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Conditions Related to Amyloidosis

Medically reviewed by Todd Gersten, M.D.
Written by Maureen McNulty
Posted on April 1, 2021

People with amyloidosis — a rare disease that develops when the body makes high levels of certain abnormal proteins — may also have other health conditions. There are a few types of amyloidosis, which can affect different parts of the body. Sometimes, amyloidosis occurs because of other health conditions, but it can also cause changes that lead to additional diseases.

When someone has more than one disease at the same time, the conditions are known as comorbidities. When an additional medical condition develops during an existing disease or following a treatment, it is known as a complication. In people with amyloidosis, having a comorbidity or complication can make treatment more difficult and add to the disease burden.

Having other health conditions in addition to amyloidosis can make it harder for your health care team to diagnose or treat your amyloidosis, and it may even influence your prognosis (outlook). Understanding how other health conditions are related to amyloidosis can help you talk to your doctor about treatment options. Addressing related conditions may improve your quality of life, prolong survival, and help you better manage your overall health.

Comorbidities in Amyloidosis

Age plays a role in the risk for developing comorbidities. The longer a person lives, the more likely they are to be diagnosed with chronic health problems. Amyloidosis is more likely to occur in older adults. The most common form of this disease, amyloid light-chain (AL) amyloidosis, most often occurs in adults over the age of 60. Another type, wild-type ATTR amyloidosis, is typically diagnosed after the age of 70. Older adults with amyloidosis may be more likely to also have other health conditions.

Inflammation-Causing Diseases

AA amyloidosis is a type of amyloidosis that develops when other diseases cause ongoing inflammation. Inflammation is a healing process the body uses when it is injured or infected. However, when this process goes on for too long, it can cause damage. During the body’s inflammatory response, the liver makes a protein called serum amyloid A (SAA) protein. When inflammation lasts for a long time, pieces of SAA can form amyloid deposits in different organs, which can result in AA amyloidosis.

Several health conditions can cause inflammation and lead to amyloidosis. About 50 percent of people with AA amyloidosis have rheumatoid arthritis. Other types of arthritis can also lead to this condition, including juvenile arthritis, psoriatic arthritis, ankylosing spondylitis, and Reiter’s syndrome.

Other underlying diseases that can be risk factors for developing amyloidosis include:

  • Inflammatory bowel disease (IBD), including Crohn’s disease or ulcerative colitis
  • Inflammation-causing genetic diseases, such as familial Mediterranean fever (FMF) or tumor necrosis factor receptor-associated periodic syndrome (TRAPS)
  • Ongoing infections, including tuberculosis, osteomyelitis, bronchiectasis, or infections due to AIDS or cystic fibrosis
  • Certain cancers, such as Hodgkin lymphoma or renal cell carcinoma

Amyloidosis is rare even for people who do have these inflammatory conditions. More than 19 out of 20 people who have one of these diseases will never develop AA amyloidosis. However, if you have a chronic infection or immune condition, it may help to keep an eye out for signs and symptoms of AA amyloidosis. The first indications of amyloidosis often include signs of kidney damage, such as swelling in the legs or ankles and high cholesterol levels.

If you have an inflammatory disease and develop amyloidosis, it is important to properly manage both conditions. One of the best ways to treat AA amyloidosis is to manage the underlying disease. Reducing inflammation through medication, treating infections with antibiotics, or managing tumors through surgery can help lower levels of SAA proteins.

Kidney Disease

When a person has kidney failure, also known as renal failure, their kidneys can’t properly carry out their tasks, which can lead to a specific type of amyloidosis. The kidneys are responsible for several important functions:

  • Filtering blood and removing waste products and extra fluid
  • Helping produce new red blood cells
  • Supporting bone health
  • Keeping the body’s levels of minerals properly balanced

Kidney problems can cause waste and fluid to build up in the body, leading to swelling and less frequent urination. People with kidney failure may also have low levels of red blood cells, which causes tiredness, and they can develop hypertension (high blood pressure) or bone disease.

Kidney failure is often treated with dialysis, a procedure in which a machine is used to filter the blood. However, dialysis can’t effectively remove certain proteins, including a protein called beta-2 microglobulin. In some people undergoing this procedure, beta-2 microglobulin blood levels rise and form amyloids, leading to a condition called dialysis-related amyloidosis. This form of amyloidosis often leads to joint stiffness and pain, bone cysts, and carpal tunnel syndrome — a condition that can cause the wrists to become weak, numb, or painful.

Diagnosis of dialysis-related amyloidosis may include blood and urine tests to look for the presence of amyloid proteins, and imaging tests such as computed tomography (CT) scans or X-rays to look more closely at the bones and joints. People with this condition will have to manage their amyloidosis while also treating their kidney failure. Some symptoms, such as bone cysts and carpal tunnel syndrome, can sometimes be treated with surgery. Additionally, kidney transplantation may stop the amyloidosis from getting worse.

Blood Cancers

There is a link between amyloidosis and certain types of blood cancer. In particular, about 15 percent of people who have multiple myeloma go on to develop AL amyloidosis. Both of these diseases affect plasma cells, a type of white blood cell found in the bone marrow. Plasma cells produce antibodies, which help fight off infection. When there are problems with the plasma cells, parts of these antibodies, known as light chains, can form amyloid proteins. While amyloidosis is not considered a type of cancer, the same treatments — including chemotherapy — can be effective for both multiple myeloma and AL amyloidosis.

Other cancers can also be comorbid with amyloidosis. About 2 percent of people with AL amyloidosis have B-cell lymphoma. People with amyloidosis may also have Hodgkin lymphoma, medullary carcinoma of the thyroid, and macroglobulinemia.

Complications of Amyloidosis

Most types of amyloidosis are systemic, meaning they affect the entire body. Systemic amyloidosis can cause proteins to build up in multiple organs. These abnormal proteins can damage the organs and prevent them from working correctly. Therefore, amyloidosis can lead to several different complications.

Kidney Problems

Kidney disease and dialysis treatments can cause dialysis-related amyloidosis, but many types of amyloidosis can also cause kidney disease. Amyloid fibrils often collect in the kidneys, leading to kidney damage or kidney failure.

To diagnose kidney damage, your doctor may collect urine samples to check for high protein levels, or blood samples to look for fat, cholesterol, or albumin in the blood. You may also need a kidney biopsy, in which your doctor removes a sample of your kidney tissue and tests for the presence of amyloid proteins and for kidney damage. Treatment for amyloidosis-related kidney problems may include taking medications to support kidney function, reducing the amount of fluids you drink, and eating fewer foods containing salt, cholesterol, and saturated fats.

Heart Problems

Amyloid fibrils can collect in the walls of the heart, making it more difficult for the heart to fill with blood between beats. This type of abnormal heart function is called cardiomyopathy. Some people with amyloidosis-related heart symptoms develop arrhythmias (unusual heart rhythms) or experience shortness of breath. Cardiovascular symptoms are more common in the subtypes of amyloidosis caused by the transthyretin (TTR) protein, including wild-type ATTR and hereditary ATTR amyloidosis. When these diseases affect the heart, they are sometimes called cardiac amyloidosis.

Diagnosis of amyloidosis-related heart disease can be accomplished through several methods:

  • Imaging tests such as CT scans, magnetic resonance imaging (MRI), positron emission tomography (PET), or echocardiography
  • Nuclear imaging tests, which use a small amount of radioactive material to provide a picture of the heart
  • An electrocardiogram, which measures the electrical activity of the heart
  • A biopsy, in which a small piece of heart tissue is removed and further studied with laboratory tests
  • An echocardiogram, which uses sound waves to create a picture of the heart

Treatments for cardiac symptoms can include medications that reduce high blood pressure, drugs that help the body get rid of extra water, a pacemaker to correct problems with the heart’s electrical activity, or a heart transplant. Heart problems can be serious or even fatal, so it is important to get proper treatment when amyloidosis affects your heart.

Nerve Problems

Amyloidosis-related nervous system problems can lead to a condition called peripheral neuropathy. In this condition, the nerves that support the arms, hands, legs, and feet may be damaged. People with neuropathy can develop feelings of tingling, burning, pain, or numbness in these areas.

Talk to Your Doctor About Related Conditions

Having other health problems in addition to amyloidosis may complicate treatment and lead to less favorable outcomes. Managing comorbid conditions can improve your quality of life. Make sure to have regular follow-up appointments with your health care team and bring up any new or unusual symptoms you are having. Speak openly with your team about all of your health concerns, and consider seeking care from an amyloidosis specialist if you are facing challenges while managing this disease.

Amyloidosis Condition Guide

Posted on April 1, 2021
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Todd Gersten, M.D. is a hematologist-oncologist at the Florida Cancer Specialists & Research Institute in Wellington, Florida. Review provided by VeriMed Healthcare Network. Learn more about him here
Maureen McNulty studied molecular genetics and English at Ohio State University. Learn more about her here

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