Secondary systemic amyloidosis

Definition

Secondary systemic amyloidosis is a disorder in which abnormal proteins build up in tissues and organs. Clumps of the abnormal proteins are called amyloid deposits.

Secondary means it occurs because of another disease or situation. For example, this condition usually occurs as a result of chronic infection or chronic inflammatory disease.

Systemic means body-wide. For example, a systemic disease affects the entire body.

See also:

• Cardiac amyloidosis
• Hereditary amyloidosis
• Primary amyloidosis

Alternative Names

Amyloidosis - secondary systemic

Causes, incidence, and risk factors

The exact cause of amyloidosis is unknown. You are more likely to develop secondary systemic amyloidosis if you have a long-term infection or inflammation.

This condition may occur along with:

• Ankylosing spondylitis
• Bronchiectasis
• Chronic osteomyelitis
• Cystic fibrosis
• Hairy cell leukemia
• Hodgkin's disease
• Long-term skin abscess and certain ulcers
• Long-term kidney dialysis
• Juvenile chronic arthritis
• Multiple myeloma
• Reiter syndrome
• Rheumatoid arthritis
• Sjogren syndrome
• Systemic lupus erythematosus
• Tuberculosis

Symptoms

• Bleeding in the skin
• Fatigue
• Irregular heartbeat
• Numbness of hands and feet
• Shortness of breath
• Swallowing difficulties
• Swollen arms or legs
• Swollen tongue
• Weak hand grip
• Weight loss

Signs and tests

Tests that may be done include:

• Abdominal ultrasound (may show a swollen liver or spleen)
• Biopsy or aspiration of fat just beneath the skin (subcutaneous fat)
• Biopsy of rectum
• Biopsy of skin
• Biopsy of bone marrow
• Blood tests, including creatinine and BUN
• ECG
• Echocardiogram
• Nerve conduction velocity
• Urinalysis

Treatment

The condition that is causing the amyloidosis should be treated. Some persons may need a bone marrow or stem cell transplant. There is no specific treatment for amyloidosis itself.

Expectations (prognosis)

How well a person does depends on which organs are affected. If the disease involves the heart and kidneys, it may lead to organ failure and death.

Complications

• Endocrine failure
• Heart failure
• Kidney failure
• Respiratory failure

Calling your health care provider

Call your health care provider if you have symptoms of this condition. The following are serious symptoms that require prompt medical attention:

• Bleeding
• Irregular heartbeats
• Numbness
• Shortness of breath
• Swelling
• Weak grip

Prevention

If you have a disease that's known to increase your risk of this condition, make sure you get it treated. This may help prevent amyloidosis.

References

Buxbaum JN. The amyloidoses. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 296.