The signs and symptoms of amyloidosis depend on the location and size of the amyloid deposits.
AL may affect any tissue. Signs and symptoms may be vague and can include the following:
Signs and symptoms of hereditary amyloidosis may include the following:
Signs and symptoms of secondary amyloidosis may include the following:
Most people who are diagnosed with AA have had their related inflammatory disease for a decade or more.
No one knows what causes amyloidosis. There may be more than one cause. Hereditary amyloidosis results from genetic changes that cause the body to make abnormal proteins. Researchers think that as we get older, damage builds up in the body and triggers the disease. This kind of damage may come from the body's use of oxygen (oxidation) and from free radicals (harmful byproducts formed when cells use energy). Amyloid is also more likely to form in people who have immune system problems. Once amyloid deposits start, they seem to continue building up in the same locations. The heart, kidneys, nervous system, and GI tract are the most commonly affected.
People with the following profile are at increased risk for developing amyloidosis:
Your health care provider may suspect amyloidosis based on your symptoms, and will perform a physical exam, including blood or urine tests. The only way your doctor can definitively diagnose amyloidosis is using a needle to remove a small amount of tissue to test for amyloid (called a biopsy). With hereditary amyloidosis, DNA tests may reveal the genetic change that caused the condition. Special x-ray studies of tissue samples may show the structure of amyloid deposits. Depending on the signs and symptoms, your health care provider may use other tests to learn more about your condition, such as which organs are affected and whether your condition is getting worse.
There is no cure for amyloidosis. Treatment focuses on lessening symptoms and reducing production of amyloid through diet and medications.
Those who have hereditary amyloidosis should consider going to genetic counseling to learn about the risks of passing the condition to their children.
Treatment involves decreasing the proteins can make up amyloid. Doctors may use chemotherapy to treat AL. Depending on which organs are affected, your health care provider may ask you to follow a special diet (a low-sodium diet, for example, may help control fluid retention if your heart or kidneys are affected). There is no cure for AA, but medication can improve survival. The underlying condition must be treated. A liver transplant may be necessary for hereditary amyloidosis.
Doctors use combination of prednisone (a corticosteroid) and melphalan (Alkeran, also used to treat some kinds of cancer) to treat AL. Stem cell transplants are also a treatment for AL.
To help manage symptoms, your health care provider may suggest:
Depending on which parts of the body are affected, if you have amyloidosis you may need one of the following procedures:
Dietary choices, supplements, and herbs that reduce inflammation may help prevent amyloidosis. Damage from oxidation may play a role in the development of amyloidosis (see What Causes It? section), so you may want to add antioxidants to your diet to help slow the disease. Not all antioxidants are compatible with certain medications. Amyloidosis should never be treated with complementary and alternative therapies alone. Work with a knowledgeable provider and inform all of your health care providers about any medications, herbs, or supplements you are taking.
Studies suggest that the following dietary choices may help prevent amyloidosis in people who are at high risk, or help slow the disease once it has developed:
Additional supplements may include:
To help prevent inflammation in general:
Flavonoids are plant compounds that fight damage from stress, oxidation, and inflammation. They may be useful as a supportive therapy to standard medical care in treating amyloidosis:
Previously, AL was thought to be untreatable and deadly. With current therapy, many patients survive more than 5 years. About 20% survive 5 years or longer. With AA, most people survive 5 - 10 years after their condition surfaces. Survival depends on how well the underlying condition is treated. In hereditary amyloidosis, the outlook varies depending on the type of gene mutation and when the condition is diagnosed. Some people survive as long as 15 years after the disease develops.
After diagnosis, your doctor may perform tests on a regular basis to check levels of protein-related substances, the size and placement of amyloid deposits, the development of the disease, and the effects of treatment.
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