Amyloidosis is a disorder in which the body makes abnormal proteins. These amyloid proteins build up within the body, leading to tissue damage, organ failure, and health problems. Although amyloidosis is a rare disease, certain groups of people are more likely to be diagnosed with this condition. Each type of amyloidosis tends to affect different people and comes with its own set of risk factors.
Amyloid light-chain amyloidosis, also called primary amyloidosis or AL amyloidosis, is the most common type. About 4,500 people are diagnosed with this condition in the United States each year. AL amyloidosis most often affects people who are 60 to 70 years old. However, some people are diagnosed with this condition earlier or later in life. About 7 out of 10 people with AL amyloidosis are men.
AL amyloidosis is linked to blood cancers like multiple myeloma. People who have problems with their plasma cells may be at risk for developing AL amyloidosis.
Plasma cells are white blood cells located in the bone marrow (the spongy tissue found inside certain bones). Plasma cells make antibodies in order to fight infection. These antibodies are made up of light chain proteins.
Plasma cells sometimes develop abnormalities, leading to disorders like amyloidosis or myeloma. AL amyloidosis develops when plasma cells make abnormal amyloid proteins instead of the usual light chain proteins. Multiple myeloma is a type of cancer that develops when plasma cells begin growing out of control. Sometimes, these two conditions occur at the same time. AL amyloidosis occurs in 12 percent to 15 percent of people with multiple myeloma.
Other plasma cell disorders or blood cancers may also lead to AL amyloidosis. People with monoclonal gammopathy of undetermined significance (MGUS) or smoldering myeloma have a higher risk of being diagnosed with AL amyloidosis. MGUS and smoldering myeloma are plasma cell disorders that sometimes lead to multiple myeloma. Additionally, people with Waldenström's macroglobulinemia (WM) are at risk for AL amyloidosis. WM is a type of cancer that affects lymphoplasmacytic cells (blood cells that are similar to plasma cells). AL amyloidosis can also develop in people with various types of leukemia and lymphoma.
AA amyloidosis, also called secondary amyloidosis, develops as a response to another underlying disease. In the United States and other Western countries, about 0.5 percent to 0.86 percent of people are living with this condition.
People of any age can be diagnosed with AA amyloidosis, making this condition the only type of amyloidosis that can affect children. However, adults are more likely to develop the disease. About half of people with AA amyloidosis are at least 50 years old. Men are slightly more likely than women to develop AA amyloidosis.
In rare cases, AA amyloidosis develops in people who do not have an underlying condition or any known cause. These cases of amyloidosis are more likely to occur in women, older adults, and people with a higher body mass index.
AA amyloidosis is usually caused by health conditions that lead to inflammation. People with certain inflammatory diseases are at risk for developing AA amyloidosis.
When the body experiences inflammation, the liver makes a protein called serum amyloid A protein (SAA). When too much of this protein is made over a long period of time, part of the SAA protein can form amyloid deposits in tissues like the kidney, heart, and liver. Underlying conditions that may lead to AA amyloidosis include:
Fewer than 1 in 20 people with inflammation-causing disorders develop AA amyloidosis. However, the number of people who develop amyloidosis varies depending on the underlying condition. For example, some studies have shown that up to 30 percent of people with rheumatoid arthritis may have AA amyloidosis.
People are diagnosed with wild-type ATTR amyloidosis at an average age of 75. This condition usually affects men. As many as 1 out of 100 men over the age of 80 have wild-type ATTR amyloidosis. However, many may not realize it. People with this form of amyloidosis often don’t have symptoms. Additionally, many other things can cause cardiovascular conditions in this group of people, so doctors may not always realize when amyloidosis is responsible for heart disease.
Carpal tunnel syndrome may be a risk factor for developing wild-type ATTR amyloidosis. Carpal tunnel syndrome is a problem with the nerves in the wrist, leading to pain, weakness, or tingling in the hand and arm. About 1 out of 5 people with wild-type ATTR amyloidosis develop carpal tunnel syndrome five to nine years before they receive a diagnosis of amyloidosis. Men who are having wrist problems may want to ask their doctor whether they are at risk for developing amyloidosis-related heart failure.
Hereditary or familial amyloidosis is equally likely to affect men and women. In the United States, this disease is more common in African Americans. Rates of hereditary amyloidosis are also higher in Spain, Portugal, Ireland, France, Germany, Sweden, Finland, Greece, and Japan.
This condition is caused by gene changes that are passed down from parent to child. Hereditary amyloidosis can be caused by different genes. This condition is divided into two main groups. One type of hereditary amyloidosis is caused by mutations in the TTR protein. Other forms of hereditary amyloidosis, called non-TTR amyloidosis, can be caused by dozens of different mutations in genes like APOA1, APOA2, FGA, and LECT2.
These gene mutations are often passed down within families, although not everyone with the gene develops amyloidosis symptoms. People with a family history of the disease have an increased chance of developing amyloidosis. If your parent has hereditary amyloidosis, you have a 50 percent chance of having the disorder. Genetic testing can help you learn whether you have these gene mutations.
Kidney problems can sometimes lead to amyloidosis. Your kidneys are responsible for filtering out waste that your body doesn’t need. If your kidneys aren’t working properly, they may not be able to eliminate harmful amyloid proteins.
Some people with kidney failure may use dialysis treatments to help get rid of waste and toxins. However, dialysis may also be ineffective at removing amyloid deposits. If you have severe kidney disease, you have a greater chance of developing dialysis-related amyloidosis.
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