring type. It is also known as immunoglobulin light chain amyloidosis. The letters AL refer to amyloid light chains— the term given to the type of protein that is responsible. It destroys the bone marrow due to certain self-generated antibodies that cannot be broken down. Multiple myelomas (a variety of blood cancer) is a correlated condition of this. The disease affects the nerves, heart, liver, kidneys, and intestines.

2. AA amyloidosis
This is caused by protein A and occurs with chronic inflammatory diseases like Crohn’s disease, rheumatoid arthritis, etc. and usually affects the kidneys. The liver, digestive tract, and heart may also be involved.

3. Dialysis-related amyloidosis (DRA)
DRA is common in people undergoing dialysis for more than five years and older adults. Protein deposits affect the joints, bones, and tendons in this type.

4. Hereditary amyloidosis
This is a rare, inherited form affecting the liver, nerves, heart, and kidneys and is thought to be caused by protein TTR.

5. Senile amyloidosis
Age-related amyloidosis is common in older men and is also caused by protein TTR deposits in the heart and other tissues.

6. Organ-specific amyloidosis
This occurs when protein is deposited in a single organ, including the skin. Some types have been linked to Alzheimer’s disease though there are no conclusive links.

7. Causes
There are no known or specific causes for the different types of amyloidosis. This illness can affect anyone though there are certain risk factors associated with it:

  • Most common between individuals aged 60–70
  • More men are affected as compared to women
  • Race
  • Inflammatory conditions
  • Family history
  • Dialysis for kidney problems

8. Treatment

There is no cure for this condition. Treatment consists mainly of managing symptoms and limiting further production of amyloid protein, based on the type of amyloidosis:

  • Chemotherapy and autologous blood cell transplant for AL amyloidosis.
  • Anti-inflammatory medication for AA amyloidosis.
  • Liver transplant for hereditary amyloidosis.
  • Change in the mode of dialysis or kidney transplant for dialysis-related amyloidosis.

Support medications for symptoms include pain relief medication, diuretics, low-salt diet, blood thinners, medication to control the heart-rate, compression stockings, and changes to diet for gastrointestinal types of amyloidosis.