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Understanding ATTR Amyloidosis
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Amyloidosis is most often diagnosed in people between ages 60 and 70. When you first receive an amyloidosis diagnosis, you may wonder about life expectancy. While there is currently no cure for amyloidosis, treatments are available that can slow the disease’s progress, manage symptoms, and help improve both life expectancy and quality of life.
Amyloidosis is a systemic condition, meaning it affects the whole body. It causes abnormal protein fibers called amyloid to build up in different organs and tissues. How much the heart is affected is often the biggest predictor of life expectancy. Amyloid buildup in the heart and blood vessels can make the condition more serious. The kidneys, nervous system, and gastrointestinal (GI) tract — including the esophagus, stomach, and intestines — can also be affected and impact overall prognosis (outlook).
Life expectancy can also depend on the type of amyloidosis you have and the treatments you receive.
Amyloid light-chain (AL) amyloidosis, also called primary amyloidosis, is named after the abnormal proteins — known as light chains — that build up in the body. Normally, plasma cells in the bone marrow make antibodies to help the body fight infection. But when one of these cells becomes cancerous, it can produce too many light chains. These excess proteins can enter the bloodstream, travel through the body, and build up in organs, where they may cause damage.
AL amyloidosis shares features with multiple myeloma, a cancer of the bone marrow. The two conditions often overlap, and many people are diagnosed with both.
If AL amyloidosis is treated effectively within the first six months after diagnosis, the outlook is generally positive, and people can live for many years after. Some of the treatments used for AL amyloidosis are also effective for treating multiple myeloma because the conditions are so similar.
Currently, the only treatment approved by the U.S. Food and Drug Administration (FDA) specifically for AL amyloidosis is a combination therapy: daratumumab plus hyaluronidase (Darzalex Faspro), along with bortezomib, cyclophosphamide, and dexamethasone. However, in some cases, doctors may prescribe other drugs off-label (outside of their approved uses) — such as medications commonly used for multiple myeloma.
Today’s treatments for AL amyloidosis can often help people live longer. In fact, certain drug combinations can sometimes lead to long-term remission — meaning months or years without symptoms or signs of disease.
Survival rates for AL amyloidosis have improved in recent decades. With newer treatments, about 1 in 5 people are still alive 10 years after diagnosis.
Doctors use a staging system to help predict outcomes for people with AL amyloidosis. The system has four stages and is based on the levels of three proteins found in the blood: cardiac troponin, N-terminal pro B-type natriuretic peptide (NT-proBNP), and serum free light chains. These markers help show how much the heart is affected by the disease.
In general, earlier stages are linked to longer survival. The median overall survival (how long a person lives after the date of diagnosis or start of treatment) for each stage is as follows:
AA amyloidosis (or secondary amyloidosis) develops when the liver produces too much of a protein called serum amyloid A (SAA). This can happen when the body is experiencing chronic inflammation from conditions like rheumatoid arthritis or inflammatory bowel disease. Over time, this protein can misfold and form amyloid deposits in organs such as the kidneys, liver, and spleen.
Factors that can shorten the life expectancy of people with AA amyloidosis include:
Treatment for AA amyloidosis focuses on controlling inflammation and managing the underlying condition causing it. When inflammation is well managed, the production of misfolded SAA proteins may decrease, and organ function can improve. Reducing the amount of amyloid deposits can help protect organs and improve life expectancy.
Recent advances in anti-inflammatory treatments have improved outcomes for people with AA amyloidosis. While no medications are currently FDA-approved specifically for this condition, doctors often prescribe treatments off-label that target inflammation. These may include medications like:
With early and effective treatment, many people with AA amyloidosis can live more than 10 years after diagnosis. However, in advanced stages of the condition, survival may be more limited. For example, in late-stage disease, about half of people survive one year or more.
The earlier the diagnosis and treatment, the better the outlook. Starting treatment early may help reverse amyloid buildup and prevent long-term damage — especially to the kidneys.
Hereditary amyloidosis is a type of amyloidosis caused by a gene mutation (change) 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 known mutations that can cause hATTR amyloidosis, and they can affect the outlook of the disease, and the specific mutation can affect both symptoms and outlook.
In hATTR amyloidosis, amyloid can build up in the nerves and in the heart. When it affects the heart, this is known as transthyretin amyloid cardiomyopathy (ATTR-CM), a form of cardiac amyloidosis that causes the heart muscle to stiffen and pump less effectively. ATTR-CM can lead to heart failure if not treated. Some people may hear this described as “stiff heart syndrome.”
The FDA has approved several medications to help manage symptoms of hATTR amyloidosis. These include:
Some of these medications are used to treat nerve symptoms (polyneuropathy), while others — including acoramidis, tafamidis, and vutrisiran — are approved to treat ATTR-CM.
Diflunisal, a nonsteroidal anti-inflammatory drug (NSAID), is sometimes prescribed off-label to treat nerve pain. It works by stabilizing the TTR protein, which may help slow the buildup of amyloid deposits in nerves and organs.
Because these medications are relatively new, it’s not yet clear how much they will extend life expectancy. However, researchers and doctors are hopeful that they will significantly improve quality of life and long-term outcomes.
Liver transplants may also be used in some cases of hATTR amyloidosis. The liver is the main source of the faulty TTR protein, so replacing it can reduce amyloid production and slow the progression of peripheral neuropathy (nerve damage). However, a liver transplant does not stop amyloid buildup in the eyes or brain, since those areas produce some TTR protein on their own. The earlier a transplant is done, the greater the chance of protecting nerve and organ function.
Survival rates for hATTR amyloidosis vary based on several factors, including which organs are affected, which gene mutation is involved, and when treatment begins. New therapies may also help extend survival and improve outlook, especially when started early.
For people with hATTR amyloidosis and polyneuropathy, studies suggest survival rates of seven and 12 years after diagnosis. For people with ATTR-CM caused by the Val122Ile mutation, the average survival rate is shorter, about 2.5 years. The Val122Ile mutation is more common in people of African ancestry, according to research in JAMA.
Some gene mutations may respond better to specific treatments. For example, people with the V30M mutation may benefit from a liver transplant, which can help improve life expectancy. Several factors can influence survival after a transplant, including:
Wild-type ATTR amyloidosis — also called wild-type amyloidosis or ATTRwt amyloidosis — affects the same protein as hATTR amyloidosis. However, it is not inherited. In this condition, the normal (or “wild-type”) ATTR protein becomes unstable, misfolds, and forms amyloid deposits in the body. These deposits most often affect the heart but may also impact the nerves and soft tissues, sometimes causing carpal tunnel syndrome or peripheral neuropathy.
Wild-type ATTR amyloidosis is usually diagnosed in men who are 60 or older, according to the Amyloidosis Research Consortium. Because the condition develops slowly over time, the prognosis is often more favorable than that of AL or hereditary ATTR amyloidosis.
Many of the same drugs used to treat hATTR amyloidosis can also be used to treat wild-type ATTR amyloidosis. These include:
These medications work by stabilizing the transthyretin protein to slow or prevent the buildup of amyloid deposits in the heart.
Other drugs, such as eplontersen, inotersen, and patisiran, are currently being studied in clinical trials for wild-type ATTR amyloidosis.
Because the heart is primarily affected, a heart transplant may be considered in select cases. However, many people with ATTRwt are older and may not be eligible for open-heart surgery due to other health conditions.
People with cardiomyopathy due to wild-type ATTR amyloidosis generally have a better outlook than those with hereditary forms of the disease. Studies show that median survival after diagnosis is around 3.5 years, though this can vary depending on overall health and the severity of heart involvement.
A heart transplant may extend life expectancy in eligible individuals, though it is typically reserved for younger or otherwise healthier individuals with severe cardiac involvement. Early diagnosis and access to stabilizing medications may also help improve outcomes.
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 outcome may be different. If you have questions or concerns about your own life expectancy, speak with your doctor. They can provide the best guidance based on your unique situation and risk factors.
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.
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I was diagnosed w/AL Amyloidosis in '16 and have been lucky to have consulted/treated by some nationally recognized experts in the disease. I know this article is well-intended, however, I am… read more
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