When amyloidosis affects the liver, it’s called hepatic amyloidosis. In most cases, liver-related symptoms of amyloidosis are mild. However, serious problems like liver failure can occasionally happen.

Keep reading to learn how amyloidosis can impact the liver and what symptoms to watch for.

What Is Hepatic Amyloidosis?

Hepatic amyloidosis occurs when amyloid deposits build up in the liver. This can lead to liver problems that range from mild to serious.

Amyloidosis is a condition in which abnormal proteins clump together to form amyloid fibrils (protein fibers). When these fibrils collect in just one part of the body — such as the skin — it’s called localized amyloidosis.

In most cases, however, amyloid fibrils spread to affect an entire organ or multiple organs. This is called systemic amyloidosis. Hepatic amyloidosis is one possible effect of systemic amyloidosis.

What Causes Hepatic Amyloidosis?

People with different types of amyloidosis can develop symptoms that affect the liver. The type of amyloid protein involved plays a big role in how severe the liver involvement is and how quickly it progresses. Hepatic amyloidosis is most common in people with amyloid light-chain (AL) amyloidosis.

AL amyloidosis happens when a type of blood cell called a plasma cell makes abnormal pieces of antibodies called light chain proteins. These faulty proteins can build up in multiple organs, including the liver, heart, kidneys, and gastrointestinal (digestive) system.

In most people with AL amyloidosis, the liver isn’t the only organ affected. Around 90 percent of people with AL amyloidosis show signs or symptoms of liver involvement.

AA amyloidosis (also called secondary amyloidosis) is linked to long-term inflammation that causes high levels of serum amyloid A protein in the blood. Over time, this excess serum amyloid A can become abnormal and form harmful amyloid deposits in organs like the liver. About 60 percent of people with AA amyloidosis have some level of liver involvement.

In both AL and AA amyloidosis, amyloid proteins can collect in liver tissue and the walls of blood vessels. Over time, these deposits can damage hepatocytes (liver cells), causing them to die.

What Are the Signs and Symptoms of Hepatic Amyloidosis?

Hepatic amyloidosis can cause problems when amyloid proteins build up in the liver — a process known as amyloid fibril deposition — and interfere with how the liver works. For many people, signs and symptoms are mild and may only be found through medical exams or lab tests. But even without obvious symptoms, liver damage may still be happening.

Hepatomegaly

Hepatomegaly is the medical term for an enlarged liver. It’s one of the most common signs of hepatic amyloidosis. Studies estimate that between 57 percent and 83 percent of people with hepatic amyloidosis have an enlarged liver. In some people, the spleen can also be enlarged (a condition called splenomegaly).

Many people with an enlarged liver don’t feel any different. In hepatic amyloidosis, the size of the liver doesn’t always match the severity of symptoms. If symptoms do occur, they may include a feeling of fullness or bloating, or pain in the upper right side of the abdomen. A healthcare provider may detect an enlarged liver or spleen during a physical exam.

Abnormal Liver Function Tests

Liver function tests (LFTs) are blood tests that measure substances related to liver health. High levels of certain liver enzymes can be a sign that amyloidosis is affecting the liver.

The most common abnormal test result in hepatic amyloidosis is a high alkaline phosphatase (ALP) level. Other enzymes, such as aspartate aminotransferase (AST) and gamma-glutamyltransferase (GGT), may also be elevated.

General Symptoms

People with hepatic amyloidosis may have general symptoms related to systemic (whole-body) amyloidosis, such as:

• Jaundice (yellowing of the skin and whites of the eyes)
• Abdominal (belly) pain
• Fatigue (tiredness that does not improve with rest)
• Loss of appetite
• Unintentional weight loss
• Changes in bowel habits (such as diarrhea or constipation)

What Serious Complications Are Associated With Hepatic Amyloidosis?

Although rare, hepatic amyloidosis can lead to serious complications — including liver failure, portal hypertension, and liver rupture. If you notice any symptoms that could point to these complications, talk to your healthcare provider right away.

Cirrhosis and Liver Failure

Liver failure happens when the liver can no longer perform enough of its normal functions to meet the body’s needs. In people with hepatic amyloidosis, this can occur if amyloid deposits and scar tissue (cirrhosis) replace too much healthy liver tissue.

Symptoms of liver failure include:

• Fatigue
• Abdominal pain
• Nausea and vomiting
• Jaundice
• Easy bleeding and bruising
• Confusion
• Itching
• Dark-colored urine (pee)
• Light-colored stools (poop)

Portal Hypertension

Portal hypertension is a complication that can happen when amyloid deposits and scar tissue press on blood vessels in the liver. This increases pressure in the portal vein — the major vessel that brings blood to the liver — and can lead to internal bleeding.

Symptoms of portal hypertension may include:

• Blood in vomit or stools
• Ascites (fluid buildup and swelling in the belly)
• Edema (swelling in the legs and feet from fluid buildup)
• Confusion

Liver Rupture

Liver rupture is a rare but life-threatening emergency. It refers to severe internal bleeding from the liver. While uncommon, it has been reported in people with hepatic amyloidosis. This complication can cause the body to go into shock — a condition where not enough blood reaches vital organs.

Early signs of a liver rupture may include sudden abdominal pain and swelling.

What Other Conditions Are Associated With Hepatic Amyloidosis?

Systemic amyloidosis often affects more than one organ, so if you have hepatic amyloidosis, you may also experience symptoms related to amyloid buildup in other parts of the body.

The organs most commonly affected by AL amyloidosis include the heart, kidney, nervous system, and gastrointestinal tract.

Between 70 percent to 80 percent of people with hepatic amyloidosis have another health condition related to systemic amyloidosis, including:

• Heart failure — When the heart can’t pump enough blood to meet the body’s needs
• Nephrotic syndrome — A kidney problem where too much protein leaks into the urine (called proteinuria)
• Orthostatic hypotension — A drop in blood pressure that causes dizziness when standing up, often due to damage to the autonomic nervous system
• Peripheral neuropathy — Nerve damage that causes numbness, tingling, or pain, usually in the hands and feet

How Is Hepatic Amyloidosis Diagnosed?

The symptoms of hepatic amyloidosis can be caused by other health conditions. A liver biopsy is the most reliable diagnostic test to check for hepatic involvement in amyloidosis. During a liver biopsy, a healthcare provider removes a small sample of liver tissue. The liver tissue is stained with special Congo red dye and examined under the microscope using polarized light to look for signs of amyloid deposits.

Your healthcare team will order different tests to learn if hepatic amyloidosis or another condition is the cause of your symptoms, including:

• Liver function tests — Checks for elevated liver enzyme levels and other signs of liver damage
• Other blood tests — Checks for abnormal proteins in the blood and other health problems associated with hepatic amyloidosis
• Urine tests — Checks for protein in the urine
• Imaging tests — Checks for signs of amyloid deposits in the liver

What Treatment Options Are Available for Hepatic Amyloidosis?

Hepatic amyloidosis is a rare condition, so there’s no one-size-fits-all treatment. The best treatment depends on the type of amyloidosis and which symptoms you have or which organs are affected.
For example, a newer combination drug — daratumumab and hyaluronidase-fihj (Darzalex Faspro) — is approved to treat AL amyloidosis, the most common type that affects the liver.

Other treatments for AL amyloidosis may include:

• Chemotherapy (chemo), targeted immunotherapy, and corticosteroids, which may help slow or stop the disease’s progression
• A combination of medications — such as bortezomib (Velcade), cyclophosphamide (Cytoxan), and dexamethasone (Hexadrol) — which helps about 40 percent of people with AL amyloidosis
• Autologous stem cell transplantation, where your healthy stem cells are collected and returned to you after chemo to help rebuild your bone marrow

These treatments don’t remove amyloid that has already built up in the liver. If amyloidosis causes permanent liver damage, a liver transplant may be an option. However, replacing the liver does not stop systemic amyloidosis, and amyloid may build up again in the new liver over time.

Additional treatments may be needed for complications. For example, if you have signs of liver failure or portal hypertension, your doctor may prescribe medications to reduce fluid buildup or lower blood pressure.

What’s the Outlook With Hepatic Amyloidosis?

The outlook (prognosis) for people with hepatic amyloidosis can be serious, especially when the liver is affected by systemic amyloidosis.

Liver involvement often signals more advanced disease. However, catching and treating hepatic amyloidosis early may help improve outcomes.

When amyloidosis mainly affects the liver, about half of people live longer than 10 to 14 months. Without treatment, life expectancy drops to around eight to nine months.

Although liver damage can be life-threatening, heart and kidney complications are more commonly the cause of death.

It’s important to remember that amyloidosis affects everyone differently. Talk with your healthcare team about how your specific symptoms and treatment plan may impact your outlook.

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