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Life Expectancy With Amyloidosis

Medically reviewed by Mark Levin, M.D.
Written by Emily Wagner, M.S.
Posted on September 13, 2021

Amyloidosis is most frequently diagnosed in people between the ages of 60 and 70. When you first receive an amyloidosis diagnosis, you may have questions about life expectancy. Currently, amyloidosis cannot be cured. However, many treatments are available that can successfully slow the progression of the disease and help control your symptoms, increasing your life expectancy and improving your quality of life.

The biggest predictor of life expectancy with amyloidosis is typically how much the heart is affected by amyloid protein deposits. Damage to the heart and cardiovascular system can negatively affect outlook. The kidneys, nervous system, and gastrointestinal tract can also be affected and play a role in overall life expectancy.

Life expectancy may also be affected by the type of amyloidosis you have and the treatments you may receive. Types of amyloidosis include:

  • Amyloid light-chain (AL) amyloidosis
  • Amyloid A (AA) amyloidosis
  • Hereditary transthyretin (hATTR) amyloidosis
  • Wild-type amyloidosis

AL Amyloidosis: Treatments and Life Expectancy

Amyloid light-chain amyloidosis (also known as primary amyloidosis) is named after the abnormal antibodies, known as light chains, that the body produces in the disease. Typically, plasma cells found in the bone marrow create antibodies that fight infections. If a plasma cell turns into a cancer cell, it produces too many light chains. These light chains can then enter the bloodstream, travel throughout the body, and settle into organs where they can cause damage.

AL amyloidosis is similar to multiple myeloma, a type of bone marrow. The two diseases often overlap, and many people are diagnosed with both.

AL Amyloidosis Treatments

If a person's AL amyloidosis is treated effectively within six months after diagnosis, their outlook is generally positive and they can live for many years. Some of the treatments used for AL amyloidosis are also effective for treating multiple myeloma because the conditions are so similar. These drugs, approved by the U.S. Food and Drug Administration (FDA), include:

AL Life Expectancy

The treatments currently available for AL amyloidosis can often help people live longer. In fact, certain drug combinations can sometimes put people with the condition into long-term remission (periods without any symptoms or signs of disease).

In a study of 46 people with AL amyloidosis, 33 percent who took both dexamethasone (sold as Decadron) and melphalan achieved complete remission. Additionally, another study found that thalidomide treatment put people with AL amyloidosis into remission for 10 years or more. These remission periods can improve life expectancy.

A staging system has been developed to help predict outcomes of people with AL amyloidosis. It is broken into four different stages and looks at three proteins that are found in people with the disease. The earlier the stage is, the better the average life expectancy:

  • Stage 1 — 9.8 years
  • Stage 2 — 6.3 years
  • Stage 3 — 5.3 years
  • Stage 4 — 2.3 years

Read more about AL amyloidosis.

AA Amyloidosis: Treatments and Life Expectancy

Amyloid A amyloidosis, also known as secondary amyloidosis, occurs when the liver makes abnormal serum amyloid A (SAA) protein. AA amyloidosis can be triggered by any condition that causes chronic (long-term) inflammation in the body. Factors that can shorten the life expectancy of people with AA amyloidosis include:

  • Being advanced in age
  • Having advanced kidney disease, known as end-stage renal disease
  • Needing dialysis treatments due to kidney failure
  • Having high levels of inflammatory proteins in the blood, such as immunoglobulin G or C-reactive protein
  • Having low levels of serum albumin protein

AA Amyloidosis Treatment

AA amyloidosis treatment focuses on treating the symptoms of the primary disease and any underlying inflammation, as well as reducing the levels of SAA protein. If these factors can be controlled properly, amyloid deposits made from the misfolded proteins can be reduced and organ function can improve. Decreasing amyloid deposits can help improve life expectancy by preventing damage that eventually leads to organ failure.

Recent advances in treatment have improved the outlook for people with AA amyloidosis. New medications focus on addressing the underlying inflammation in the body and can help lower SAA levels. These FDA-approved medications include:

  • Enbrel (etanercept)
  • Humira (adalimumab)
  • Ilaris (canakinumab)
  • Kineret (anakinra)
  • Remicade (infliximab)

AA Amyloidosis Life Expectancy

Overall, people with AA amyloidosis who undergo proper treatment can have a good outlook. The average life expectancy after diagnosis is 11 years. The earlier a person is diagnosed, the better their outlook tends to be. Treatments that begin early enough can often reverse the formation of AA amyloid deposits and help prevent kidney damage.

As research continues, new drugs are being discovered that can help control inflammation and improve outcomes for people living with AA amyloidosis.

hATTR Amyloidosis: Treatments and Life Expectancy

Hereditary amyloidosis is a type of amyloidosis caused by a gene mutation passed down from family members. The most common type is hereditary transthyretin (hATTR) amyloidosis, caused by an inherited mutated TTR gene. There are more than 100 different mutations that can cause hATTR amyloidosis, and they can affect the outlook of the disease. Treatments can also affect this outlook.

In hATTR amyloidosis, amyloid deposits can also form in the heart tissue, making it difficult for the heart to pump blood properly (a condition known as cardiomyopathy). This buildup in the heart can lead to a condition known as cardiac amyloidosis, or stiff heart syndrome. If left untreated, cardiac amyloidosis can lead to heart failure.

hATTR Amyloidosis Treatments

The FDA has approved two drugs for treating symptoms associated with hATTR amyloidosis. Onpattro (patisiran) and Tegsedi (inotersen) were both approved to treat polyneuropathy (nerve pain) in adults with hATTR amyloidosis.

In addition, diflunisal and tafamidis can also be used to treat nerve pain. These medications work by slowing the buildup of amyloid deposits on the nerves and in other parts of the body.

Because these medications are relatively new, researchers and doctors do not know how they affect life expectancy or outlook. More long-term studies are needed to determine whether these treatments are helpful. However, experts are hopeful that the medications will have a positive impact on people with hATTR amyloidosis.

Liver transplants can also be used to help treat hATTR amyloidosis because the amyloid is produced by the liver. Decreasing amyloid production through a liver transplant can help slow the progression of nerve damage in the arms, hands, legs, and feet (known as peripheral neuropathy). However, amyloid buildup can still continue to develop in the brain and eyes. The earlier the procedure can be done, the better the chances of preventing neuropathy and further damage.

hATTR Amyloidosis Life Expectancy

Overall, people with hATTR amyloidosis live between seven and 12 years after diagnosis. The particular genetic mutation in a person’s case of hATTR amyloidosis can either improve or worsen their outcome. For example, only 21 percent of people with the genetic mutation S50R live 10 years or longer after diagnosis. However, 85 percent of people with another mutation, V71A, are live 10 years or longer after diagnosis.

Genetic mutations can also help predict a person’s response to different treatments. For example, people with the V30M mutation in hATTR amyloidosis typically respond well to liver transplants, which can improve their life expectancy. Other factors can influence outlook after a liver transplant, including:

  • Age
  • The type of amyloid present
  • How much the brain and heart are affected by amyloid deposits

Read more about hereditary amyloidosis.

Wild-Type Amyloidosis: Treatments and Life Expectancy

Wild-type amyloidosis — also called wild-type ATTR amyloidosis, ATTRwt amyloidosis, or wild-type transthyretin amyloidosis — affects the same protein as hATTR amyloidosis. However, wild-type amyloidosis cannot be inherited through family. The normal, or “wild-type,” ATTR protein misfolds and deposits into tissues. This type mainly affects the heart and can cause carpal tunnel syndrome and peripheral neuropathy in some people.

Wild-type ATTR amyloidosis is usually diagnosed in people who are 65 or older. The disease slowly develops over time as amyloid deposits are formed, so the prognosis is generally better than for AL amyloidosis or hATTR amyloidosis.

Wild-Type Amyloidosis Treatments

The same drugs used to treat hATTR amyloidosis can also be used to treat wild-type ATTR amyloidosis, including tafamidis and diflunisal. Because the heart is primarily affected, a heart transplant can also be a good treatment option to help a person live longer. However, most people with wild-type amyloidosis are older and may not be good candidates for such an intense surgery.

Wild-Type Amyloidosis Life Expectancy

People with wild-type amyloidosis generally have a good outlook. One study found that 85 percent of people with the disease survived to their one-year follow-up appointment. People who had severe heart complications were likely to have a worse outcome. However, heart transplants may help people with wild-type amyloidosis live longer; another study found that every participant was alive three years after their heart transplant.

Read more about wild-type amyloidosis.

What Does This Mean for You?

Many factors go into estimating life expectancy and survival with a condition such as amyloidosis. Know that the above information is generally based on averages, and your own outcomes may be different. If you have questions or concerns about your own life expectancy, the best thing you can do is speak with your doctor. Your doctor can provide the best guidance based on your unique situation and risk factors.

Talk With Others Who Understand

MyAmyloidosisTeam is the social network for people with amyloidosis and their loved ones. On MyAmyloidosisTeam, members come together to ask questions, give advice, and share their stories with others who understand life with amyloidosis.

Have something to add to the conversation? Share your experience in the comments below, or start a conversation by posting on MyAmyloidosisTeam.

References
  1. Amyloidosis — Mayo Clinic
  2. Amyloidosis — Stanford Health Care
  3. AL (Primary) Amyloidosis — Stanford Health Care
  4. AL Amyloidosis — Cedars Sinai
  5. Recent Advances in the Diagnosis, Risk Stratification, and Management of Systemic Light-Chain Amyloidosis — Acta Haematologica
  6. A Changing Landscape of Mortality for Systemic Light Chain Amyloidosis — JACC: Heart Failure
  7. AL Amyloidosis — Amyloidosis Patient Information Site
  8. AA Amyloidosis — Amyloidosis Foundation
  9. Diagnosis and Treatment of AA Amyloidosis With Rheumatoid Arthritis: State of the Art — Amyloidosis
  10. Introduction to Amyloidosis — Amyloidosis Patient Information Site
  11. Treatment of AA Amyloidosis — Amyloidosis Patient Information Site
  12. Natural History and Outcome in Systemic AA Amyloidosis — The New England Journal of Medicine
  13. What Is Cardiac Amyloidosis? — Penn Medicine
  14. ATTR Amyloidosis — Amyloidosis Patient Information Site
  15. Hereditary Amyloidosis — Amyloidosis Foundation
  16. Familial Transthyretin Amyloidosis — Genetic and Rare Diseases Information Center
  17. Solid Organ Transplantation in Amyloidosis — Acta Haematologica
  18. Recent Advances in the Treatment of Familial Amyloid Polyneuropathy — Therapeutic Advances in Neurological Disorders
  19. Heart Failure Resulting From Age-Related Cardiac Amyloid Disease With Wild-Type Transthyretin — Circulation
  20. Wild-Type ATTR — Amyloidosis Foundation
  21. Wild-Type (Senile) ATTR Amyloidosis — Standford Health Care
  22. Outcomes After Heart Transplantation for Amyloid Cardiomyopathy in the Modern Era — American Journal of Transplantation
Posted on September 13, 2021
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Mark Levin, M.D. is a hematology and oncology specialist with over 37 years of experience in internal medicine. Review provided by VeriMed Healthcare Network. Learn more about him here
Emily Wagner, M.S. holds a Master of Science in biomedical sciences with a focus in pharmacology. She is passionate about immunology, cancer biology, and molecular biology. Learn more about her here

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