Tangier disease is a very rare disease that you can inherit from your parents. People with Tangier disease have very little HDL, or the “good cholesterol” that helps you get rid of LDL or “bad cholesterol.” This allows LDL to build up in various parts of your body, such as your tonsils. It can also raise your risk of heart disease.
Tangier disease is a rare condition you can get from your parents. People with Tangier disease have very low levels of HDL (high-density lipoprotein). This type of cholesterol is known as “good cholesterol” because it helps get rid of the bad kind of cholesterol (LDL or low-density lipoprotein) that clogs your arteries.
Tangier disease can affect multiple organs in your body and can give you a higher risk of heart disease, which can lead to a heart attack or stroke.
The name for this disease comes from Tangier Island, which is off the coast of the commonwealth of Virginia in the United States. This is where people discovered the first cases of Tangier disease. However, people in other parts of America and the world have it too.
Tangier disease is extremely rare. Healthcare providers have diagnosed about 100 cases of it throughout the world.
In people with Tangier disease, fat (lipids) can build up because there’s not enough HDL to carry it to your liver, which gets rid of it. This collection of fat enlarges certain body parts, such as:
You may also have:
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Tangier disease symptoms are different from person to person, depending on where the fatty deposits settle. Symptoms can include:
Symptoms can appear at any time from shortly after birth to age 65.
You inherit Tangier disease through the _ABCA1 _gene you get from your parents. When this gene is abnormal, a problem with its instructions makes your body unable to move cholesterol out of your cells and into HDL. HDL takes bad cholesterol to your liver, which gets rid of it.
If you get an abnormal ABCA1 gene from each of your parents, you’ll have Tangier disease. If you get an abnormal gene from only one parent, you become a carrier for Tangier. You may not show signs of Tangier, but still can have low HDL.
If two parents who are carriers for the abnormal gene have a child, that child — regardless of gender — may:
Your healthcare provider can make a Tangier disease diagnosis after doing a physical exam and doing blood tests for HDL and one of its proteins. Genetic testing can confirm the diagnosis. If you can’t get a genetic test, your provider can take a tissue sample (biopsy) from several parts of your body.
You have a very small amount of apolipoprotein A1 (ApoA1) when you have Tangier disease. This protein is part of HDL, which is also low in people with this disease.
Tests to help with a Tangier disease diagnosis or to monitor the disease’s effects may include:
Healthcare providers don’t have a specific Tangier disease treatment they use for every person with the disease. Some people with Tangier disease may need to have an operation to remove an affected area, such as their tonsils or spleen.
Some foods can help raise your HDL (good cholesterol) and lower your LDL (bad cholesterol). These include:
Your healthcare provider may prescribe cholesterol-lowering drugs.
They may also recommend lifestyle changes to improve your HDL level, such as:
Side effects of medicines that lower your cholesterol may include:
You can’t prevent Tangier disease if your healthcare provider has confirmed that it’s in your genes. However, a genetic counselor can help you figure out the chances of passing it on to your child or children, based on your status and the other parent’s genetics.
The Tangier disease prognosis is usually good. However, it depends on how your symptoms progress. As it is with many diseases, it’s best if you get an early diagnosis before symptoms get worse.
There’s no cure for Tangier disease, but gene therapy may help in the future.
Since Tangier disease is in your genes, you’ll have it for the rest of your life. Early diagnosis and continued checkups can help you manage this disease.
If you have an enlarged spleen from Tangier disease, you shouldn’t play contact sports that could put you at risk of your spleen rupturing. Also, you can manage your other risk factors for heart disease, such as:
You should see your healthcare provider regularly so they can respond quickly if your symptoms get worse.
Your provider should do regular checkups of your:
You may want to ask your healthcare provider the following questions:
A note from Cleveland Clinic
Because Tangier disease can affect different parts of your body, it’s best to get regular checkups for those areas. You may need to see several different healthcare providers in order to monitor the various issues you can have with Tangier disease. Be sure to keep taking any medicine your provider prescribed for you and do your best to make diet and exercise work in your favor.
Last reviewed by a Cleveland Clinic medical professional on 08/05/2022.
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