What Is Amyloidosis?

Amyloidosis develops when specific proteins, known as amyloids, are abnormally deposited in the tissues and organs of the body. The deposited amyloids impair the function of organs, making the condition potentially fatal.

Amyloid is produced by plasma cells (a type of immune cell) in the bone marrow. Amyloids can be deposited in any tissue or organ in the body.

Primary amyloidosis - Begins in the bone marrow and primarily affects heart, kidneys, tongue, nerves and intestines. Not usually associated with other conditions.

Secondary amyloidosis - Associated with chronic infectious or inflammatory diseases. Mostly affects kidneys, spleen, liver and lymph nodes. Other organs may be affected.

Familial amyloidosis - Inherited condition which affects nerves, heart, and kidneys.

Certain types of arthritis and related conditions may be associated with secondary amyloidosis including:

• Rheumatoid arthritis
• Lupus
• Reiter's syndrome
• Ankylosing spondylitis
• Sjogren's syndrome
• Osteomyelitis

The symptoms of amyloidosis are defined by the organs affected. Some symptoms associated with amyloidosis include:

fatigue

numbness

weakness

weight loss

shortness of breath

swelling of arms and legs

difficulty swallowing

arrhythmia

skin abnormalities

tongue abnormalities

Serious conditions can result from improperly functioning organs. Decreased heart function and kidney failure are just two of the possible complications.

Definitive diagnosis is made by detecting amyloid in a biopsy specimen. A biopsy of the affected tissue or organ may be performed. Biopsy of skin including subcutaneous fat, rectal mucosa (inner lining of the rectum), or bone marrow may also help diagnose.

Other tests may include:

Heart evaluations (EKG or echocardiogram)

Kidney function tests

Abdominal ultrasound to detect enlarged liver or spleen

Evaluation for carpal tunnel to detect nerve involvement

Primary amyloidosis may be treated with chemotherapy. Autologous stem cell transplantation is another possible treatment. Secondary amyloidosis may be helped by aggressive treatment of the underlying condition. Organs affected should be treated as well.

Amyloidosis cannot be prevented and cannot be cured. As stated, the course of amyloidosis depends on what organs are affected. If the heart and kidney are affected, organ failure and death are possible. Organ involvement may result in death within 1 to 3 years.

_Sources:

_{Amyloidosis, MayoClinic.com}

_{Primary Amyloidosis, MedlinePlus}

_{Secondary Systemic Amyloidosis, ADAM}