Everything You Need To Know About Amyloidosis

Amyloidosis occurs when abnormal amounts of amyloid proteins build up in the body's tissues and organs. These proteins are put together in the bone marrow. They are present in minuscule amounts around the body.

When amyloids accumulate in organs excessively, it can prevent the organ from properly functioning. Some of the organs affected by this disease include but are not limited to: the heart, kidneys, liver, spleen, nervous system, and digestive tract.

Amyloidosis: What Are the Different Types?

Various proteins can cause amyloid deposits, but only a handful have connections to major health issues. The type of protein and its location shows the kind of amyloidosis you have. Amyloid deposits may accumulate throughout your body or in just one area.

There are different types of amyloidosis that affect parts of the body. Some of these include:

1. AA Amyloidosis

This condition formally referred to as secondary amyloidosis, is brought on by other chronic infectious or inflammatory diseases. The presence of fibrils in extracellular tissue often identifies diseases such as rheumatoid arthritis, ulcerative colitis, or Crohn’s disease.

These fibrils are from fragments of serum amyloid A protein (SAA) or intact SAA proteins. It most commonly attacks your kidneys but can also affect your digestive tract, liver, and heart.

2. Senile (Age-related) Systemic Amyloidosis

A senile systemic amyloidosis is a form of amyloidosis that occurs with age. The presence of amyloid fibrils made from normal transthyretin protein is a defining feature of this disease. Amyloid deposition typically affects the myocardium, resulting in arrhythmia and heart failure.

3. Immunoglobulin Amyloid Light Chain (AL) Amyloidosis

Immunoglobulin Amyloid Light Chain (AL) Amyloidosis is the most common type of amyloidosis. "AL" stands for "amyloid light chains," which are the proteins that cause the condition. Though there's no known root cause, it develops from bone marrow.

Primary amyloidosis occurs when your bone marrows create abnormal antibodies which the body can't break down. It's linked with a form of blood cancer called multiple myeloma and can affect organs such as your kidneys, heart, liver, intestines, and nerves.

4. Hereditary Amyloidosis (Familial Amyloidosis)

This form is rare and passed down through families. It affects the liver, nerves, heart, and kidneys. Many genetic defects are associated with amyloid disease, like the abnormal protein called transthyretin (TTR).

5. Organ-specific Amyloidosis

This condition results in the buildup of amyloid protein in specific organs, including the skin (cutaneous amyloidosis).

Amyloidosis: What Are the Risk Factors?

There are no known specific causes of amyloidosis, but there are factors that may increase the risk of developing it. These include:

1. Age

The majority of cases of amyloidosis occur in individuals between the ages of 60 and 70. While onset can occur earlier, it is more common for children to show symptoms later on.

2. Dialysis

If on dialysis, large proteins can sometimes stay in your blood. If this happens, abnormal proteins might accumulate over time and deposit into your tissue. Excessive buildup of protein is less likely to occur with newer dialysis methods.

3. Gender

Out of the population with AL amyloidosis, nearly 70 percent are men.

4. Genes

Hereditary amyloidosis may occur in families due to a genetic mutation passed down from one generation to the next.

5. Race

People with African ancestry are more likely to have a genetic mutation that causes amyloidosis, which can damage organs, including the heart.

6. Related Diseases

There is a known connection between amyloidosis and other diseases. 12% to 15% of people with multiple myeloma also have AL amyloidosis.

Amyloidosis: What Are the Symptoms?

Symptoms of amyloidosis differ based on the type of protein involved and where in the body it collects. For example, symptoms of cardiac amyloidosis may appear as fainting, shortness of breath, or weakness due to irregular heart rhythms or heart failure.

Meanwhile, kidney-related amyloidosis may show swollen feet and legs. General symptoms associated with this condition include:

• Skin tone changes
• Extreme fatigue
• Sudden weight loss
• Weak grip
• Tongue swelling
• Frailty

Amyloidosis: What Are the Treatment Options?

Although there is no cure for amyloidosis, certain medications can impede amyloid protein buildup. Working with your doctor makes it easier to manage amyloidosis symptoms. In addition to treating amyloidosis, they need to prioritize the underlying condition if it is related to another.

A multidisciplinary team is crucial because amyloidosis doesn't affect a single organ but several organs simultaneously. This treatment plan requires different specialists to work together to provide a good possible solution for the patient.

These are the people who usually make up the treatment team:

• Cardiologist
• Gastroenterologist
• Hematologist
• Nephrologist
• Neurologist
• Pulmonologist

The kind of amyloidosis you have dictates what treatment option you'll need and how many organs are afflicted.

Treatment options for amyloidosis include:

• Kidney transplant
• Liver transplant
• Anti-inflammatory medicines like steroids for secondary amyloidosis
• High-dose chemotherapy with stem cell transplant
• Modern therapies hinder abnormal transthyretin protein (TTR) growth

Additional treatments that may ease symptoms of amyloidosis include:

• Diuretic medicine. Also known as water pills, these drugs help expel salt (sodium) and water from your body. Many diuretics work by aiding the kidneys to release more sodium into the urine. The added sodium helps reduce the amount of water in the blood and decreases the pressure on veins and arteries.

• Compression stockings. The compression from these stockings helps your blood vessels by making it easier for the arteries to transport oxygenated blood to your muscles and boost the veins. As a result, they can return deoxygenated blood to your heart.

• Thickeners. Some experts theorize that modifying a person's diet by adding thickeners may help improve swallowing difficulties regardless of the primary cause.

• Change in diet. Although diet changes cannot prevent or treat amyloidosis, eating a nutritious and balanced diet can help people with this condition achieve their good health and avoid certain complications.

Consult a Doctor Today!

Many may have amyloidosis and not know it until the later stages. It can be fatal if left untreated, but there are treatment options available that can help improve your prognosis.

If you think you may have amyloidosis, it's vital to see a doctor so you can get started on treatment as soon as possible. Consult a doctor today!